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Part 4 80 Endocrinology & Rheumatology

SBAs, EMQs & MCQs Endocrinology SBA 1 Hypoparathyroidism Which one of the following is associated with hypoparathyroidism?

A. Coeliac disease B. Cystic fibrosis C. DiGeorge syndrome D. CREST syndrome E. Crigler Najjar syndrome

C: DiGeorge syndrome is a familial condition, characterised by hypoparathyroidism associated with intellectual impairment, cataracts and calcified basal ganglia. Endocrinology SBA 2 Addison’s disease Which of the following combination of biochemical disturbances is the classical finding in a patient with untreated Addison’s disease?

A. Hypernatraemia, hypokalaemia, hypoglycaemia B. Hyponatraemia, hyperkalaemia, hypoglycaemia C. Hyponatraemia, hypokalaemia, hypoglycaemia D. Hypernatraemia, hypokalaemia, hyperglycaemia E. Hypernatraema, hyperkalaemia, hyperglycaemia

B. Hypoadrenalism classically causes hyponatraemia, hyperkalaemia and hypoglycaemia. Mineralocorticoid deficiency has its predominant effect on the extracellular balance of sodium and potassium in the distal tubule of the kidney. Glucocorticoid deficiency has a major effect on carbohydrate metabolism, with associated hypoglycaemia.

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Endocrinology SBA 3 Phaeochromocytoma Which of the following is the most appropriate initial investigation if concerned re phaeochromocytoma?

A. 24 hour urinary 5-HIAA B. 24 hour urinary catecholamines C. Dexamethasone suppression test D. Insulin tolerance test E. Short synacthen test

B. 24hr urinary catecholamines. The measurement of urinary metabolites is a useful screening test if concerned regarding phaeochromocytoma. Normal levels on three 24hr collections practically exclude the diagnosis. High concentrations of 24hr urinary 5-HIAA (major metabolite of 5-HT) are found in patients with carcinoid tumours. The dexamethasone suppression test is useful in diagnosing Cushing’s syndrome. The short synacthen test is helpful in diagnosis of hypoadrenalism. The insulin tolerance test is used in the diagnosis or exclusion of ACTH and growth hormone deficiency. Endocrinology SBA 4 Hirsutism A 23yr old female presents with a 2month history of hirsutism, acne and deepening of her voice. She has a markedly elevated testosterone. What is the likely aetiology?

A. Polycystic ovarian syndrome B. Adrenal tumour C. Prolactinoma D. Carcinoid syndrome E. Wilson’s syndrome

B. Adrenal tumour. The short duration of her symptoms, and the substantially raised testosterone would make one suspicious of a virilising tumour. Serum testosterone may be elevated in PCOS, but not markedly so, and the symptoms would usually be of longer duration.

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Endocrinology SBA 5 Hyperaldosteronism A patient present with elevated blood pressure and low potassium. You are concerned about a possible secondary cause for her hypertension. She is no on any medication. Which screening investigation would be most appropriate?

A. Synacthen test B. Chest X Ray C. Plasma aldosterone:renin ratio D. 24 hour urinary catecholamines E. Clonidine suppression test

C. Plasma aldosterone:renin ratio. The main concern in a patient with hypertension and hypokalaemia is primary hyperaldosteronism. An elevated plasma aldosterone:renin ratio is a valuable screening test. Endocrinology SBA 6 Hypogonadism Which of the following is not associated with hypogonadism?

A. Loss of libido B. Testicular atrophy C. Osteoporosis D. Subfertility E. Gynaecomastia

E . Gynaecomastia. This may be a side-effect of the treatment for hypogonadism. Endocrinology SBA 7 Hypopituitarism Which of the following is not a cause of hypopituitarism?

A. Pituitary adenoma B. Kallmann’s syndrome C. Pituitary apoplexy D. Sarcoidosis E. Multiple sclerosis

E. Multiple sclerosis. There are numerous conditions causing hypopituitarism, including neoplastic causes (eg: pituitary adenoma), congenital (eg: isolated deficiency of pituitary hormones as in Kallmann’s syndrome), vascular (eg: pituitary apoplexy) and infiltrative causes (eg: sarcoidosis)

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Endocrinology SBA 8 Pituitary tumours A patient presents with galactorrhoea and amenorrhoea. Pregnancy test is negative. MRI pituitary confirms a pituitary mass. Which hormone is most likely being produced in excess?

A. Prolactin B. Testosterone C. Dopamine D. FSH E. LH

E. Prolactin. This lady’s symptoms suggest hyperprolactinaemia. There are many causes of hyperprolactinaemia, including prolactinoma, co-secretion of prolactin in acromegaly, stalk compression due to pituitary masses, hypothyroidism, polycystic ovary syndrome and medication. Dopamine has an antagonistic effect on prolactin production. Endocrinology SBA 9 Complications of diabetes Hypoglycaemia is defined when blood sugar falls below what level?

A. 2 mmol/l B. 2.5 mmol/l C. 3 mmol/l D. 4 mmol/l E. 4.5 mmol/l

D: 4mmol/l ENDOCRINOLOGY MCQ 1 Hypoparathyroidism A patient develops hypoparathyroidism following neck surgery. Which of the following features may develop?

A. Convulsions B. Circumoral numbness C. Short PR interval on ECG D. Diarrhoea E. Prolonged QT interval on ECG

A, B, E- Hypoparathyroidism presents as neuromuscular irritability and neuropsychiatric manifestations. The hypocalcaemia that results from hypoparathyroidism may be severe enough to cause a prolonged QT interval on ECG

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ENDOCRINOLOGY MCQ 2 Addison’s disease Routine investigations in patients with Addison’s disease may show:

A. hypercalcaemia B. hyponatraemia C. hyperkalaemia D. hypoglycaemia E. metabolic alkalosis

A, B, C, D- Patients typically get a metabolic acidosis, not alkalosis, in hypoadrenalism. Sodium reabsorption in the distal tubule is linked with acid secretion. Low levels of aldosterone stimulation of the distal tubule therefore leads to sodium wasting in urine and H+ retention in serum. ENDOCRINOLOGY MCQ 3 Hyperaldosteronism A patient has recently been diagnosed with Conn’s syndrome. Which of the following features may a patient with this have?

A. hypertension B. hyperkalaemia C. alkalosis D. hyponatraemia E. hypocalcaemia

A, C- Conn’s syndrome is elevated aldosterone in the absence of activation of renin-angiotensin system. Potential causes are adrenal adenoma, bilateral adrenal hyperplasia and adrenal carcinoma (rare). Patients typically have a hypokalaemic alkalosis. Sodium is usually mildly elevated or normal. ENDOCRINOLOGY MCQ 4 Phaeochromocytoma A patient attends with panic attacks, sweating, palpitations, facial flushing and hypertension. You suspect phaeochromocytoma. Which of the following investigations may assist you in the diagnosis of this condition?

A. 24 hour urinary catecholamines B. CT Adrenals C. Clonidine suppression test D. Dexamethasone suppression test E. Abdominal X Ray

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A, B, C- 24hr urinary catecholamines would be elevated. A CT Abdomen often helps to localise the tumours, MRI usually shows the lesion clearly. In a clonidine suppression test, plasma catecholamines are measured before and after an oral test dose is given to the patient. A positive test occurs if there is no reduction in plasma levels. ENDOCRINOLOGY MCQ 5 Hirsutism A 22yr old girl presents with hirsutism and oligomenorrhoea. She has a history of acne. Her BMI is 30. You think she likely has polycystic ovarian syndrome (PCOS). Which of the following may be used in the management of PCOS?

A. weight loss B. topical eflornithine C. sulphonylurea D. loop diuretic E. dianette

A, B, E- Topical eflornithine is used to treat facial hirsutism. The anti-androgen, cyproterone acetate competitively inhibits androgen production at peripheral receptors and may also reduce androgen synthesis. This is found in combined preparation in the oral contraceptive Dianette. ENDOCRINOLOGY MCQ 6 Hypogonadism Which investigations may be undertaken to assist in the diagnosis of hypogonadism?

A. FSH, LH B. Testosterone C. PSA D. ECG E. Chromosomal analysis

A, B, E- Testosterone level is essential to diagnose hypogonadism. FSH and LH are important to determine whether it is a primary pituitary cause (if FSH and LH are low). Chromosomal analysis is important, in particular to exclude Klinefelter’s syndrome. PSA is measured when patients are treated with testosterone. ENDOCRINOLOGY MCQ 7 Pituitary tumours Which of the following treatments may be used in management of a microprolactinoma?

A. bromocriptine B. cabergoline C. carbamazepine D. cimetidine E. thyroxine

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A, B- A and B are dopamine agonists, therefore reducing the production of prolactin. ENDOCRINOLOGY MCQ 8 Hypopituitarism Which of the following are possible clinical features in a patient with hypopituitarism?

A. loss of axillary hair B. loss of libido C. dysmenorrhoea D. hypertension E. bitemporal hemianopia

A, B, E- The clinical features associated with hypopituitarism are many – due to both an underproduction of pituitary hormones and possible mass effects from a pituitary tumour. Females may have oligo- or amen-orrhoea rather than dysmenorrhoea. It is also more likely for patients to have hypotension than hypertension. Rheumatology SBA on joint pain A 25 year old lady presents with increasing joint pains. Clinical examination reveals swollen tender DIP joints, pitting in her nails and swollen toes. Her spinal movements are also restricted. She also describes episodes of bloody diarrhoea and weight loss over the past year which have never been investigated. Which of the following is the most likely cause of her joint pains?

A. Rheumatoid arthritis B. Psoriatic arthritis C. Fibromyalgia D. SLE E. Reactive arthritis

b. Psoriatic arthritis Psoriatic arthritis (PSA) is the most likely cause as evidenced by the DIP joint involvement and the dactylitis (swollen toes). Although she may not have the actual skin rash she has evidence of nail psoriasis in the form of pitting. Oncholysis is another manifestation of nail psoriasis. She may well have an associated spondylarthropathy which needs to be investigated as do her bowel symptoms - she may have inflammatory bowel disease which can be associated with PSA and spondylarthropathy

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Rheumatology MCQ on joint pain A 45 year old lady complains of increasing widespread joint pains which are worse in the evening after a stressful day at work. She describes puffy hands and feet and a painful neck. Her concentration is very poor and she has recently suffered from marital problems. Rheumatoid factor is mildly positive . Examination reveals an increased BMI and global restriction of movement due to pain, but no synovitis. Which of the following investigations would be useful in this case?

A. Anti-CCP antibody B. USS hands and feet C. Anti-JO-I antibody D. ESR and CRP E. Anti-mitochondrial antibody

A, B and D. This lady most likely has fibromyalgia as evidenced by the lack of objective signs and the association with stress and family problems. However, it is always very important to exclude organic disease before a firm diagnosis can be reached. Rheumatoid factor can be mildly positive in many of the normal population therefore anti-CCP antibody which is more sensitive for inflammatory disease and inflammatory markers, such as ESR and CRP would be helpful. Plain x-rays would likely be normal even in early inflammatory disease, however USS is more sensitive at picking up early joint damage which could be indicative of underlying inflammation. Rheumatology SBA on back pain A 25 year old sportsman attends with increasing low back pain over the past 3 months particularly in the mornings. He describes no trauma or sudden initiating event. History taking reveals several episodes of an acutely painful eye for which he needed a course of steroid eye drops on each occasion. His sister and mother suffer from psoriasis. Neurological examination is normal, however the pain is fairly localised to his buttocks with no radiation. He also describes several attacks of a painful left Achilles’ tendon. What is the most likely cause of his back pain?

A. Osteoporosis B. Prolapsed intervertebral disc C. Sacro-iliitis D. Infective disciitis E. Sciatica

C. Sacro-iliitis This patient describes several features which are in keeping with the sero-negative group of arthridites - Sacro-ilitiis, psoriasis and iritis. Even a family history of these conditions is an association to be noted. Localised buttock pain is fairly typical of sacro-ilietis. The pain of sciatica would be expected to radiate while both infective discitis and a prolapsed disc should also produce

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some pain in the spine. There might also be systemic upset with infection. Osteoporosis itself should not give pain unless there is an associated collapsed vertebra. Rheumatology MCQ on back pain A 50 year old lady develops sudden mid-thoracic pain while coughing. The GP suspects a fractured rib so she attends for an x-ray. Her ribs look fine, however there is an abnormality at the level of her seventh thoracic vertebrae. She has a history of anorexia nervosa and although she has now recovered her BMI remains 18. She is not on any regular medication. Which of the following would be appropriate treatments in this case?

A. Steroids B. Physiotherapy C. Bisphosphonates D. Opiate analgesia E. High fat diet

C and d . This lady most likely has suffered a collapsed vertebrae as a result of underlying osteoporosis. Risk factors for osteoporosis include steroid use, coeliac disease, anorexia nervosa, low body weight, early menopause, positive family history, smoking and alcohol. A young age of this patient suggests that she is suffering from secondary osteoporosis. The acute pain of a collapsed vertebrae is very severe so she will need strong analgesia and in the longer term will need a DEXA scan and bisphosphonate treatment. Rheumatology SBA on RA A 30 year old girl with known seropositive RA is maintained on methotrexate 20mg weekly and is doing well. She is keen to start a family. What is the best advice to give her?

A. Continue with methotrexate and add oral steroids B. Stop methotrexate and start leflunamide C. Stop methotrexate and start cyclosporin D. Stop methotrexate and give oral steroids if necessary for disease flares E. Reduce the dose of methotrexate

D- Stop methotrexate and give oral steroids if necessary for disease flares. Methotrexate is a known teratogenic agent and therefore is not safe in pregnancy or prior to conception. The current advice is that it should be stopped for at least several months before conception. Obviously this may results in disease flares so therefore steroids can be used if necessary. Although there is limited data on a lot of the DMARDS, only sulfasalazine is thought to be probably safe if used with folate supplementation. Low dose steroids are relatively safe although high doses are associated with a small risk of cleft palate.

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Rheumatology MCQ on RA A 70 year old woman has an 8 week history of increasing small joint pain and swelling with associated weight loss and fatigue. She is very stiff especially in the mornings and complains of severe neck pain. She has painful soft tissue lumps over the extensor aspect of her upper forearms and tingling affecting her fingers which wakes her at night. Her rheumatoid factor is strongly positive. X-rays show erosions at her MCP joints. Which of the following are diagnostic criteria for rheumatoid arthritis?

A. Subcutaneous nodules B. Positive rheumatoid factor C. Weight loss D. Erosions on x-ray E. Carpal tunnel syndrome

A, b and d. The 1987 ACR revised criteria for the classification of RA include morning stiffness > 1 hour, swelling of three or more joint areas, swelling of the PIP, MCP or wrist joints, symmetric arthritis, positive rheumatoid factor, subcutaneous nodules and erosions or peri-articular osteopenia or x-ray. Patients must have four out of six criteria. Neck pain, weight loss, fatigue, carpal tunnel syndrome are all associated features, but are not diagnostic criteria. Rheumatology SBA on septic arthritis A 40 year old man with known rheumatoid arthritis presents with an acutely hot swollen knee which is extremely painful. ESR is 70mm/hr and CRP is > 200 mg/l. He is vomiting and pyrexic with a temp of 39 degrees. Joint aspiration yields 60 mls of turbid thick fluid. What is the most appropriate anti-microbial treatment?

A. IV flucloxacillin and oral fusidic acid B. IV co-amoxiclolav C. IV clindamycin D. Oral flucloxacillin and oral fusidic acid E. IV piperacillin

A- IV flucloxacillin and oral fusidic acid. Most septic joints are caused by either staphylococcus or streptococcus organisms therefore these would be the most appropriate antibiotics. Fuscidic acid is absorbed as effectively in oral form as long as the patient is not vomiting and can tolerate it. Flucloxacillin should be given intravenously in the first instance. Clindamycin is useful for patients who are penicillin allergic. Piperacillin is second line and may be used on the advice of the microbiologist if the patient is not responding or if antibiotic sensitivities are known.

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Rheumatology MCQ on septic arthritis A 25 year old male presents to Casualty with an acutely painful hot swollen ankle. There is no history of arthritis. He did however sustain a deep laceration to his calf a few days previously. He is pyrexic with rigors, his pulse is 120bpm and his blood pressure is 80/40mmHg. IV fluids have been started and paracetamol has been given. You are asked what immediate investigations are needed.

A. Immediate joint aspiration for culture and microscopy B. ESR and CRP C. Blood cultures D. Rheumatoid factor E. Throat swab

A, b and c. This patient is clearly very unwell and is severely haemodynamically compromised. The laceration is very likely the portal of entry for the infection which is causing severe systemic upset. It is very important to aspirate the joint in order to culture any organisms so that the correct antibiotics can be administered once sensitivities are known. Broad spectrum antibiotics can be started until sensitivities are known. Blood cultures will also be useful as the organisms may also be present in the blood (septicaemia). Inflammatory markers (ESR, CRP) will be useful to monitor the situation. Rheumatoid factor will not be particularly, helpful neither is there any indication for a throat swab. Rheumatology MCQ on osteoarthritis A 50 year old lady presents with increasing pain in her left knee particularly when descending stairs. There is no obvious swelling, but there is notable crepitus on examination. She also has bony swellings at her PIP and DIP joints. Her BMI is 32 and she is a heavy smoker who drinks occasional alcohol. Her mother has bilateral hip replacements. Which of the following are risk factors for osteoarthritis (OA)?

A. Positive family history B. Old surgery/trauma to the joint C. Obesity D. Alcohol excess E. Osteoporosis

A, b and c. Although the precise genetics of OA are not yet entirely known, initial work has shown a definite hereditary element to the disease so family history is definitely relevant and it is not solely a ‘wear and tear’ disease. Previous trauma or surgery to a joint makes it more pre-disposed to developing osteoarthritis as the original joint architecture has been disrupted. Obesity places extra stress/loading on the joints and therefore makes cartilage loss and subsequent OA more common. There is no definite evidence that alcohol increases the risk. Osteoporosis is a different disease which causes a loss of bone density.

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Rheumatology SBA on osteoarthritis A 60 year old teacher presents with widespread joint pain. Her hands are particularly painful notably the small joints in her fingers and the joint at the base of her thumb. Her wrist is also painful although she has a scar from surgery to repair a Colles’ fracture 20 years ago. She also describes left groin and knee pain when walking and also low back pain. She has difficultly finding shoes to fit due to ‘bunions’ bilaterally. Which of the following joints are NOT affected by primary osteoarthritis?

A. DIP joints B. CMC joints C. Wrist D. Hip E. MTP

C- Wrist. Primary or idiopathic OA affects the DIP and CMC joints in the hands, MTP joints in the feet, spine, acromio-clavicular shoulder joint and also the knee and hip. If any other joints are affected by OA then a secondary cause should be searched for. Secondary causes include congenital disorders or dysplasias, joint hypermobility syndromes, previous trauma, metabolic diseases such as haemachromatosis or crystal deposition diseases, endocrine disorders such as acromegaly or hyperparathyroidism, neuropathic joints, previous infections or inflammatory disorders. Rheumatology SBA on crystal arthropathies A 50 year old man presents with an acutely swollen knee. His CRP is 200mg/ l . He is apyrexic. Aspiration yields 2mls of turbid fluid. Negatively birefringent needle shaped crystals are seen on microscopy and there are no organisms seen. He is in severe pain, however his renal function shows a creatinine of 300µmol/l. His INR is normal. What is the best course of action?

A. NSAIDS B. Start allopurinol and colchicine C. Opiate analgesia D. Intra-articular steroid injection E. Oral steroids

D. Intra-articular steroid injection. The appearance of the negatively birefringent needle shaped crystals give a diagnosis of gout which is in keeping with the clinical features. His poor renal function limits the treatment options. NSAID’s are contraindicated as they could precipitate renal failure. Allopurinol should not be started straight away anyway, however it will need to be used with caution (if at all) with this degree of renal impairment. Likewise colchicine can cause diarrhoea which could lead to dehydration which again would not be a sensible risk to take with this patient. Steroids are useful to settle the acute inflammation and giving them locally directly into the joint would be more appropriate as it would reduce their systemic absorption and hopefully act more quickly. Opiate analgesia could be used sparingly, however it will not settle the inflammation cause by the crystal deposition.

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Rheumatology SBA on autoimmune CT diseases A 40 year old solicitor presents feeling ‘out of sorts’. She describes cold hands and feet and the typical colour changes of Raynaud’s phenomonen. On examination however, tightening and thickening of her skin is noted from her fingertips extending up to below her shoulders. Her face is not affected. She also describes increasing shortness of breath on minimal exertion. Urinalysis shows proteinuria and haematuria and she is hypertensive. A recent auto-antibody screen shows a positive anti-topoisomerase (anti-Scl-70) antibody.Which of the following is the most likely diagnosis?

A. Limited cutaneous scleroderma B. Diffuse systemic sclerosis C. Limited systemic sclerosis D. SLE E. Sjogrens disease

B. Diffuse systemic sclerosis. Systemic sclerosis (SSc) is a connective tissue disease characterised by progressive vascular dysfunction, auto-immune inflammation and finally fibrosis which is often irreversible. The skin and internal organs are affected. It is classified into several groups. Limited systemic sclerosis involves the face and the skin distal to the elbows and knees only, while diffuse systemic sclerosis involves skin both proximal and distal to the elbows and knees. Unfortunately it carries a worse prognosis and more often is associated with kidney disease (manifesting in this patient as proteinuria and haematuria). Urgent attention needs to be given to the kidney disease as patients can develop an acute renal crisis accompanied by severe hypertension. Mortality is high in these cases. Lung fibrosis can present in both groups although it is more common in diffuse SSc. Anti- SCL -70 antibody is more commonly associated with diffuse SSc, whilst anti-centromere antibody is more common in limited SSc. Rheumatology MCQ on autoimmune CT diseases A 35 year old plumber presents with difficulty swallowing and shortness of breath. He is unable to rise from a squatting position and so finds difficulty with his job. He has discolouration around his eyes and on his hands. Examination reveals grade 3 muscle weakness in his thighs. Which of the following would be helpful in diagnosis in this case?

A. Anti- Jo-1 antibody B. HRCT chest and PFT’s C. Anti-Ro antibody D. Muscle biopsy E. Anti-LA antibody

A, B and D. The most likely diagnosis in this case is dermatomyositis. This is an autoimmune inflammatory disease of muscle which typically causes proximal muscle weakness. There are associated skin manifestations in the form of purplish eyelid discolouration and papules over the knuckles

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(Gottron’s papules). It can be associated with lung fibrosis so imaging of the lungs is needed in this case.. A muscle biopsy would be important in order to get a definite histological diagnosis which is useful for prognostic purposes. Many cases are associated with the anti-Jo-I antibodies which are anti-synthetase antibodies. CK levels are also usually raised, which reflects the muscle inflammation. Rheumatology MCQ on DMARD side-effects A 35 year old lady has a history of poorly controlled rheumatoid arthritis. She has previously been treated with salazopyrin, leflunamide and gold. She is now doing well on a combination of methotrexate and hydroxychloroquine. As a result her prednisolone dose is being gradually reduced. She has had a persistently low white cell count for several years, which needs constant monitoring. Her most recent results show a white cell count of 2.5 x 109/L and a neutrophil count of 0.5 x 109/L . Which of the following could have contributed to her neutropenia over the years?

A. Methotrexate B. Leflunamide C. Prednisolone D. Diclofenac E. Salazopyrin

A,B ,E. Unfortunately neutropenia is a side-effect of many of the DMARD’s. Patients must be informed of this and must undergo regular blood monitoring. If the white cell count is persistently low, but stable, and if the patient is not suffering from regular infections then they may usually remain on the treatment. If however the cell count continues to drop or they get repeated infections then the drug must be stopped. Prednisolone is a steroid which does not usually affect the white cell count. Diclofenac is a NSAID. Rheumatology SBA on DMARD side-effects A 60 year old lady with known rheumatoid arthritis attends the clinic with her daughter. She is doing very well with no inflammation and leads an active life. She has been maintained on the same therapy for over 20 years. Her daughter reports that she has developed a blue-grey discolouration on her face gradually over the past year and it has become more noticeable. Otherwise she is well with no other side-effects. She attends her GP practice for regular blood monitoring which has been satisfactory. Which of the following DMARDS could be responsible for her skin discolouration?

A. IM gold B. Methotrexate C. Leflunamide D. Azathioprine E. Salazopyrin

A) IM gold

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This skin discolouration is known as Chrysiasis and develops as a result of prolonged IM gold therapy. It is usually asymptomatic apart from the obvious cosmetic appearances. Often if patients are achieving good disease control with the IM gold they are willing to accept the skin discolouration. Rheumatology MCQ on vasculitis A 30 year old male attends A and E with fever, abdominal pain and arthralgia. He is severely hypertensive with haematuria and proteinuria on urinalysis. He has a purpuric rash over his legs and trunk and also admits to severe testicular pain. ESR >100mm/hr, CRP is normal. An urgent vasculitic screen shows a negative p ANCA and c ANCA. A subsequent renal angiogram shows renal artery aneurysms. What is the most likely diagnosis?

A. Wegener’s granulomatosis B. Polyarteritis nodosa (PAN) C. Takayasu’s artertitis D. Polymyalgia rheumatica E. Microscopic polyangiitis

b. PAN Pan is a vasculitis affecting medium sized vessels. It typically causes constitutional symptoms. The most serious effects are as a result of aneurysm formation and can affect blood vessels in the brain, kidney or heart causing organ failure and subsequent death if not treated. ESR will be non-specifically raised in any vasculitic disease. CRP is usually normal in vasculitis unless there is co-existing infection. cANCA is usually indicative of Wegener’s granulomatosis and p ANCA (although less specific) can be positive in microscopic polyangiitis. Rheumatology SBA on vasculitis A 30 year old secretary presents with haemoptysis. She also has a vasculitic rash on her legs where some areas are coalescing and forming ulcers. She has a background of sinusitis for the past few years. Her ESR is 90mm/hr. She has proteinuria on dipstick urinalysis. A chest-x-ray shows multiple focal opacities in the lungs. An autoimmune screen shows a negative anti-nuclear antibody, negative anti-ds DNA, negative rheumatoid factor but a positive cANCA screen. What is the most likely diagnosis?

A. Polymyalgia rheumatica B. Wegener’s granulomatosis C. Microscopic polyangiitis D. Kawasaki’s disease E. Churg-Strauss vasculitis

b. Wegener’s granulomatosis Wegener’s granulomatosis is a small vessel vasculitis which can affect any organ and is fatal if not treated. Typically the respiratory tract can be affected causing sinusitis, rhinitis, epistaxis, otitis media or haemoptysis. The formation of necrotising granulomata is typical of the disease (likely in the lungs in this case). Glomerulonephritis is another serious manifestation which needs urgent treatment. A vasculitic screen will typically show a positive c ANCA. Treatment with high dose

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steroids initially is needed followed by strong immunosuppression in the longer terms usually in the form of cyclophosphamide or mycophenalate. Polymyalgia is an inflammatory syndrome which usually affects older patients and causes pain and stiffness in the shoulder and/or pelvic girdles. It usually responds rapidly to steroid therapy. Kawasaki’s disease is a systemic vasculitis presenting usually in young children. Rheumatology MCQ on spondylarthropies A 45 year old solicitor attends for his routine out-patient clinic review. He has a diagnosis of ankylosing spondylitis with peripheral joint involvement and is maintained on salazopyrin and regular anti-inflammatory drugs. He is doing well from the point of view of his joints, however reports increasing shortness of breath on minimal exertion to the extent that he can no longer exercise for any length of time. He also describes episodes of a ‘red eye’. Clinical examination of the precordium detects a heart murmur and on auscultation of his chest fine bibasal crackles are heard. A chest-x-ray, echocardiogram and pulmonary function tests are requeseted in the first instance. Which of the following can be associated with ankylosing spondylitis?

A. Aortic stenosis B. Aortic regurgitation C. Apical lung fibrosis D. Anterior uveitis E. Episcleritis

B,C and D. Ankylosing spondylitis can be associated with several extra-articular features and this must be borne in mind when treating these patients. Aortic regurgitation (AR) is the associated valvular abnormality. His dyspnoea could be related to his AR or he may indeed have a degree of lung fibrosis which needs to be investigated with a chest x-ray and probably further imaging in the form of HRCT of the chest. Anterior uveitis is associated with the group of spondylarthropathies, namely ankylosing spondylitis, psoriatic arthritis, Reiter’s disease Rheumatology SBA on SPOND A 30 year old male teacher was diagnosed with ankylosing spondylitis when he was 22 year old. He had been maintained on regular anti-inflammatory drugs and was taking regular exercise in the form of swimming and football. Over the past year however the pain and stiffness in his spine (thoracic, cervical and lumbar) has been increasing and he is finding difficulty driving as his neck movements are becoming more restricted. His early morning stiffness lasts until lunchtime which makes teaching very difficult. He has tried several alternative anti-inflammatory drugs with no benefit. He has no other joint involvement and is otherwise healthy. What is the most appropriate course of action?

A. Commence anti-TNF treatment B. Start methotrexate C. Start oral steroids D. Regular hydrotherapy E. Add an additional NSAID

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A- Commence anti-TNF treatment. This patient’s disease is clearly progressing and is affecting his quality of life. There is no evidence for methotrexate for the treatment of spinal disease in ankylosing spondylitis – it is only useful if there is peripheral joint involvement. Steroids may help in the short term but are not advisable in the long term due to their many side-effects. Again hydrotherapy and physiotherapy are definitely beneficial, but this patient is already swimming and exercising to no avail. It is not advisable to take any more than one NSAID at any one time. Anti-TNF treatment is therefore the most appropriate and has proven to be very beneficial in these patients. Rheumatology EMQ 1 Causes of joint pain a)Rheumatoid arthritis b)Septic arthritis c)Gout d)Haemarthrosis e)Chondrocalcinosis f) Psoriatic arthritis g)Osteoarthritis h)Reactive arthritis i)SLE j)Ankylosing spondylitis k)Fibromyalgia l)Myositis Which of the above is the most likely cause of joint pain in each case: Q1. A 50 year old man presents with an acutely swollen red hot 1st MTP joint. His serum creatinine is 252 µmol/l and he drinks half a bottle of wine daily. c) Gout. Predisposing factors are male sex, alcohol, poor kidney function, diabetes, diuretic use. Typically extremity joints such as the toes are affected when the uric acid crystallises to form urate crystals which provoke an inflammatory reaction. Q2. A 70 year old woman presents with a hot swollen knee. There is no history of trauma. She is on warfarin for atrial fibrillation and her INR is > 7. She is apyrexic and inflammatory markers are normal. d) Haemarthrosis. Spontaneous bleeding is common with a very elevated INR. Aspiration will yield frank blood. Absence of a temperature and normal inflammatory markers makes sepsis less likely.

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Q3. A 42 year old lady is referred with fatigue, low mood, palpitations and widespread joint pains. There is no obvious synovitis on examination, but her muscles are very tender. Blood tests are normal. k) Fibromyalgia. Fibromyalgia is the most likely although it is important to exclude other causes. Normal blood tests and clinical examination make inflammatory joint disease less likely. The other symptoms such as tender muscles (‘trigger points’), fatigue, low mood and palpitations are also typical of fibromyalgia. Q4. A 23 year old girl complains of increasing morning stiffness. She is unable to play the piano because of bilateral MCP joint swelling . Her ESR is 52 mm/h and CRP 45 mg/l. Autoimmune screen is normal. Rheumatoid factor is positive. a) Rheumatoid arthritis (RA) is likely because of the stiffness and small joint symmetrical hand swelling. Psoriatic arthritis is an important differential diagnosis, but it is often asymmetrical and also can affect DIP joints. Elevated inflammatory markers are also in keeping with RA as is the positive rheumatoid factor. Q5. A 70 year old woman is recovering from pneumonia in hospital. She develops a painful swollen wrist. She is apyrexic. Positively birefringent rhomboid shaped crystals are aspirated. e) Chrondrocalcinosis. Chondrocalcinosis (pseudogout ) is very common is patients (especially the elderly) with an intercurrent illness such as diarrhoea or pneumonia. Wrists and knees are joints commonly affected and aspiration yields calcium pyrophosphate dehydrate (CPPD) crystals as opposed to gout which is caused by needle-shaped negatively birefringent urate crystals. Rheumatology EMQ 2 Which of the following are the most likely causes of joint pain in each case? a)Juvenile idiopathic arthritis b)Septic arthritis c)Scleroderma d)Osteoporosis e)Chondrocalcinosis f)Psoriatic arthritis g)Osteoarthritis h)Reiter’s syndrome i)SLE j)Ankylosing spondylitis k)fibromyalgia l)myositis

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Q1. A 50 year old diabetic man presents with rigors and a hot swollen knee, CRP > 200 mg/l, Temperature: 38 degrees. He is unable to weight bear. b) Septic arthritis. Infection must be considered because of the elevated temperature, rigors and high CRP. Diabetes is an added risk factor for sepsis. He will need immediate joint aspiration and IV antibiotics to prevent systemic sepsis and permanent joint damage. Q2. A 25 year old man presents with a swollen knee. He also has ‘scabby’ feet for over 3 months and recalls pain on urination on several occasions. He is sexually active with several partners and also describes episodes of a ‘pink-eye’ within the last few weeks. h) Reiter’s syndrome. Reiter’s syndrome comprises the triad of arthritis, conjunctivitis and urethritis. Chlamydia is an important organism to be considered. Keratoderma blenorrhagica or ‘scabby feet’ can occur in up to 10% of cases. Q3. A 22 year old man develops a swollen right ankle. Further questioning reveals he suffers from back and neck pain which improves with exercise. Spinal x-rays show loss of the normal lumbar lordosis and ‘bony bridges’ between the vertebrae. He also has an early diastolic murmur on auscultation of his precordium. j). Ankylosing spondylitis. Ankylosing spondylitis typically affects young males and peripheral joint involvement is common. Usually the back pain and stiffness are eased by physiotherapy and an exercise programme. Radiological features include sacro-iliitis, loss of lumbar lordosis and ‘bamboo – spine’ appearance caused by calcification of spinal ligaments. Aortic regurgitation is one of the less common extra-articular features. Q4. A 25 year old female presents with two swollen toes on the left foot, a swollen ankle and wrist. She has also suffered from episodes of iritis in the past and has been diagnosed with colitis following a recent colonoscopy. Her sister and mother suffer from psoriasis. f). Psoriatic arthritis. This patient describes dactylitis and has a positive family history of psoriasis. She also has other features consistent with the sero-negative group of arthridites - iritis and colitis. Q5. A 40 year old lady has painful swollen MCP joints on the left hand and a swollen right wrist. She as a photosensitive facial rash. Urinalysis reveals significant proteinuria. Rheumatoid factor is negative, but anti-nuclear antibody (ANA) and anti-ds DNA antibodies are positive. i). SLE. This lady fulfils the criteria for the diagnosis of SLE as she has five of the eleven ACR (American College of Rheumatology) diagnostic criteria – namely arthritis, photosensitivity, renal involvement (proteinuria), positive anti-ds DNA and ANA. Four are needed for a diagnosis of SLE.

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Rheumatology EMQ 3 Causes of Hip Pain Which of the following are the most likely causes of hip pain in each case? a)osteoarthritis b)septic Hip c)trochanteric bursitis d)synovitis e)iliopsoas abscess f)infective sacro-iliitis g)Loosening of a prosthesis h)referred pain from the knee i)Fractured neck of femur j)Avascuar necrosis Q1. A 60 year old farmer is suffering from increasing hip pain over several months. X-ray shows loss of joint space. Clinical examination reveals reduced movement overall, but total loss of internal and external rotation. He also has bony swelling at the PIP and DIP joints in his hands. a). Osteoarthritis (OA). The more chronic nature of the pain makes infection less likely. Typical x-ray features of OA are loss of joint space, osteophyte formation and sub-chondral sclerosis. Internal and external rotation are usually the first movements to be affected. He has evidence of widespread OA as he has Heberden’s (DIP) and Bouchard’s (PIP) nodes in his hands. Q2. A 45 year old lady complains of right sided hip pain especially when walking and lying in bed on the affected side. Hip movements are good, but she is locally tender to palpation over her left lateral thigh. c). Trochanteric bursitis. Trochanteric bursitis typically affects middle aged female patients. It occurs as a result of inflammation of the bursa overlying the greater trochanter of the femur. Pain is typically fairly localised to the area and the hip joint itself is not affected. Local steroid injection can be of benefit. Q3. An 18 year old girl complains of sudden onset right hip pain. All movements are severely reduced and any attempt to move results in severe pain. She is pyrexic with strongly elevated inflammatory markers. X-ray is normal. Ultrasound shows a large joint effusion. b). Septic Hip. A Septic hip must be considered given the sudden onset of the pain, the systemic upset (fever and raised inflammatory markers) and the severity of the pain. A large effusion on ultrasound is a clue that there is either inflammation or infection and infection seems more likely in this case. She needs urgent aspiration and IV antibiotics.

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Q4. A 58 year old lady has been on long term steroids for many years because of COPD. She develops sub-acute right hip pain. Her temperature is normal. X-ray shows total destruction of the femoral head. j). Avascular necrosis. The history is important here. Patients on long term steroids are at risk of many complications, including problems with their bones. Both osteoporosis and avascular necrosis are side-effects. In this case avascular necrosis is the cause as her femoral head has been totally destroyed. She will be in severe pain, but should be systemically well. Q5. A 30 year old girl with rheumatoid arthritis develops severe left hip pain. Her temperature is normal. X-ray is normal. Ultrasound shows a large effusion and aspiration yields 20 mls clear straw-coloured fluid which cultures no organisms. d). Synovitis. This girl’s rheumatoid disease has involved her hip joint although infection needs to be excluded. Again the effusion is a clue to either inflammation or infection however aspiration was sterile (no organisms) so infection can be safely excluded. A local steroid injection may be of benefit. Rheumatology EMQ 4 Treatment of back pain a)physiotherapy, muscle relaxants and analgesia b)IV antibiotics c)emergency radiotherapy d)emergency spinal decompression e)Exercise and NSAIDS f)local steroid injectiong g)hydrotherapy h)Opiate analgesia followed by bisphosphonates i)Emergency repair of AAA Q1 A 25 year old male patient suffers from back pain which is worse in the morning and after periods of rest. Spinal x-rays show a ‘bamboo spine’ appearance. He has also suffered from iritis in the past and has patches of a dry scaly rash on his skin. e). Exercise and NSAIDS. This young man most likely has ankylosing spondylitis which typically gives pain and stiffness which is worse after rest and improves with exercise. Ankylosis or bony fusion of the vertebrae give rise to a straight ‘bamboo-like’ appearance on x-ray. He also has other symptoms which would fit into the sero-negative group of arthritides - iritis and psoriasis. Physiotherapy/Exercise and NSAIDS (in the first instance) are the mainstay of treatment.

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Q2 A 50 year old diabetic patient develops sudden acute severe pain in her mid-thoracic region. She is pyrexic with a CRP > 200mg/l. X-ray is normal but an MRI scan shows destruction of the T7 vertebra and evidence of infection within the disc. b). IV antibiotics. This patient has discitis which is infection of an intervertebral disc. It can cause severe systemic sepsis and needs urgent IV antibiotics and supportive care. Surrounding bony destruction is common in severe infection as is abscess formation. Diabetics are particularly susceptible with staphylococcal infection being the most likely organism. Those who have undergone recent spinal intervention, such as spinal anaesthesia, are also at risk. Q3. A 60 year old man with prostatic cancer develops sudden pain in his lower back with loss of bladder control and weak legs. d). Urgent MRI and neurosurgical consultation. This patient may well have developed cauda equine syndrome as he is displaying several of the ‘red flags’ of back pain. His history of prostatic cancer is important as he may have undiagnosed bony/spinal metastatic deposits and he needs urgent decompression. Q4. A 72 year old woman develops sudden mid-thoracic pain while lifting her grandson. A recent DEXA scan had shown T scores of -4.5 in her spine. h).Opiate analgesia and bisphosphonates. This lady has severe osteoporosis as evidenced by her bone density scan results (< -2.5 ). As a results she is at high risk for a non-traumatic vertebral fracture which is likely what has happened in this case. She will need acute pain relief followed by bisphosphonates in the longer term to prevent further bone loss. Q5. A 42 year old man who was previously well develops sudden back pain while bending over. He is systemically well. There are no neurological signs on examination. a). Initial rest, muscle relaxants and analgesia, followed by physiotherapy. A prolapsed or ‘slipped’ intervertebral disc is the most likely cause in this case. Dr Elisabeth Ball

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Rheumatology EMQ 5 Autoantibodies of connective tissue disorders Which of the following tests could help in diagnosis in each of the following? a)Anti-ds DNA b)Anti-Ro C)Anti-Ro and LA d)Anti-RNP e)Anti-nuclear antibody f)CRP G)rheumatoid factor h)anti-CCP antibody i)Anti-JO – 1 J)ESR K)anti-mitochondrial antibody l)anti-SCL 70 m)anti-centromere antibody Q1 A 32 year old girl presents with a butterfly rash on her face, painful swollen finger joints, proteinuria and fatigue. ESR is 60mm/h, CRP is 5mg/l. a). Anti-ds DNA is fairly specific for SLE for which this patient displays characteristic symptoms – butterfly rash, arthritis/arthralgia, renal involvement and fatigue. ESR is typically elevated while CRP is low unless there is co-existing infection. Anti-nuclear amtibody may also be positive although it is not specific for SLE. Q2. A 40 year old lady complains of sore joints. Further questioning reveals a history of dry eyes and a dry mouth. c).Anti - Ro and La antibodies are typically found in Sjogren’s disease. The underlying pathology involves lymphocytic infiltration of salivary and lacrimal glands interfering with saliva and tear production. Infiltration of other organs, such as the lungs and GI tract can result in a variety of other less common manifestations. Q3. A 50 year old man has been diagnosed with pulmonary fibrosis after a HRCT chest. He also has proximal muscle weakness which responds to steroids and large ‘mechanics’ hands. i). Anti Jo-1 antibodies are found in myositis (giving rise to proximal muscle weakness). The syndrome of myositis, pulmonary fibrosis, anti-Jo-1 antibodies and ‘mechanics hands’ is known an anti-synthetase syndrome.

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Q4. A 47 year old lady suffers from painful colour changes of her hands precipitated by cold. She also has difficulty swallowing and has noticed tightness of her skin especially on her face and her fingers. m). Anti-centromere antibody. These symptoms are typical of limited scleroderma (previously known as CREST). Raynaud’s syndrome, oesophageal motility problems and sclerodactly are the features she exhibits. Anti-centromere antibodies are fairly specific to limited scleroderma. Anti-SCL-70 (anti-topoisomerase) antibodies are found in diffuse scleroderma. Q5. A 33 year old lady is under investigation for arthritis. She has a strong family history of rheumatoid arthritis (RA) affecting her mother and sister, and her own joint involvement is typical of RA with symmetrical small joints affected. However, her Rheumatoid factor antibody is negative as is her anti-nuclear antibody and anti-ds DNA antibody. h). anti-CCP antibody is a more recently discovered antibody which is much more specific for rheumatoid arthritis than rheumatoid factor which can be falsely positive or negative. The absence of ANA and anti-ds DNA make an auto-immune disease less likely. It is anticipated that her anti-CCP antibody would be positive as it is likely that she is indeed suffering from RA. Rheumatology EMQ 6 Which of the following is the most likely cause/association of Raynauds’ phenomonen?

A. Limited Scleroderma B. Cryoglobulinaemia C. Polycythaemia D. Primary Raynaud’s E. Beta blockers F. Thiazide diuretics G. ACE inhibitors H. SLE I. Polymyositis J. Gout K. Diffuse scleroderma

Q1. A 30 year old lady suffers from colour changes in her hands and feet secondary to the cold. She has also notices small white deposits under the skin on the tips of her fingers. She has difficulty swallowing. Her serology shows positive anti-centromere antibodies. a). Limited scleroderma. Raynaud’s in a common presenting feature in up to 70% of cases. Other features include calcinosis (hard white deposits), oesophageal dysmotility, sclerodactaly and telangectasia. Anti-centromere antibody is positive in 50-90% of cases of limited scleroderma.

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Q2. A 22 year old student notices increasingly painful colour changes in her hands and feet to the extent that she cannot go outside without gloves or fur-lined boots. Even putting her hand in the freezer precipitates the colour changes. She is otherwise very well with no associated symptoms. She has no family history of arthritis or connective tissue disease and all her blood tests and immunology tests are normal. d). Primary Raynaud’s or ‘Raynauds disease’. Primary Raynaud’s is the most likely in the absence of any other symptoms or positive antibodies. Q3. A 40 year old lady developed Raynaud’s fairly acutely. She has also noticed palpable purpura on her skin. A small ulcer has developed at the tip of her 3rd finger. Her joints are generally painful and she also has proteinuria on urinalysis. b). Cryoglobulinaemia. Cryoglobulinaemia results from the presence of cryoglobulins which are immunoglobulin complexes which precipitate and form a gel at low temperatures. Raynaud’s syndrome and digital and leg ulceration are common presentations; as are glomerulonephritis, arthritis and neuropathy. There are three different types depending on the type of cryoglobulin isolated from the blood. Q4. A 42 year old woman develops Raynaud’s since her anti-hypertensive medication was adjusted. She takes a beta-blocker, a thiazide diuretic and an ACE inhibitor. e ). Beta blockers. Beta blockers can cause Raynaud’s as a results of vasoconstriction. Other drugs which can be precipitating agents are vinblastine, bleomycin, cyclosporine, cisplatin, interferon-alpha. Q5. A 50 year old man complains of abdominal pain. He describes symptoms of Raynaud’s phenomonen. Examination reveals splenomegaly and also bruising on his skin. Laboratory investigations show a raised haemoglobin, haematocrit, red cell count and whole blood viscosity. c). Polycythaemia. Hyperviscosity which in turn restricts blood flow is a cause of Raynaud’s phenomonen. Polycythaemia refers to an increase in red cell count, haematocrit and haemoglobin. It can be primary (Polycythaemia Rubra vera) or secondary (due to increased erythropoietin levels either from hypoxia or increased production by the kidney). This patient is more likely to have PRV as evidenced by the splenomegaly and the Raynaud’s syndrome.

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Rheumatology EMQ 7 Causes of proximal myopathy a)polymyositis b)Alcohol use c)thyrotoxicosis d)Polymyalgia rheumatica e)McArdle’s syndrome f)dermatomyositis g)osteomalacia h)Cushing’s syndrome i)hypothyroidism j)Amiodarone k)myotonic dystrophy l)osteoporosis Q1 A 45 year old man develops weakness of his legs – examination reveals proximal myopathy, a rash over his knuckles and a purple discolouration around his eyes. He reports difficulty swallowing. CK levels are >3,000iu/l and his anti-Jo-1 antibody is positive. f). Dermatomyositis. This patient has all the features of this inflammatory myopathy. Dermatomyositis differs from polymyositis in that there is cutaneous involvement. CK levels are typically high and muscle biopsy would show inflammation within the muscle. Treatment is steroids initially. A percentage of patients will have an underlying malignancy so it is important that this is screened for. Q2. An 80 year old lady develops acute significant pain, stiffness and weakness of her pelvic and shoulder girdle. She is unable to dress herself or brush her hair. She also complains of a temporal headache. Her ESR is 90mm/h. d). Polymyalgia rheumatica (PMR). The age of the patient is a good clue to diagnosis as generally patients with PMR are elderly. Lack of functional ability is common. Elevated ESR is another hallmark feature and there is an association with temporal arteritis which is characteristed by a temporal headache, jaw claudication and in severe cases the optic nerve can be affected. It responds very dramatically to high dose steroids which should be tapered quickly. If there is no response to steroids then the diagnosis should be questioned. Q3. A 50 year old man complains of pain in his long bones and pelvis. On examination he has proximal muscle weakness and a waddling gait. He has a history of chronic liver disease, but no history of alcohol abuse. Laboratory tests show a low serum calcium level and an elevated serum alkaline phosphatase (ALP).

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g). Osteomalacia. Osteomalacia occurs as a result of impaired mineralization of bone. It can happen as a result of Vitamin D deficiency, abnormal metabolism of Vitamin D (eg in liver disease), low phosphate levels or the presence of bone mineralization inhibitors such as aluminium. It results in pain, deformity and proximal myopathy. Typically calcium and phosphate levels are low, while alkaline phosphatase levels are high. If Vitamin D deficiency occurs during the period of bone growth it is known as rickets. Q4. A 45 year old lady become unable to rise from a squatting position. She develops acne, truncal obesity, headaches and very thin skin with purpura. Fasting glucose levels are elevated. A CT scan shows a pituitary lesion. h). Cushing’s Disease. A pituitary tumour is secreting ACTH leading to excess cortisol and the typical features described above. Proximal myopathy can also occur as a result of iatrogenic/therapeutic steroid use given for respiratory or rheumatological disorders. Q5. A 40 year old patient describes weight loss and palpitations. Her family have commented that her eyes are very prominent. Examination shows pulse 98 bpm irregularly irregular, proximal myopathy, sweaty skin, and a palpable neck mass. c). Thyrotoxicosis. Excess thyroxine hormone causes eye signs, goitre, atrial fibrillation, weight loss, proximal myopathy. The symptoms of hypothyroidism are different and include cold intolerance, weight gain, bradycardia, coarse hair and hoarseness. Rheumatology EMQ 8 Causes of chrondocalcinosis Which of the following is the most appropriate course of action in each case? a) Start IV antibiotics immediately then aspirate the joint. b) Aspiration of joint to look for CPPD crystals, check serum calcium levels. c) Start oral methotrexate d) X – ray hands to look for CPPD deposition and ‘hook-like’ spurs at the MCP joints, check serum ferritin. e) Aspirate joints and perform synovial fluid microscopy to look for CPPD crystals, x-ray joints and check anti-CCP antibody. f) Start intra-muscular Gold injections g) Aspirate the joint and start IV antibiotics. If no growth on culture inject with steroid. h) A short course of tapering oral prednisolone followed by prophylactic low dose oral colchicine. i) Check serum magnesium and serum phosphate j) Perform full auto-antibody screen

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Q1. A 50 year old man has a swollen painful wrist. He also complains of excessive thirst and low mood. He has a history of renal stones. b). Aspiration of joint to look for CPPD crystals, check serum calcium levels. Q2. A 45 year old man is discovered to have abnormal liver function tests. He has bronze pigmentation of the skin. Fasting glucose levels are elevated and his MCP joints are swollen and painful. d). X-ray hands to look for CPPD deposition and ‘hook-like’ spurs at the MCP joints, check serum ferritin. The most likely diagnosis is haemachromatosis which is a recognised cause of chondrocalcinosis. Serum ferritin and transferrin saturation are useful investigations. In addition to the usual radiological features of CPPD, patients with haemachromatosis can also have degenerative changes at the MCP joints (‘hook-like’ spurs). Q3. A 50 year old man has swollen knees, elbows and wrists which have evolved over several weeks. Rheumatoid factor is mildly elevated. He complains also of stiffness and fatigue. e). Aspirate joints and perform synovial fluid microscopy to look for CPPD crystals, x-ray joints and check anti-CCP antibody. Rheumatoid and pseudogout can be confused as their presentations can be similar. The presence of CPPD crystals will confirm the diagnosis. Anti- CCP antibody is more specific for RA and should be negative in patients with CPPD even though the RF may be mildly false positive. X-rays should also help to differentiate between both conditions. Q4. A 64 year old lady presents with an acutely painful swollen wrist. She is apyrexic, but CRP and WCC are mildly elevated. g). Aspirate the joint and start IV antibiotics. If no growth on culture inject with steroid. It can be difficult to distinguish acute pseudogout from sepsis and if there is any doubt IV antibiotics should be given until the culture results are known. The presence of CPPD crystals on microscopy will confirm the diagnosis and a therapeutic steroid injection can be given. Q5. A 50 year old man suffers from repeated episodes of pseudogout affecting his knees, wrists and and MCP joints. Acute steroid injections are helpful, but often multiple joints are affected. f). A short course of tapering oral prednisolone followed by prophylactic low dose oral colchicine.

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Colchicine in known to interrupt attacks of pseudogout, however it must be used with care especially in the elderly as it can cause diarrhoea. Steroids should not be used in the long term, but can be helpful to settle an acute attack involving multiple joints

Editors: Ian Bickle & Paul Hamilton

Question Writers:

Cardiology by Navtej Chahal & Chris Lockhart Endocrinology by Fionnula Pollock Rheumatology by Elisabeth Ball Data interpretation by Ian Bickle