many faces of systemic lupus erythematosusbsmedicine.org/congress/2016_1/prof._md._titu_miah.pdf ·...
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Many Faces of Systemic Lupus Erythematosus
Prof. Md. Titu Miah Professor of Medicine Dhaka Medical College & Hospital
” “ The Very Appearance
of SLE might be DECEIVING!
NO TWO LUPUS PATIENTS HAVE EXACTLY THE SAME MANIFESTATIONS AND ONE PERSON DOES NOT USUALLY HAVE ALL THE SYMPTOMS.
.
So a VERY HIGH INDEX OF CLINICAL SUSPICION should be there during diagnosis of
SLE
SLE might present as “Catch Me if U Can!”
Movie: Catch Me If u Can Actor: Leonardo de Caprio Main Role: Con Artist Substitue Role: Lawyer, Doctor , Pilot, Detective
Epidemiology
In Asia: Prevalence rates 30—50/100,000 population. Incidence rates 0.9/100,000 to 3.1% per annum. In U.S.A: Incidence: 5 cases per 100,000 population. Race, sex and age-related demographics: The prevalence of SLE is highest in women aged 14 to 64 years. Black women have a higher rate of SLE followed by Asian women and then White women.
Clinical presentation among the SLE patients can be Diverse, Highly
variable !
Ranging from INDOLENT to FULMINANT
Prevalance of SLE Worldwide Vs DMCH
85 100
90 85
50
20
35
25
45
15
60
70
90
70 70
10
25
40
30
5
0
10
20
30
40
50
60
70
80
90
100
Worldwide DMCH
Number of SLE patients in DMCH 100 in 2015
In SLE clinic 60
In Nephrology 28
In Dermatology 5
In Pediatrics 8
A prospective study was done from January 2002 to December 2006 in Mymensingh Medical College and Hospital.
Number of SLE patients : 33 Objective: To observe the clinical profile
and outcome of the patients
Constitutional
Fatigue, the most common constitutional symptom associated with SLE.
Can be due to active SLE, medications, lifestyle habits, or concomitant fibromyalgia or affective disorders. Fever may reflect active SLE, infection, and reactions to medications (drug fever)
Musculoskeletal
Joint pain is the most common clinical presentation.
In contrast to Rheumatoid Arthritis SLE arthritis may be Asymmetrical
Pain is disproportionate to swelling.
Increased Prevalence of Avascular necrosis in the patients with SLE.
SLE RA
Musculoskeletal cont.
A 32yrs Lady with SLE since 1998 was on hydroxychloroquine and steroid.
4 yr later she develop AVN
SLE Vs Overlap Syndrome O
verla
p sy
ndro
me
SLE
Systemic sclerosis
Myositis
SLE
MYOSITIS
SYSTEMIC SCLEROSIS
Central Nervous System
Patients with SLE can have several Neuropsychiatric symptoms represent a subcategory termed NPSLE
According to 10 high quality prospective studies including 2049 SLE patients the prevalence of NPSLE manifestations among them was 56%, were CNS manifestations were 90%.
Brey RL, Holliday SL, Saklad AR, Navarrete MG, Hermosillo-Romo D, Stallworth CL. Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology. 2002; 58:1214–20.
Neuropsychiatric Lupus
Central Nervous System 1. Headache 2. Seizure disorders 3. Cerebrovascular disease 4. Demyelinating syndrome 5. Myelopathy 6. Movement disorder
7. Aseptic meningitis 8. Cognitive dysfunction 9. Mood disorder 10.Anxiety disorder 11.Psychosis 12.Acute confusional state
Neuropsychiatric Lupus
Peripheral nervous system:
1. Mononeuropathy 2. Polyneuropathy 3. Cranial neuropathy 4. Acute inflammatory demyelinating polyradiculoneuropathy (GBS) 5. Plexopathy 6. Autonomic disorder 7. Myasthenia gravis
CBC: Hb% 9.1 g/dl WBC: 6580/cmm Platelet: 27,8000/cmm ESR: 86 mm in 1st hr ANA: Positive Anti ds DNA: Positive
Furtther investigations
Final Diagnosis
CNS LUPUS
A young normotensive nondiabetic patient presented with left sided hemiperesis
Further investigations Lipid profile: normal ANA: positive Anti ds DNA: positive Anti phospholipid Ab: positive Patients with lupus had higher
risk for all stroke subtypes except in subarachnoid
hemorrhage
• Mrs Y 23yrs old was presented with -Convulsion for 3 days. -Pain in multiple joints for4months. -Fever for 6 months
She had history of hallucination.
O/E PlantarResponse: Extensor bilaterally
Hb: 7.92 g/dL WBC: 2.50 x10^9/L ESR: 88 mm in 1st hour ANA screening: +ve (42.5 U/mL) Anti-dsDNA: 145.0 IU/ml
Furtther investigations
Diagnosis
CNS LUPUS
Pulmonary
SLE may lead to multiple pulmonary
complications such as pleurisy, pleural effusion, DPLD, pneumonitis, pulmonary hypertension.
Hemoptysis may herald diffuse alveolar hemorrhage, a rare, acute,life-threatening pulmonary complication of SLE.
Pulmonary cont.
Mrs x 65 yrs old was admitted with the complaints of- Fever for one month Chronic dry cough for last 15
days O/E: Anemia: Present Lung: fine basal crepitation
Chest Xray
Pulmonary cont.
CBC: • Hb:11.0 g/dl • WBC:9000 • Platelet:335000 • ESR:101 mm in 1st hr MT:negative Urine R/M/E: • Rbc: 1-2/HPF • PC:2-3/HPF • Albumin:Trace
Pulmonary cont.
Mrs. X, presented with - fever for 1 month -cough for 1 month.
Clinically she had features of consolidation. She had non resolving pneumonia for 3 month. All other relevant investigations were normal apart from neutropenia and then her ANA and Anti Ds DNA revealed high titre subsequently she was diagnosed as a case of SLE.
Skin Changes in SLE
Malar Rash Rash In Trunk Or Extremities Urticaria Bullae Maculopapular Lesions Ulcerations Raynauds
Male presented with -Fever for 15 days -Erythomatous rash involving chest for same duration
ANA: NEGATIVE ANTI DS DNA: NEGATIVE ALL OTHER INVESTIGATIONS REVEALED NORMAL.
Subsequently it was diagnosed as case of SLE on histopathology which showed liquefactive degeneration of basal layer of epidermis.
6 month later he was diagnosed as acase of DPLD with SLE
Mr. X 82 yrs old was admitted with the complaints of Multiple painless
nodular leison all over his body for 4 month.
O/E: • Anemia : + • Lymphnode : generalized
lymphadenopathy. • P/A/E: no organo megaly • USG of W/A: Fatty change of
liver • Urine R/M/E: Normal
Hb:11.4gm/dl ESR:35 WBC:4000/UL Platelet:79,000 Atypical cell: 10% PBF: Leucoerythroblastic blood picture with marked thrombocytopenia
SKIN cont.
SKIN cont.
Investigations S. ALT:45 U/L, S. AST:90 U/L CBC: Hb-10gm/dl WBC10.6×10³/mm ESR: 100 mm in 1st hour Dengue NS1 antigen : negative.
A 23years female medical student of DMC presented with • High grade continued fever for 6
days • Headache for same duration
O/E: • Temp:102 F • Bp:80/60 • Pulse:114/min • Anemia:+
After treating with antibiotic no remission of fever. subsequently she develop lymphadenopathy involving ant and post cervical chain,left supra clavicle and both inguinal region.
.
FNAC of lymphnode: Focal aggregation of epitheloid cells. Features suggestive of granulomatous inflammation.
Biopsy of Lymph node: Acute necrotizing lymphadenopathy (Kikuchi’s disease)
.
DIAGNOSIS ?
• Several authors reported association between SLE and Kikuchi disease
• Kikuchi disease has been diagnosed before,during, and after diagnosis of SLE was made in same patients.
• Histological appearance of lymphnodes of both disease are similar.
• Kikuchi disease may represent a forme fruste SLE.
Further investigations: ANA: Strongly positive Speckled variety Anti Ds DNA: positive
Gastrointestinal
Occasional abdominal pain in active SLE may be directly related to active lupus
-including peritonitis, pancreatitis, mesenteric vasculitis, and bowel infarction. Jaundice due to autoimmune hepatobilliary disease
Miss Joba,13 Years of age, was admitted with the complaints of
1) Swelling of whole body for 3weeks. 2) Rashes over whole body – same duration. 3) H/0 Burst Abdomen with peritonitis followed by appendectomy 3 wks back.
Gastrointestinal
Gastrointestinal
O/E: • Anemia : ++ • Odema : +++ • Multiple purpuric, non palpable, non tender
rashes on the back of the body. Excoriating lesions over abdomen and both upperlimb
P/A/E: • wound dehiscence present • Ascites and Hepatospleenomegaly • Vulval Swelling
Gastrointestinal
• Hb:12.5 g/dl • ESR :105mm/1st hr • TC: 12.5×109 • Platelet :70×109
Urine R/M/E • RBC: Plenty • Pus cell: 4-5 • Protien:+++ Anti Ds DNA :Positive ANA :Positive 24 hrs UTP:29.91 g/24hrs
D- Dimer > 4.00mg/ml(↑) FDP > 120 ug/ml(↑) CRP 7.45mg/L
USG of W/A : 1) Hepatosplenomegaly. 2) Suggestive of acute renal parenchymal disease. 3) Bilateral mild pleural effusion. 4) Huge ascites.
Gastrointestinal
Final Diagnosis
SLE with Lupus Nephritis with septicemia with wound dehiscence following appendectomy
A young women presented with sudden severe Abdominal Pain.
There is diffuse circumferential wall thickening with diffuse oedema involving entire small intestine resulting in double halo or target sign
Further investigations
Lupus Mesentric Vasculitis
• ANA: Positive • Anti Phospholipid Ab : Positive • Anti ds DNA: Positive
Diagnosis
Gastrointestinal
A lady of 33 year presented with: Sudden onset abdominal distension for 7 days. Abdominal discomfort . Respiratory distress.
On examination • She was mildly icteric • Shifting dullness +ve • Splenomegaly
USG shows hepatic vein thrombosis, with arrow pointing to the thrombus
Diagnosis
Budd Chiari Syndrome Resulting From Hyper Viscosity Caused By Anti Phospholipid Syndrome Secondary To SLE
• ANA: Positive • Anti Phospholipid Antibody:
Positive • Anti ds DNA: Positive
Further investigations
SLE during Pregnancy
Fertility When and how to time
pregnancy Obstetric issues -Pre eclampsia -Lupus nephritis -Thrombosis
• Prevalence of Pre eclampsia is 13% in SLE Vs 6-8% in normal condition.
• Risk factor include -pre-existing hypertension, -nephritis and -presence of antiphospholipid antibodies (APL)
SLE in CHILDREN: Malar rash, ulcers, mucocutaneous involvement, proteinuria,urinary cast, seizures, haemolytic anemia, thrombocytopenia, fever and lymphadenopathy are more commonly in childhood onset SLE
A female patient presented with -respiratory distress for one month -palpitation for same duration O/E: Irregularly irregular pulse lungs: basal crepitation ECG: AF with first ventricular rate T3: Raised ; T4: raised; CRP: Raised
Diagnosed as a case of Autoiimune thyroidits with myocarditis
Association with other Autoimmune disease?
After 3 month further investigations revealed: ANA: Positive Anti Ds DNA: Positive
Take Home Message
Even in difficult situation to diagnose SLE, most physicians need
high index of suspicion, special intuition, obsession to finally bring a differential diagnosis in appropiate clinical scenario in every discipline. So no matter whatever might be the faces or presentation SLE should be
Red handed