sjogren’s syndrome 10/21/20151dr. alka stoelinga

SJOGREN’S SYNDROME

04/20/23 1Dr. Alka Stoelinga

SJOGREN’S SYNDROME

• Autoimmune disorder of unknown etiology• Occurs as a result of chronic dysfunction of

exocrine glands and is characterized by:– Dryness of mouth, eyes, and other areas covered

by mucus membranes

• Onset:40-50 years• F:M=9:1


Clinical features: Salivary gland and lacrimal glands are mainly affected1. Salavary gland enlargement• Decrease salivary secretion—Dryness of mouth i.e Xerostomia

leading to difficulty in speaking and swallowing, and to severe dental caries. There may be loss of taste and smell

2.Ocular:• Decrease tear production• Keratoconjunctivitis sicca– Results from inadequate tear

production caused by lymphocyte and plasma cell infitration of lacrimal glands

3.Pancreatitis,Pleuritis,neuropsychiatric dysfunction and vasculitis may be present.

4.Renal tubular acidosis and chronic interestitial nephritis may also occur.


Revised Classification Criteria for Sjögren's Syndrome• SS diagnosis requires the presence of signs, symptoms, and lab findings.• Patient-reported symptoms must include

1. ocular symptoms, such as daily, persistent, troublesome dry eyes for more than 3 months, and

2. oral symptoms, such as needing to drink water to swallow food.• Signs

– Positive Schirmer's test • Histopathology studies should show

– Focal lymphocytic proliferation (Sialadenitis)– Ultrasound examinations The parenchyma of the gland demonstrates

multiple, small-2-6 mm hypoechoic lesions (lymphocytic infiltrates). Sialectasis with calculi are demonstrated if the disease is advanced

– Parotid sialography, or salivary scintigraphy (Widespread puddling of the injected contrast scattered throughout the gland)

– Autoantibodies against Ro (SSA) and/or La (SSB) antigens


Investigations:

1. Autoantibodies:• ANA• Rheumatoid Factor• Anti-Ro, Anti-La2. Schirmer’s test• To determine/demonstrate decrease tear

production3. Raised ESR4. Salivary gland biopsy04/20/23 5Dr. Alka Stoelinga

Management:

1.General• Artificial tear drop• Oral gel for soothing effect2.Steroid • Prednisolone3. Immunosuppressive• Methotrexate • Azathioprine04/20/23 6Dr. Alka Stoelinga

INFLAMMATORY JOINT DISEASES

• Rheumatoid arthritis• Seronegative spondarthritis• Crystal-associated disease• Joint infection• Juvenile idiopathic arthritis


Look and Evaluate first for1. Distribution of joint involvement

– Four basic patterns• Polyarticular symmetric – RA, SLE• Monoarticular arthritis – OA, Gout, Septic arthritis• Oligoarticular asymmetric- Spondyloarthropathis- AS• Migratory arthropathy- RF, Gonococcal arthritis, Lyme disease

2. Acute or Chronic– Acute -Crystal induced arthritis (Gout, pseudogout), Septic arthritis– Chronic -OA

3. Evidence of systemic Manifestations– SLE – Skin, Lungs, Kidney, CNS, Hematologic manifestations– Systemic sclerosis- Skin+ Raynaud’s phenomenon– Sjögren's syndrome- Keratoconjunctivitis sicca+ Parotid enlargement– Wegener’s granulomatosis - Upper and lower RTI+ Renal involvement– OA- No systemic symptoms

4. Evidence of Inflammation– Joint inflammation- RA– No joint inflammation- OA, Osteoporosis

5. Findings – Morning stiffness >1 hr– Red, warm, erythematous joints– Elevated ESR


Rheumatoid arthritis


Case

• A 30 year old woman presents with 3 week h/o joint swelling and stiffness. She points to her PIPs and MCPs and wrists. She informs that the stiffness lasts for around 2 hrs every morning and symptoms improves as the day progresses. She denies back pain/ stiffness. She has fatigue and low grade fever, Swollen and reddened MCPs and PIPs, DIPs wnl. Fluid in wrist joints. Other examinations are wnl.


Rheumatoid arthritis • Chronic inflammatory multisystem disease with

main target being synovium.• Non suppurative, proliferative inflammation of

synovium• Progresses to destruction of the articular

cartilage• Cause bone erosions• Deform joints• Ankylosis ( stiffness ) of the joints.


HALLMARK OF RA

• Inflammatory synovitis• Presenting in symmetrical distribution


Diagnostic criteria for rheumatoid arthritis

Four of the following criteria must be met:– Morning stiffness for > 1 hour everyday for 6 weeks– Swelling of the wrists, MCPs, PIPs for 6 weeks– Swelling of 3 joints for 6 weeks– Symmetric joint swelling for 6 weeks– Joint erosions on X-Ray– RF positive– Rheumatoid nodules


NEWER CRITERIA2010 ACR / EULAR Rheumatoid Arthritis Classification Criteria• The American College of Rheumatology (ACR) and the European League

Against Rheumatism (EULAR) establish a point value between 0 and 10. • Every patient with a point total of 6 or higher is unequivocally classified

as an RA patient– Provided S/he has synovitis in at least one joint and– That there is no other diagnosis better explaining the synovitis.

Four areas are covered in the diagnosis:1. Joint involvement – depending on the type and number of joints: up to

5 points2. Serological parameters – including the rheumatoid factors as well as

ACPA (Anti- citrullinated protein antibody): up to 3 points depending on titre level

3. Duration of arthritis: 1 point for symptoms lasting six weeks or longer4. Acute phase reactants: 1 point for elevated ESR, or elevated CRP value

(c-reactive protein)


Risk factors

– Female– Genetic susceptibility – HLA DR4, DR1, DW15– Monozygotic twins– I⁰ relatives– SE positive alleles (HLA DR β₁)– Cigarette smoking


Pathogenesis – RA is an autoimmune disease triggered by exposure

of a genetically susceptible hostAutoimmune reaction

Activation of CD4 +T cells and lymphocytes and the release of inflammatory mediators and cytokines

Produce

stimulates

Synovial cells

Produce

Various mediators of inflammation (PG ) and Matrix metalloproteinase (cartilage destruction)

Produce by macrophages and synovial lining cells that are activated by the T – cells in the joints

Osteoclasts

Bone destruction

Progressive joint damage

Activates

Promotes


Synovium ( hyperplastic )

Synovium rich in Inflammatory cells becomes adherent and grows over the articular surface , forming a Pannus

- an inflammatory exdudate overlying the synovial cells on the inside of a joint

stimulates

Release of IL-1 , platelet – derived growth factor Prostaglandin

Cause cartilage destruction and bone erosion


Clinical Features1. Slow and Insidious onset

– Joint pain, Stiffness, Symmetrical swelling of peripheral joints– Initially pain on movement of joint- later – rest pain– Florid morning stiffness– Polyarthritis- First affecting the small joints of fingers and toes;

followed by wrists, elbows, shoulders, knees, ankles, subtalar and midtarsal joints

– Swelling of PIP joints Spindle shaped fingers– Swelling of MTP joints Broadening of forefoot

2. Acute Onset– Acute Polyarthritis with severe systemic symptoms

3. Systemic Onset– Mild fever– Malaise– Fatigue– Weight loss

4. Palindromic onset– Recurrent acute episodes of joint pain and stiffness lasting for few hrs

to days04/20/23 20Dr. Alka Stoelinga

Clinical Features

Damage to ligaments and tendons:– Radial deviation of wrists and Ulnar deviation of digits (due

to rupture of the collateral ligaments at the MCP joints ) – Swan-neck deformity ( due to rupture of the volar plate of

the PIP joints ) and – Boutonniere deformity (due to rupture of central extensors)

of the fingers – Z- deformity of thumbs– Forefoot- Clawing of toes


Clinical Features1. Systemic• Fever• Malaise• Fatigue• Weight loss

4. Lymphatic• Splenomegaly• Felty’s syndrome

7. Ocular• Scleritis, Episcleritis• Scleromalacia performs• Keratoconjunctivitis

sicca

2. Musculoskeletal• Muscle wasting• Tenosynovitis; Bursitis• Osteoporosis

5. Pulmonary• Pleurisy, Effusions• Nodules in lungs and

Pleura• Fibrosing alveolitis

8. Vasculitis• Digital Ischemia• Leg Ulcers• Pyoderma gangrenosum• Mesenteric ischemia

3. Hematological• Anemia• Thrombocytosis• Eosinophilia• Felty’s syndrome (RA+

Neutropenia+ Splenomegaly)

6. Cardiac• Pericarditis, Myocarditis,

Endocarditis• Nodules in Pericardium• Pericardial Effusion

9. Neurological• Peripheral neuropathy• Spinal cord compression

(Cervical)• Mononeuritic Multiplex• Carpal Tunnel Syndrome

10. Rheumatoid nodules 11. Amyloidosis


NOTE

• Rheumatoid nodules– Initial event caused by vasculitis– Common Sites: Olecranon, Occiput, Achilles tendon

• Felty’s syndrome (RA+ Neutropenia+ Splenomegaly)• Caplan syndrome (RA+ Pulmonary involvement)


.


INVESTIGATIONSIMAGING1. X-rays of Hands and Feet

– Early Stage: No change– Juxta-articular osteopenia, soft tissue swelling and loss of joint space– Advanced Stage: Bony erosions and subluxation

2. High-frequency transducers - Can depict 20% more erosions than conventional radiography

3. Color Doppler and Power Doppler ultrasound- Show vascular signals of active synovitis depending on the degree of inflammation; are useful in assessing synovial inflammation

BLOOD TESTS:1. Immunological tests

– Anti-citrullinated protein antibodies (ACPAs)- Anti-CCP/ Anti- MCV assays– Serological point-of-care test (POCT)– RF

2. OTHER TESTS:– ANA, ESR, CRP : usually raised but may be normal. – CBC- Normochromic, Normocytic anemia and reactive thrombocytosis.– Ferritin levels- Raised ferritin but low serum iron concentration and total iron

binding capacity. – LFT- mild elevation of alkaline phosphatase– Uric acid/synovial fluid analysis: excludes polyarticular gout. – Urinalysis: microscopic hematuria/proteinuria may suggest connective tissue

disease. 04/20/23 30Dr. Alka Stoelinga

XRAY– Show soft tissue swelling, – periarticular osteopenia – loss of joint space – erosions– Deformity– Radial deviation of the wrist– Ulnar deviation of the fingers– Swan neck deformity ( flexon –

hyperextension deformity ).


DIAGNOSIS

• Based upon Clinical criteria• 2010 ACR / EULAR Rheumatoid Arthritis

Classification Criteria >/= 6• Anti-CCP (Positive in 67% of RA cases; but are

rarely positive if RA is not present- • Specificity- 95%)• Anti MCV• POCT (RF+ Anti MCV) for early diagnosis of RA (72%

Sensitive; 99.7% Specificity)


TREATMENT

The goal of treatment is:• Alleviating the current symptoms• Preventing the future destruction of the joints

with the resulting handicap if the disease is left unchecked

04/20/23 Dr. Alka Stoelinga 34

TREATMENT1. Disease modifying anti-rheumatic drugs (DMARDs)

– reduces the rate of damage to bone and cartilage– Methotrexate (MTX)– Cyclosporine– Sulfasalazine– Azathioprine– D-Penicillamine– Hydroxychloroquine

2. Combi-therapy of DMARDs– Methotrexate – Hydroxychloroquine– Methotrexate – sulfasalazine– Sulfasalazine – Hydroxychloroquine– Methotrexate – Hydroxychloroquine – sulfasalazine

3. Anti-inflammatory agents and analgesics– Glucocorticoids (Prednisolone, Methylprednisolone)– NSAIDS (Acetaminophen, Ibuprofen, Naproxen, meloxicam, etodolac,

nabumetone, sulindac, tolementin, choline magnesium salicylate, diclofenac, diflusinal, indomethicin, Ketoprofen, Oxaprozin and piroxicam)


TREATMENT4. Surgery

– Synovectomy– Osteotomy– Arthrodesis– Arthroplasties


Prognosis

• Average life span is reduced by 8-15 years• About 50%- 5 year survival rate with severe disease• 50% disabled/ unable to work within 10 years • Poorer prognosis is associated with:

– Insidious onset – Extra-articular manifestations – Functional disability at 1 year after start of disease – High RF titres – HLA-DR4 present – X-Ray evidence of erosions within 3 years


sjogren’s syndrome 10/21/20151dr. alka stoelinga

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