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
04/20/23 2Dr. Alka Stoelinga
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.
04/20/23 3Dr. Alka Stoelinga
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
04/20/23 4Dr. Alka Stoelinga
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
04/20/23 7Dr. Alka Stoelinga
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
04/20/23 8Dr. Alka Stoelinga
Rheumatoid arthritis
04/20/23 9Dr. Alka Stoelinga
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.
04/20/23 10Dr. Alka Stoelinga
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.
04/20/23 11Dr. Alka Stoelinga
HALLMARK OF RA
• Inflammatory synovitis• Presenting in symmetrical distribution
04/20/23 12Dr. Alka Stoelinga
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
04/20/23 13Dr. Alka Stoelinga
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)
04/20/23 14Dr. Alka Stoelinga
04/20/23 15Dr. Alka Stoelinga
Risk factors
– Female– Genetic susceptibility – HLA DR4, DR1, DW15– Monozygotic twins– I⁰ relatives– SE positive alleles (HLA DR β₁)– Cigarette smoking
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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
04/20/23 17Dr. Alka Stoelinga
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
04/20/23 18Dr. Alka Stoelinga
04/20/23 19Dr. Alka Stoelinga
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
04/20/23 21Dr. 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
04/20/23 22Dr. Alka Stoelinga
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
04/20/23 23Dr. Alka Stoelinga
04/20/23 24Dr. Alka Stoelinga
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)
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04/20/23 28Dr. Alka Stoelinga
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04/20/23 29Dr. Alka Stoelinga
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 ).
04/20/23 31Dr. Alka Stoelinga
04/20/23 32Dr. Alka Stoelinga
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)
04/20/23 33Dr. Alka Stoelinga
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
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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)
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TREATMENT4. Surgery
– Synovectomy– Osteotomy– Arthrodesis– Arthroplasties
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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
04/20/23 37Dr. Alka Stoelinga
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