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SYSTEMIC CONNECTIVE TISSUE DISEASES

SYSTEMIC CONNECTIVE TISSUE DISEASES. DR CB NEL. INTRODUCTION. Multiple body systems involved Wide spectrum of clinical manifestations Aetiology is multifactorial. SYSTEMIC LUPUS ERYTHEMATOSUS. SLE. Predominantly females, 9:1 ratio Peak onset second and third decades

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SYSTEMIC CONNECTIVE TISSUE DISEASES

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  1. SYSTEMIC CONNECTIVE TISSUE DISEASES DR CB NEL

  2. INTRODUCTION • Multiple body systems involved • Wide spectrum of clinical manifestations • Aetiology is multifactorial

  3. SYSTEMIC LUPUS ERYTHEMATOSUS

  4. SLE • Predominantly females, 9:1 ratio • Peak onset second and third decades • More common in persons of Afro-Caribbean origin • Several autoantibodies associated with SLE

  5. CLINICAL FEATURES • Raynaud’s phenomenon • Colour changes of mainly the digits provoked by cold or emotion • White (vasoconstriction) • Blue (cyanosis) • Red (reactive hyperemia) • Secondary if associated SLE • Broad spectrum of causes (Talley and O’Connor) • Musculosketal • Mild morning stiffness • Migratory arthralgia • Small joint synovitis • Joint deformities are rare • Non-erosive x-ray changes

  6. RAYNAUD’S PHENOMENON

  7. CLINICAL FEATUES Cutaneous lesions Non-lupus specific Vasculitis Livedo reticularis Non-scarring alopecia Panniculitis • Lupus specific • Acute • Malar ”butterfly” rash • Generalized erythema • Subacute • Annular • Papulosquamous (psoriasiform) • Chronic • Discoid • Lupus profundus

  8. MALAR RASH

  9. SUBACUTE CUTANEOUS LUPUS

  10. DISCOID LUPUS

  11. CLINICAL FEATUES • Renal • Proteinuria, haematuria, casts on urine microscopy • Proliferative glomerulonephritis • Six classes of nephritis according to histology • Cardiopulmonary • Pleurisy • Pleural effusion • Interstitial lung disease • Lung fibrosis • Pericarditis • Myocarditis • Libman-Sacks endocarditis (non-infective vegetations)

  12. LIBMAN-SACKS ENDOCARDITIS

  13. CLINICAL FEATURES • Nervous system • Headaches • dysfunction • Visual hallucinations • Chorea • Psychosis • Seizures • Aseptic meningitis • Neuropathies • Transvers myelitis

  14. CLINICAL FEATURES • Secondary Antiphospholipid syndrome • Recurrent arterial and venous thromboses • Recurrent fetal losses • Thrombocytopenia • Antiphospholipid antibodies (lupus anticoagulant, β2 glycoprotein 1, anticadiolipin) • Life-long warfarin therapy required • Non-specific • Lymphadenopathy • Fever • Weight loss • Fatigue

  15. SPECIAL INVESTIGATION • FBC • Haemolytic anaemia (Coombs positive) • Thrombocytopenia • Lymphopenia • Neutropenia • Kidney function • dipstix (proteinuria, haematuria) • Microscopy for active sediment (red cell, white cell and hyaline casts) • U&E can still be normal in advanced disease • Urine protein/Creatinine ratio • 24hrs urine protein

  16. SPECIAL INVESTIGATION • ESR and CPR • ESR elevated in active disease • CRP often normal in active disease • Autoantibodies • ANA (high sensitivity, low specificity) • Anti-double-stranded DNA (ds-DNA) specific for SLE • Anti-Smith (anti-Sm) specific for SLE • Antiphospholipid antibodies

  17. LUPUS CLASSIFICATION CRITERIA Need four of the eleven criteria for diagnosis

  18. MANAGEMENT • NO curative treatment available • Treat symptoms • Treat complications/Life threatening disease aggressively with immunosuppressants

  19. SYSTEMIC SCLEROSIS

  20. Peak onset fourth and fifth decade • 4:1 female predominance • Divided into • Diffuse cutaneous systemic sclerosis (DCSS) • Limited cutaneous systemic sclerosis (LCSS) • “CREST” syndrome (many patients with LCSS) • Calcinosis • Raynaud’s • Oesophageal dysfunction • Sclerodactyly • Telangiectasia • Aetiology of systemic sclerosis is unknown

  21. CLINICAL FEATURES • Cutaneous • Raynaud’s early in disease • Sclerodactyly(skin tight, shiny, and thickened) • Calcinosis (subcutaneous calcium deposits) • Thinning and radial furrowing of the lips • Telangiectasia • In LCSS skin involvement distal to knees and elbows and include the face • In DCSS skin involve proximal to knees and elbows and include the trunk

  22. FACE IN DIFFUSE SCLEROMERMA

  23. HANDS IN SCLERODERMA

  24. CLINICAL FEATURES • Musculoskeletal • Arthralgia, • Morning stiffness • Flexor tenosinivits • Decreased hand movement due to skin rather the joints

  25. CLINICAL FEATURES • Gastro-intestinal features • Lower oesophagus (smooth muscle atrophy, fibrosis) • Acid reflux • Dysphagia • Barrett’s esophagitis • Carcinoma • Stomach • Early satiety • Outlet obstruction

  26. CLINICAL FEATURES • Gastro-intestinal features • Small intestine • Malabsorption due to bacterial overgrowth • Bloating and pain • Large bowl • Dilatation with pseudo-obstruction • Rectal incontinence

  27. CLINICAL FEATURES • Cardiorespiratory features • Pulmonary involvement major cause of mortality • Pulmonary fibrosis mainly in diffuse disease • Pulmonary hypertension mainly in limited systemic sclerosis

  28. CLINICAL FEATURES • Renal features • Hypertensive renal crisis (diffuse disease) • Can be precipitated by corticosteroids • Malignant hypertension • Renal failure • Death • Treatment with ACE-inhibitors

  29. INVESTIGATIONS • ANA positive • Anti-topoisomerase I antibodies in diffuse disease • Anti-centromere antibodies in limited disease • Antibodies not in all patient • Still mainly a clinical diagnosis

  30. MANAGEMENT • Raynaud’s • Avoid cold, smoking, vasoconstrictors e.g.. B-blockers • Keep the whole body warm • Vasodilators e.g.. Ca-channel blockers (nifedipine), Angiotensin II receptor antagonists (losartan) • PPI in oesophageal involvement • Pulmonary hypertension • Vasodilators • Prostaglandin analogues • 5-phosphodiesterase inhibitors e.g.. Viagra • Heart-lung transplants

  31. MANAGEMENT • Interstitial lung disease • High doses corticosteroids • Immunosuppressants e.g.. Cyclophosphamide • Skin • Moisturizing creams, emulsifying ointments • Aggressive treatment of ulcers • Treat the cause • Prevent secondary infections

  32. POLYMYOSITISAND DERMATOMYOSITIS

  33. POLYMYOSITIS AND DERMATOMYOSITIS • Rare • 40-60yrs of age at onset • Possible paraneoplastic manifestation dermatomyositis > polymyositis

  34. CAUSES OF PROXIMAL MUSCLE WEAKNESS • Inflammatory • Polymyositis • Dermatomyositis • Endocrine • Hypo/hyperthyroidism • Cushing’s syndrome • Addison’s disease • Genetic • Muscular dystrophies • Drugs/toxins • Corticosteroids • Alcohol • Statins • Fibrates • Infections • HIV • Cytomegalovirus • schistosomiasis

  35. CAUSES OF PROXIMAL MUSCLE WEAKNESS • Metabolic • Vit D deficiency • Hypocalcaemia • Hypokalaemia • Uraemia • Hepatic failure • Rheumatological • RA • SLE • Scleroderma

  36. CLINICAL FEATURES • Polymyositis • Symmetrical proximal weakness • Lower limbs mostly first • Difficulty in raising from a chair • Difficulty in climbing stairs • Insidious onset over weeks • Systemic features (fever, fatigue, weight loss) common • Respiratory muscle involvement is life-threatening

  37. CLINICAL FEATURES • Dermatomyositis • Proximal muscle weakness + typical skin manifestations • Skin manifestations: • Gottron’s nodules and plaques • Heliotrope rash (over eye lids) • Peri-orbital oedema • V-sign (erythematous rash, anterior neck and thorax) • Shawl-sign (erythematous rash, shoulders0

  38. GOTTRON’S

  39. HELIOTROPE RASH AND PERI-ORBITAL OEDEMA

  40. SHAWL SIGN

  41. V-SIGN

  42. INVESTIGATIONS • Raised Total-CK • EMG – confirm myositis and exclude neuropathy • Muscle biopsy – identify type of myositis • MRI- identify areas of abnormal muscle

  43. MANAGEMENT • Initially high doses of corticosteroids (1mg/kg/day) • Taper according to response • Sometimes more potent immunosuppressant needed e.g.. Azathioprine, methotrexate

  44. MIXED CONNECTIVE TISSUE DISEASE • A SPECIFIC ENTITY • Features of • RA • SLE • Scleroderma • Polymyositis • Serology: positive anti-RNP (other serology negative)

  45. SJӦGREN’S SYNDROME • Lymphocytic infiltration of salivary and lachrymal glands • Glandular fibrosis and exocrine failure • Primary or secondary • Dry eyes, dry mouth, vaginal dryness, dry cough, dry skin • Fatigue, non-erosive arthritis, Raynaud,s • 40-Time increase risk for lymphoma • Anti-Ro(SS-A) and anti-LA(SS-B) anti-bodies • Symptomatic treatment

  46. DRY MOUTH IN pSS

  47. BILAT PAROTID GLAND ENLARGEMENT IN pSS

  48. QUESTIONS?

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