SYSTEMIC CONNECTIVE TISSUE DISEASES
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Transcript of SYSTEMIC CONNECTIVE TISSUE DISEASES
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SYSTEMIC CONNECTIVE TISSUE DISEASES
DR CB NEL
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INTRODUCTION
• Multiple body systems involved
• Wide spectrum of clinical manifestations
• Aetiology is multifactorial
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SYSTEMIC LUPUS ERYTHEMATOSUS
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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
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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
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RAYNAUD’S PHENOMENON
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CLINICAL FEATUES
Cutaneous lesions• Lupus specific
– Acute• Malar ”butterfly” rash• Generalized erythema
– Subacute • Annular• Papulosquamous (psoriasiform)
– Chronic• Discoid• Lupus profundus
• Non-lupus specific– Vasculitis– Livedo reticularis– Non-scarring alopecia– Panniculitis
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MALAR RASH
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SUBACUTE CUTANEOUS LUPUS
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DISCOID LUPUS
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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)
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LIBMAN-SACKS ENDOCARDITIS
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CLINICAL FEATURES
• Nervous system– Headaches– dysfunction– Visual hallucinations– Chorea– Psychosis– Seizures– Aseptic meningitis– Neuropathies – Transvers myelitis
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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
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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
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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
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LUPUS CLASSIFICATION CRITERIA
Need four of the eleven criteria for diagnosis
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MANAGEMENT
• NO curative treatment available
• Treat symptoms
• Treat complications/Life threatening disease aggressively with immunosuppressants
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SYSTEMIC SCLEROSIS
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• 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
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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
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FACE IN DIFFUSE SCLEROMERMA
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HANDS IN SCLERODERMA
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CLINICAL FEATURES
• Musculoskeletal – Arthralgia,– Morning stiffness – Flexor tenosinivits – Decreased hand movement due to skin rather the joints
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CLINICAL FEATURES
• Gastro-intestinal features– Lower oesophagus (smooth muscle atrophy, fibrosis)
• Acid reflux• Dysphagia• Barrett’s esophagitis• Carcinoma
– Stomach• Early satiety• Outlet obstruction
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CLINICAL FEATURES
• Gastro-intestinal features– Small intestine
• Malabsorption due to bacterial overgrowth• Bloating and pain
– Large bowl• Dilatation with pseudo-obstruction• Rectal incontinence
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CLINICAL FEATURES
• Cardiorespiratory features– Pulmonary involvement major cause of mortality– Pulmonary fibrosis mainly in diffuse disease– Pulmonary hypertension mainly in limited systemic
sclerosis
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CLINICAL FEATURES
• Renal features– Hypertensive renal crisis (diffuse disease)• Can be precipitated by corticosteroids• Malignant hypertension• Renal failure• Death• Treatment with ACE-inhibitors
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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
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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
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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
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POLYMYOSITISAND
DERMATOMYOSITIS
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POLYMYOSITIS AND DERMATOMYOSITIS
• Rare• 40-60yrs of age at onset• Possible paraneoplastic manifestation
dermatomyositis > polymyositis
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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
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CAUSES OF PROXIMAL MUSCLE WEAKNESS
• Metabolic– Vit D deficiency– Hypocalcaemia– Hypokalaemia– Uraemia– Hepatic failure
• Rheumatological– RA – SLE– Scleroderma
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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
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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
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GOTTRON’S
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HELIOTROPE RASH AND PERI-ORBITAL OEDEMA
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SHAWL SIGN
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V-SIGN
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INVESTIGATIONS
• Raised Total-CK • EMG – confirm myositis and exclude neuropathy• Muscle biopsy – identify type of myositis• MRI- identify areas of abnormal muscle
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MANAGEMENT
• Initially high doses of corticosteroids (1mg/kg/day)• Taper according to response• Sometimes more potent immunosuppressant needed
e.g.. Azathioprine, methotrexate
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MIXED CONNECTIVE TISSUE DISEASE
• A SPECIFIC ENTITY• Features of– RA– SLE– Scleroderma– Polymyositis
• Serology: positive anti-RNP (other serology negative)
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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
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DRY MOUTH IN pSS
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BILAT PAROTID GLAND ENLARGEMENT IN pSS
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QUESTIONS?