Rheum Flashcards
SLE RFs
Women 9:1 - child bearing age
Genetic link
Symptoms of SLE
Remitting and relaxing of various course Constituitional Fever Malaise Weight loss Fatigue Myalgia CVS Chest pain - percarditis (synovitis) Stroke (antiphos?) Resp SOB, Chest pain - Pleuritis/ pleurisy (synovitis), pneumonitis. Chronic diffuse ILD, PE GI Abdo pain - peritonitis (synovitis) Oral/ nasal ulcers Derm Malar rash - butterfly sparing nasolabial folds Discoid rash - erythematous papules or plaques on head and neck with scaling, follicular keratosis, atrophic scarring and depigmentation Photosensitive rash Neuro Seizures Psychosis Depression Rheum Jacaud's arthropathy Synovitis of more than 2 jointys Morning stiffness Usually small joints of hand Less destructive/ disabling than RA Renal Renal disease proteinuria >0.5g/d Red cell casts Other Raynauds (30%) Antiphospholipid syndrome (see box 20-30%)] Sjrogren's 15-20% Autoimmune thyroid disease 5-10% Nail fold necrosis Lymphadenopathy Alopecia Retinal exudates Dx
Diagnosis of SLE
Blood Cells FBC ESR (high) Biochem LFTs (baseline), Ues (reanal ddx) CRP (normal!) - high would indicate infection or other rheum disease Immune Anti dsDNA - specific but only in 60% of cases -predicts nephritis Complement - decreased 3 +4 ANA 95% ENA - 20-30% RHf - 40% Antiphospholipid antibodies Other BP (baseline for steroids - RF for CVS disease) Urine for casts or protein
Management of SLE
Generally
Sun block
Hydroxycholoroquine (skin and joint)
Topical steroid
Screen for comorbidities (TFTs and Antiphos and Sjrogens)
Maintenance
NSAIDs (unless renal disease), Hydroxycholoquine (antimalarial) for skin and joint
Aza, MTX, mycophenolate as steroid sparing
Biologics - belimumab if high activity
Flares
Mild (no organ damage)- Steroids or hydroxychloroquine
Moderate flare (organ) - DMARDs or mycophenalate
Severe (life threatening) - high dose steroids, myco, rituximab or cyclophosphamid
Symptoms of antiphos syndrome
Associated with SLE (20-30%) Autoimmune Symptoms C-Clotting - cerebral and renal tendency L- Livedo reticularis Pink-blue mottling caused by cap dilatation and stasis in skin venules - can be physiological/ cold O - Obstetric - recurrent miscarriage T - Thrombocytopenia s Diag Antiphospholipid antibodies Lupus anticoagulant & Clotting
Treatment in antiphos syndrome
Anticoagulation
Epidemiology polymyalgia rheumatica
F>M
Age >59
Symptoms polymyalgia rheumatica
Proximal myopathy, bilateral aching and tenderness Morning stiffness >1hr General Fatigue Fever Weight loss Dep Anorexia Capel tunnel syndrome Mild polyarthritis tenosynovitis association NO weakness
Diagnosis
Bloods Cells ESR >40 Biochem CRP AlkP raised in 30% Creatine kinase (main but also and muscle enzymes (ALT AST LDH and aldolase) in plasma) normal Immune- only if for DDX
Clinical diag with inflammation on blood
Polymyositis symptoms
Muscles Progressive, insideous onset (linked with cancer to remember) of proximal muscle weakness. May affect other muscles causes Dysphonia (not dysphasia) Dyspnea Skin (dermatomyosis) Macular rash (scarf sign on neck and shoulders) Nail bed erythema Heliotrope - (Lilac-purple) rash on eye lids often with oedema Gottron's papules - pathognomic Knuckles +/- elbows and knees Extra-muscler Link with cancer!! (especially dermato (30%)) Paraneoplastic Fever Arthralgia Raynaud's Interstitial lung fibrosis Myocarditis Arrhythmias (muscles of CVS think)
Polymyositis diagnosis
Blood Cells Biochem CRP Creatine kinase and other muscle stuff e.g. LDH Immune ANA ENA (both can be associated) Imaging CXR (lung and heart) MRI (odema around muscle Other Muscle biopsy EMG - characteristic fibrillation potentials Full cancer screen
Treatment of polymyositis
Pred
DMARDs in resistant disease
Hydroxychloroquine/ topical tracoliumus for dermatomyositis
Scleroderma symptoms
C - Calcinosis
R - Raynauds
E - oesophageal dysmotility
S - Sclerodactyly
T- Tangiectasia
Widespread fibrosis of organs and microvasc abnormalities
Arthralgia, Renal failure, ILD, Pulmonary artery hypertension, ED
Diagnosis scleroderma
ANA common but anticentromere antibodies are the most specific
Treatment of scleroderma
Manage symptoms Sildenafil - ED Bosentan - Pulmonary artery hypertension DMARD or biologics ACEi to decrease chance of renal failure
Who gets scleroderma?
Women aged 20-50
Symptoms of Granulomatosis with polyangitis
Upper and lower resp tract Dysnpnoea, haemoptysis - pulmonary nodules/haemorrhage Epistaxis, sinusitis, nasal crusting Saddle-shaped nose deformity Vasculitis Headache Malaise Rash Proptosis Cranial nerve lesions Renal Rapidlyprogressive glomerulonephritis (75%) Nephritic syndrome Arthritis (60%)
Diagnosis of granulomatosis with polyangitis
Cavitating lung lesions
Immune
cANCA (more Wegerner’s)
pANCA (more Churg-Strauss)
