Sarcoidosis in 6 minutes and 40 seconds Matt Dickson Definition • Chronic granulomatous disorder • Multiorgan involvement • First case described by Sir Jonathan Hutchinson 1878 Epidemiology • Incidence of 5-10 per 100,000 • Bimodal age distribution • Highest incidence: • • • • Northern Europe (Scandinavia) Irish African Americans West Indians Aetiology • Unknown • HLA-DRB1*1101 associated with disease susceptibility • HLA-DRB1*0301 associated with acute and remitting disease Pathophysiology Pathophysiology • ACE levels • Lymphopenia • Delayed type hypersensitivity reactions Presentation • Variable – acute vs chronic • Thoracic involvement ~90% • Respiratory symptoms • Constitutional symptoms • Lofgren's syndrome: Fever Bilateral Hilar Lymphadenopathy Erythema nodosum Arthralgia • Asymptomatic Radiological appearances Radiological appearances • HRCT Other investigations • FBC – anaemia, leucopenia • Hypercalaemia • Immunoglobulins • ACE • ECG/Echo • PFTs • Bronchoscopy • EBUS Management • Treatment not recommended for : • Asymptomatic stage I disease • Asymptomatic stage II or III disease with mildly abnormal lung function and stable disease • Oral corticosteroids first line • 0.5mg/kg/day prednisolone for ~ 4 weeks, reduce to maintenance dose (5-20mg OD) for period of 6 months to 2 years • ICS not of significant benefit • In treatment failure/life threatening – pulsed IVMP Management • Second-line agents • • • • Methotrexate Azathioprine Mycophenolate Leflunomide • Third-line agents • Biologics e.g infliximab • Lung transplantation Prognosis • Remission rates can correlate with the Scadding classification • Lofgren's syndrome or Stage I – 80-90% will resolve • Poor prognosis with chronic disease: • • • • • • • • Lupus pernio Nasal mucosa involvement Chronic uveiitis Chronic hypercalcaemia Nephrocalcinosis Neural involvement >40 Black population Extrapulmonary manifestations • Hypercalcaemia • Dysregulated calcitriol production • Increased intestinal absorption • Mild – dietary advice, reduce sun exposure • Significant - steroids Extrapulmonary manifestations • Skin • Papules and plaques • Erythema nodosum • Lupus pernio Extrapulmonary manifestations • Heart • • • • Conduction defects Palpitations Syncope Wall motion abnormalities Extrapulmonary manifestations • Eyes • • • • • Uveiitis Episcleritis Scleritis Glaucoma Conjunctivitis • Kidney • Obstructive uropathy • Nephrocalcinosis • Glomerulonephritis Extrapulmonary manifestations • CNS • MSK • GI • Others Conclusion • Multisystem disorder • Non-caseous granulomas • Thoracic involvement most common • Acute vs Chronic • Bilateral hilar lymphadenopathy → fibrosis Conclusion • Mainstay of treatment is steroids • Variable prognosis • Disease requiring MDT input
