Sarcoidosis in 6 minutes and 40 seconds Matt

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-DRB 1*1101 associated with disease susceptibility • HLA-DRB 1*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. 5 mg/kg/day prednisolone for ~ 4 weeks, reduce to maintenance dose (5 -20 mg 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