Summary of the initial and long-term management of pulmonary and cardiac sarcoidosis.
| Organ | Clinical features | Initial therapy | Chronic therapy |
|---|---|---|---|
| Lungs | CXR stage I and asymptomatic | Observation | Observation |
| Cough, wheezing | Inhaled corticosteroids | ||
| CXR stage II–IV; dyspnoea with FEV1 or FVC <70% | Prednisolone at 20–40 mg/day for 1–3 months. | Tapering of dose to 5–10 mg/day after initial 1–3 months. Treatment continues for 9–12 months before reassessment (and possible discontinuation)
Steroid-sparing therapies: > Methotrexate 5–25 mg/week, with folic acid > Azathioprine 50–200 mg/day > Leflunomide 10– 20 mg/day Lung transplantation | |
| Refractory pulmonary sarcoidosis | Infliximab 3–5mg/kg as a stat intravenous dose. | Infliximab 3–5mg/kg intravenously at 2 weeks, then monthly
Lung transplantation Rituximab has also shown potential | |
| Heart | Asymptomatic with preserved LV systolic function | Disease monitoring | Disease monitoring: implantable loop recorders, regular echocardiography, baseline cardiac MRI |
| Complete heart block | Prednisolone
Conventional pacemaker/ICD | Conventional pacemaker/ICD | |
| Ventricular fibrillation, tachyarrhythmias | Beta-blocker therapy (caution if no device)
Intravenous methylprednisolone for resistant ventricular arrhythmias. ICD | Amiodarone, sotalol
ICD | |
| Reduced left ventricular ejection fraction (<35%) | Prednisolone
ACE inhibitors, AII-blockers, mineralocorticoid receptor antagonists, beta-blockers | Prednisolone with
steroid-sparing therapies: > methotrexate > cyclophosphamide > azathioprine > hydroxychloroquine Conventional heart failure pharmacotherapy (ACE inhibitors, AII-blockers, mineralocorticoid receptor antagonists, diuretics, beta blockers) CRT-D | |
| Refractory cardiac sarcoidosis | Infliximab, (contraindicated in NYHA class III–IV heart failure) | Infliximab 3–5mg/kg intravenously at 2 weeks, then monthly. |
ACE = angiotensin-converting enzyme; AII = angiotensin II receptor; CRT-D = cardiac resynchronisation therapy defibrillator; CXR = chest X-ray; EP = electrophysiology; FEV1 = forced expiratory volume in 1 sec; FVC1 = forced vital capacity; ICD = implantable cardiac-defibrillator; LV = left ventricular; MRI = magnetic resonance imaging; NYHA = New York Heart Association.