Table 3.

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.