Table 3.

Summary of the initial and long-term management of pulmonary and cardiac sarcoidosis.

OrganClinical featuresInitial therapyChronic therapy
LungsCXR stage I and asymptomaticObservationObservation
Cough, wheezingInhaled 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 sarcoidosisInfliximab 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
HeartAsymptomatic with preserved LV systolic functionDisease monitoringDisease monitoring: implantable loop recorders, regular echocardiography, baseline cardiac MRI
Complete heart blockPrednisolone
Conventional pacemaker/ICD
Conventional pacemaker/ICD
Ventricular fibrillation, tachyarrhythmiasBeta-blocker therapy (caution if no device)
Intravenous methylprednisolone for resistant ventricular arrhythmias.
Amiodarone, sotalol
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)
Refractory cardiac sarcoidosisInfliximab, (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.