Table 1
Management of cardiac sarcoidosis.
| Monitoring | At least yearly or sooner depending on symptoms, disease severity, laboratory parameters (echocardiography, troponin, BNP) |
| Addressing concomitant cardiovascular risk factors | Coronary artery disease, hypertension |
| Standard medical treatments of heart failure, diastolic dysfunction | |
| Managing conduction abnormalities (AV blocks) | Consider pacemaker |
| Managing ventricular arrythmias, risk for sudden cardiac death | Consider implantable cardioverterdefibrillator |
| Immunosuppressive treatment* | Administer in functional cardiac abnormalities
(arrythmias, myocardiopathy, blocks)® In asymptomatic cases with normal ejection fraction: individualized assessment as per treatment initiation |
| First-line treatment: corticosteroids | Second-line treatment: methotrexate,
leflunomide, azathioprine, mycophenolate mofetil Third-line treatment: infliximab, adalimumab, cyclophosphamide |
[i] * Risk factors: Age greater than 50, left ventricular ejection fraction of less than 40%, New York Heart Association functional class 3 or 4, increased left ventricular end-diastolic diameter, late gadolinium enhancement on cardiac MRI, ventricular tachycardia, cardiac inflammation identified by fluorodeoxyglucose positron emission tomography (FDG-PET) scan, echocardiographic evidence of abnormal global longitudinal strain, interventricular septal thinning, elevated troponin or BNP. BNP: brain natriuretic peptide; AV: atrioventricular
®: Monitor myocardial inflammation with FDG-PET
Table 2
Drugs used to treat sarcoidosis. qd: every day; bid: twice a day
| DRUG | DOSE | SIDE EFFECTS | CARDIAC SARCOIDOSIS |
|---|---|---|---|
| Prednisone | 20 mg qd Follow-up 5-10 mg qd | Diabetes Weight gain Osteoporosis Glaucoma Cataract Depression/psychosis | Initiating dose 30 mg qd |
| Methotrexate | 10-15 mg once a week | Nausea, bone marrow suppression, liver toxicity, pulmonary toxicity | Effective in combination with corticosteroids |
| Leflunomide | 10-20 mg qd | Nausea, peripheral neuropathy, interstitial pneumonia | |
| Azathioprine | 50-250 mg qd | Nausea, bone marrow suppression, liver toxicity, malignancies | Less effective than methotrexate |
| Mycophenolate mofetil | 500-1500 mg bid | Diarrhea, monitor blood cell counts | Reasonably effective |
| Infliximab | 3-5 mg/kg initially, 2nd dose after 2 weeks, then every 4-6 weeks | Tuberculosis activation, infections, contraindicated in severe heart failure, demyelinating neurological diseases, active tuberculosis, prior malignancy | Effective, caution for infections, cardiac symptoms47 |
| Adalimumab | 40 mg every 1-2 weeks | Tuberculosis activation,
infections Contraindicated in severe heart failure, demyelinating neurological diseases, active tuberculosis, prior malignancy | Less effective than infliximab, caution for infections, cardiac symptoms47 |
| Rituximab | 500-1000 mg every 1-6 months | Screen for hepatitis, tuberculosis activation, humoral deficiency | |
| Hydroxychloroquine | 200-400 mg qd | Retinopathy |
Figure 1
Schema for sarcoidosis treatment. Systemic treatment initiates regularly with corticosteroids, with dose depending on indication. Among second- and third-line treatments, methotrexate and infliximab (in bold) have the most evidence for use. Agent selection depends on indication, comorbidities, and anticipated toxicity. A proportion of these patients develop features of advanced sarcoidosis (right column) with respective mortality risk where additional treatment modalities may be required. RHC: right heart catheterization; ICD: implantable cardioverter defibrillator14,69,76,81,83,84