TABLE 1.

Current widely accepted stepwise medications for the treatment of Sarcoidosis (5, 7, 48, 64).

Historic designation	Drug name	Usual dosage	Major toxicity	Drug monitoring	Comments
“First-Line”	Prednisone/ Prednisolone	20 mg/day initial dose, tapered to 5–10 mg QD to QoD	Weight gain, Diabetes Mellitus, Hypertension, Osteoporosis, Cataracts, Glaucoma, Sleep disturbance, Depression	Blood pressure and serum glucose monitoring, Bone density, Eye exams Body mass index	Causes cumulative toxicity that is dose and duration dependent.
“Second-Line” (Anti-metabolites)	Methotrexate	10–15 mg once a week PO Maybe given SQ if severe GI intolerance	GI Intolerance, Hepatotoxicity, Leukopenia, Fatigue, Pneumonitis.	CBC, LFT, renal function Folate supplementation is recommended.	Preferred anti-metabolite Teratogenic; avoid in pregnancy in both males and females of child-bearing age. Cleared by kidney, avoid in significant renal failure. Doses < 15 mg/week associated with inefficacy.
	Azathioprine	50–250 mg QD	Nausea, Leukopenia, Hepatotoxicity, Risk of Infections, Cutaneous and Lymphoproliferative Cancers.	CBC, LFT	Consider check TPMT level at initiation
	Leflunomide	10–20 mg QD	Nausea, Leukopenia, Hepatotoxicity, Peripheral Neuropathy, Pneumonitis	CBC, LFT, renal function	Due to long half-life, cholestyramine may be necessary to clear drug and its metabolites in toxicity. Teratogenic, avoid in pregnancy and breastfeeding. Cleared by kidney, avoid in significant renal failure
	Mycophenolate Mofetil	500–1,500 mg BID	Diarrhea, Leukopenia, risk of infections, Lymphoproliferative, and Cutaneous cancers	CBC, LFT Negative hepatitis B/C screening and negative IGRA are required prior to initiation	Less experience in sarcoidosis than other agents. Non-nephrotoxic
“Third-Line” Reserved for patients who have failed prior treatment with steroids and/or anti-metabolites	Infliximab or Biosimilars	3–5 mg/Kg IV at weeks 0, 2 and every 4–6weeks	Infections, allergic reactions. Contraindicated in demyelinating neurologic disease, active tuberculosis, deep fungal infections, prior malignancy, and severe CHF	Monitor for allergic reactions Screen for prior tuberculosis (negative IGRA testing) prior to initiation. Negative hepatitis B/C screening also advised.	Allergic reactions can be life threatening. Consider co-administration with Methotrexate to minimize formation of anti-drug antibodies.
	Adalimumab	40 mg SQ every 1–2 weeks	Infections, Allergic reactions Contraindicated in demyelinating neurologic disease, active tuberculosis, deep fungal infections, prior malignancy, and severe CHF	Monitor for allergic reactions Screen for prior tuberculosis (negative IGRA testing) prior to initiation. Negative hepatitis B/C screening also advised.	Less toxic than infliximab. Has been successfully used in patient’s intolerant to infliximab.
	Rituximab	500–1,000 mg IV every 1–6 months	Infections	Screen for viral hepatitis. Check IgG level with chronic therapy	High risk for viral reactivation. Can lead to IgG deficiency.
	Repository corticotropin Injection (RCI)	40–80 Units SQ twice a week	Diabetes Mellitus, Hypertension, Anxiety, Edema, Weight gain, Cataracts, Glaucoma, Sleep Disturbance.	Blood pressure and serum glucose monitoring, Bone density, Eye Exams Body Mass Index	Need to wean prednisone quickly to avoid cumulative toxicity.
Others	Hydroxychloroquine	200–400 mg QD	Loss of vision GI side effects,—abdominal pain, anorexia.	Regular eye exams depending on age and renal function	Beneficial for cutaneous disease. Minimal impact in cardiac and neurologic disease.

CBC, complete blood count; LFT, liver function test; IGRA, interferon gamma release assay for tuberculosis; PO, per oral; SQ, subcutaneously; IV, intravenously; QD, daily; QoD, every other day; TPMT, thiopurine S-methyltransferase (TPMT) genotype or enzyme activity; IgG, Immunoglobulin G; GI, Gastrointestinal (Intolerance, Nausea, vomiting, diarrhea); CHF, congestive heart failure.