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. 2021 Nov 22;7:e17. doi: 10.15420/cfr.2021.16

Table 1: Common Immunosuppressive Agents in Cardiac Sarcoidosis, Dosing, Toxicities, Surveillance and Prophylaxis Recommendations.

Drug Suggested dosing Important toxicities Other considerations
Corticosteroid
Prednisone 0.5–1 mg/kg daily (max dose 60 mg daily) with tapering guided by clinical and imaging response. See treatment algorithm (Figure 4)
  • Neuropsychiatric: depression, insomnia, psychosis

  • Sodium and fluid retention; worsening heart failure

  • Impaired wound healing

  • Hyperglycaemia

  • Hypertension and increased risk of cardiovascular disease

  • Musculoskeletal: myopathy, osteoporosis, avascular necrosis

  • Adrenal insufficiency

  • Gastrointestinal: gastritis and ulceration

Pre-treatment:
  • Assess cardiovascular risk (lipid, hypertension and glycaemic status) and optimise where possible

  • Exclude latent TB and ensure vaccinations up to date

  • Determine fracture risk using validated tool (e.g. FRAX)[70] and bone densitometry as required

  • Screen for psychiatric comorbidities that may be exacerbated by steroid use

  • Baseline eye exam

Monitoring:
  • Hypertension, hyperglycaemia and hyperlipidaemia screening

  • Close monitoring for fluid retention

  • Regular review of fracture risk and bone density screening as required

  • Eye screening for glaucoma and cataract formation

Prophylaxis:
  • Gastric: H2 blocker or PPI

  • Pneumocystis prophylaxis for doses ≥20 mg daily

  • Pharmacologic therapy for osteoporosis if indicated by fracture risk

Antimetabolite
Methotrexate Initiate at 5–15 mg weekly.
Titrate in 5 mg increments every 4 weeks to a target dose of 20 mg weekly[42]
  • Hepatotoxicity: avoid concurrent alcohol use

  • Myelosuppression: may be preceded by rising MCV

  • Gastrointestinal side-effects: consider increased folic acid or leucovorin rescue therapy; consider splitting daily doses or change to subcutaneous therapy

  • Mucositis: dose dependent

  • Pneumonitis: usually within first year of treatment.

  • Teratogenic: contraindicated in men and women 3 months before planned pregnancy, during pregnancy and breastfeeding

Pre-treatment:[48]
  • Exclude latent TB. Screen for hepatitis B and C and HIV if at risk

  • Baseline chest radiograph, CBC, LFTs, serum creatinine

  • Ensure vaccinations up to date

Monitoring:
  • CBC, LFTs and serum creatinine every 2–4 weeks for first 3 months of treatment, every 8–12 weeks for months 3–6 of therapy and every 12 weeks beyond 6 months of therapy[71]

Prophylaxis:
  • Folic acid 1 mg daily or 5 mg weekly. Consider leucovorin (folinic acid) rescue therapy in toxicity unresponsive to increased folic acid

TNFi biologic agent
Infliximab 3–5 mg/kg at weeks 0, 2, 6 and every 4–8 weeks[8]
  • Worsening of pre-existing heart failure

  • Hypersensitivity reactions

  • Worsening of multiple sclerosis and other demyelinating diseases: avoid[63]

  • Risk of serious infections and malignancy

Pre-treatment:
  • Exclude latent TB. Screen for hepatitis B and C and HIV if at risk

  • Baseline chest radiograph, CBC, LFTs, serum creatinine and LVEF

  • Ensure vaccinations up to date

Monitoring:
  • Regular specialist review every 1–3 months with CBC and LFTs

  • Active infection: temporarily hold. High index of suspicion for opportunistic infections and PML

  • Close monitoring in patients with LV dysfunction for decompensated heart failure

  • Local recommended population-based malignancy screening

Prophylaxis:
  • Low-dose methotrexate ± corticosteroid should be considered to limit development of anti-TNF antibodies

Adalimumab 80–160 mg at week 0, 40 mg at week 1 and 40 mg weekly thereafter[57]
  • Similar to infliximab

  • Similar to infliximab

CBC = complete blood count; CS = cardiac sarcoidosis; FRAX = fracture risk assessment tool; LFT = liver function test; LV = left ventricular; LVEF = left ventricular ejection fraction; MCV = mean corpuscular volume; PML = progressive multifocal leukoencephalopathy; PPI = proton pump inhibitor; TB = tuberculosis; TNFi = tumour necrosis factor inhibitor.