Management of sarcoidosis
Overview
  • The management of patients with sarcoidosis is complicated by the fact that there are no clear guidelines to help determine who to treat, when to treat, or how to monitor disease activity
  • In half of patients, the disease will resolve spontaneously within 2 years. After 10 years, up to two-thirds of patients will have complete remission. About one-third of patients have chronic disease that leads to significant organ impairment. [2,3]
Pulmonary disease
  • Incidence - ≥ 90% of patients
  • Symptoms - cough, shortness of breath, chest discomfort, abnormal PFTs, wheezing
  • Who to treat
    • There is no clear guideline or consensus on when, or if, pulmonary sarcoidosis should be treated
      • General points on treatment:
        • Treatment should typically be reserved for patients who are symptomatic
        • Symptomatic disease should be confirmed with pulmonary function tests
        • Radiographic findings do not correlate well with disease chronicity or changes in pulmonary function
        • There is no clear evidence that treatment affects long-term outcomes in sarcoidosis (e.g. fibrosis) [2,3,6]
  • Treatment
    • First-line: prednisone 20 - 40 mg/day for 6 - 12 weeks, then taper to 5 - 10 mg/day; typical treatment duration is 12 months, although course may be shortened in quick-responders [2,5,6]
    • Second-line: methotrexate 5 - 15mg/week; give with folic acid 1 mg/day; requires lab monitoring [7]
  • Monitoring
    • Active disease: chest X-ray and PFTs every 3 - 6 months
    • Stable disease: monitor annually
    • Remission: follow for 3 years after remission [3,5]
  • Chronic sequelae
    • Pulmonary fibrosis
    • Pulmonary hypertension [3]
Eye disease (uveitis)
  • Incidence - 10 - 60% of patients
  • Symptoms - redness, injection, tearing, photophobia, pain; up to a one-third may be asymptomatic
  • Treatment
    • Anterior uveitis (65%) - treat with topical corticosteroids
    • Posterior uveitis (30%) - treat with systemic corticosteroids
    • Refractory cases: methotrexate, infliximab, adalimumab, and rituximab may be beneficial [2]
  • Monitoring
    • Active disease: as needed
    • No eye disease: at least yearly eye exam
  • Chronic sequelae
    • Glaucoma
    • Vision loss [3]
Skin disease
  • Incidence - 20 - 35% of patients
  • Symptoms - erythema nodosum, papular and plaque lesions, lupus pernio; lesions may be precipitated by skin trauma
  • Treatment
  • Studies
    • A randomized controlled trial (N=39) published in 2014 found that thalidomide was no better than placebo in treating cutaneous sarcoidosis [PMID 24945194]
Cardiac disease
  • Incidence - 5% of patients have symptomatic disease
  • Symptoms - palpitations, syncope, EKG changes (e.g. AV node and bundle branch block), sudden death
  • Treatment: prednisone 30 - 40 mg/day; pacemaker/implantable defibrillator when indicated
  • Monitoring: periodic EKG (every 6 - 12 months); holter, PET scan, MRI, ECHO, when indicated [2,4,6]
Neurologic disease
  • Incidence - 5% of patients
  • Symptoms - cranial nerve dysfunction (particularly the facial nerve), peripheral neuropathy
  • Treatment: - prednisone, may require higher doses (40 - 80 mg/day), infliximab [3,4,6]
  • Studies
    • A case series published in 2017 reviewed the efficacy of infliximab in treating CNS sarcoidosis [PMID 29030454]
Bone disease
  • Incidence - up to 5% of patients
  • Symptoms - typically affects the hands and feet; is usually asymptomatic
  • Treatment: prednisone, denosumab [8]
Hypercalcemia / hypercalciuria
  • Hypercalcemia - occurs in 10 - 20% of patients
    • Caused by macrophages that convert vitamin D to more active form
    • Treat with hydroxychloroquine or corticosteroids
    • Typically treat when serum calcium is ≥ 11 mg/dl [4,6]
  • Hypercalciuria - occurs in up to 50% of patients; may lead to kidney stones; treat with corticosteroids or hydroxychloroquine [4]
Liver / kidney disease
  • Liver granulomas: occur in 50 - 80% of patients; liver enzymes are elevated in one-third of patients; severe liver disease is rare [4]
  • Kidney granulomas: occur in 7 - 23% of patients; significant kidney disease is rare [4]