About Sarcoidosis

Sarcoidosis is an autoimmune disease with increased inflammation and organ infiltration driven by granuloma formation.

  • Females = males

  • Average age of diagnosis: 40-55 yo
  • Risk Factors
    • Family history (strongest)
    • Human leukocyte antigens (HLA) association
    • Environmental: inorganic and organic materials (dust, mold, silica, metals, etc.)
  • Classic presentations (Lofgren’s, Heerfordt) most times do not need histology for diagnosis and steroid can be started without biopsy.
  • 90-95% patients have pulmonary involvement.
    • Most common: Hilar lymphadenopathy
  • Upon diagnosis of sarcoidosis, even without ocular symptoms, need baseline EKG and ophthalmologic exam 
  • Ruling out infection is critical as immunosuppression is the cornerstone for sarcoid therapy.
  • Not all patients need immunosuppressive therapy!

Classic Presentations

May not need bx for confirmation

  • Lofgren: arthritis (ankles, knees), erythema nodosum, bilateral hilar LN
  • Heerfordt: parotiditis, uveitis, facial nerve palsy, fever, arthritis

Organ Related

Pulmonology
  • 90-95% pulm involvement
  • Hilar LN
  • Endobronchial
  • ILD
  • pHTN
Ophthamology
  • Anterior uveitis-most common
Dermatology
  • EN
  • Pernio
Cardiac
  • Conduction abnl
  • Infiltrative cardiomyopathy
Neurology
  • CN palsy
  • Sensorimotor polyneuropathy
  • Leptomeningeal involvement
MSK
  • Polyarthritis
  • Osseous involvement

Lab Workup

  • CBC, CMP, ESR, CRP
  • Serum angiotensin- converting enzyme (ACE):
      – Poor specificity and sensitivity!
      – Supportive, not diagnostic
  • Vitamin D 25-OH, vitamin D 1,25-OH

Tests

  • Baseline PFTs
  • Baseline ophth exam
  • Baseline EKG

Imaging

  • High res chest CT: more sens than CXR
  • Suspected cardiac sarcoid: Need cardiac MRI or  cardiac PET

Histology

  • Shows well-circumscribed, compact, noncaseating granulomas of the epithelioid type rimmed by hyaline collagen

Infections

  • Mycobacterial
  • Fungal
  • Mycoplasma
  • PJP
  • Brucellosis
  • Toxoplasmosis

Environmental

  • Berylliosis
  • Tattoos
  • Hypersensitivity pneumonitis
  • Inorganic agents

Drug Induced

  • TNFi

Other Autoimmune Disease

Other

  • Cancer
  • Lymphocytic
  • Interstitial pneumo

Who needs immunosuppression?

  • Not all patients!
  • Symptomatic (baseline or exertional)
       and/or
  • Significant abnormalities on imaging or PFTs:
    • Severe reticular disease
    • Traction bronchiectasis
    • Mod-sev low FVC or DLCO  

Initial Therapy

  • Steroids (Pred 20-30mg/d, 6mo taper
  • Consider: 
    • Organ involved 
    • Disease severity 
    • Comorbidities

Monitoring

  • Repeat ophthalmologic exams annually or with development of symptoms
  • Monitor for symptoms of cardiac involvement
  • Many do not ever develop symptoms and so don’t ever require therapy. 

Prognosis

  • After treatment, many patients have spontaneous remission
  • Other patients have chronic active disease
  • Lofgren syndrome prognosis good!
    • 70-80% of patients in remission 2 yrs after diagnosis