Sarcoidosis

Lab Workup

• CBC, CMP, ESR, CRP
  • Watch for hypercalcemia
• Serum angiotensin-converting enzyme (ACE)
  • Poor specificity and sensitivity!
  • Supportive, not diagnostic
• Vitamin D 25-OH, vitamin D 1,25-OH
  • High conversion of vitamin D 25-OH to vitamin D 1,25-OH contributes to hypercalcemia in sarcoidosis

Imaging

• High-resolution chest CT: more sensitive than CXR
• Suspected cardiac sarcoid: need cardiac MRI or cardiac PET

Tests

• Baseline PFTs
• Baseline ophthalmology exam
• Baseline EKG

Histology

• Shows well-circumscribed, compact, non-caseating granulomas of the epithelioid type rimmed by hyaline collagen
  • If necrotizing/caseating features on histopathology, consider alternate diagnosis,
    although can be seen in sarcoidosis
• Erythema nodosum histopathology will show panniculitis

Infections

• Mycobacterial
• Fungal
• Mycoplasma
• Pneumocystis jiroveci pneumonia (PJP)
• Brucellosis
• Toxoplasmosis

Environmental

• Berylliosis
• Tattoos
• Hypersensitivity pneumonitis
• Inorganic agents

Drug Induced

• TNF inhibitors

Other Autoimmune Disease

• Small-vessel vasculitis
• Inflammatory bowel disease
• Behcet’s syndrome
• Rheumatoid nodules

Other

• Cancer
• Lymphocytic
• Interstitial pneumonia

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
• Symptomatic extra pulmonary manifestations

Initial Therapy

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

Second-line Therapy

Includes but is not limited to:

• Methotrexate
• Leflunomide
• Mycophenolate
• Azathioprine
• TNFi like infliximab

Monitoring

• Labs: CBC and differential, creatinine, calcium, LFTs, 25 hydroxy and 1,25 dihydroxy vitamin D at least every year
• Repeat ophthalmologic exams annually or with development of symptoms: ocular pain, scleral redness, vision changes
• EKG at least every year, and monitor for symptoms of cardiac involvement – new arrhythmia, new heart failure –  to see if more frequent cardiac evaluation needed
• Any new symptom of pulmonary involvement; consider spirometry every 3-6 mos with consideration of chest imaging
• Many do not ever develop symptoms and hence don’t ever require therapy.

Prognosis

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

• 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 (view image)
• 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!