Updated: 3/23/2020

Sarcoidosis

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
11
0
0
0%
0%
Evidence
9
0
0
Topic
Snapshot
  • potatonodeA previously healthy 30-year-old African American woman has fatigue, arthralgia, and a nodular rash over the trunk and upper extremities for three weeks. There are twelve 3-8 mm, pale, indurated plaques over the chest, back, and upper extremities. The liver is palpable 2 cm below the right costal margin with a percussion span of 14 cm, and the spleen tip is palpable 3 cm below the left costal margin. There is no pain or limitation of any of the joints. A chest radiograph shows bilateral lymphadenopathy.
Introduction
  • Idiopathic condition characterized by granulmomatous inflammation of multiple organs
    • lungs most commonly involved organ
    • commonly causes restrictive lung disease 
      • may also cause obstructive or mixed pattern
    • mediated by CD4 TH cells
      • ↑ IFN-gamma 
    • granulomas can affect all organ systems
      • liver/spleen
      • bone
      • heart
  • Epidemiology
    • most common in black females
    • smoking does not ↑ risk
    • presents most commonly in 3rd or 4th decade
  • Associated conditions
    • diabetes insipidus (granulomatous infiltration of posterior pituitary)
Presentation
  • Symptoms
    • may be asymptomatic
      • 50% as incidental chest radiograph findings
    • cough
    • fever, malaise
    • arthritis
      • symptoms mainly in the ankle and legs
    • can be GRUELING
      • Granulomas
      • Rheumatoid arthritis
      • Uveitis
      • Erythema nodosum
      • Lymphadenopahy
      • Interstitial fibrosis
      • Negative TB
      • Gammaglobulinemia
  • Physical exam
    • skin lesions
      • nodular granulomatous lesions
      • facial rash
      • erthyma nodosum 
        • painful nodules
        • also seen in rheumatic fever
    • eye lesions
      • uveitis 
        • ↓ vision and glaucoma
    • enlarged salivary and lacrimal glands
Evaluation
  • Diagnosis is clinical and often one of exclusion
  • Chest radiograph
    • bilateral hilar lymphadenopathy
    • lungs involved in 90% of the cases
  • Labs
    • ↑ serum ACE (not uniformly) 
    • hypercalcemia 
      • ↑ 1-α-hydroxylase → hypervitaminosis D
        • can cause hypercalemia and renal failure chronically
      • commonly tested, less commonly seen (only about 11%)
    • serum protein electrophoresis (SPE) shows polyclonal gammopathy
    • lack of response to CD4 TH skin tests (like PPD) due to ↑ lung activity and  systemic activity
  • Pulmonary function tests
    • restrictive pattern is common (normal FEV1/FVC with normal TLC)
      • however, obstructive or mixed pattern may also be seen
  • Bronchoalveolar lavage
    • high CD4:CD8 T-cell ratio 
      • due to CD4 infiltrate into the interstitium as well as intralveolar space
      • contrasted to the low ratio in hypersensitivity pneumonitis and HIV
  • Biopsy 
    • noncaseating granulomas with multinucleated giant cells   
      • very suggestive of sarcoidosis 
    • laminated calcium concretions present (cause of density on CXR)
Treatment
  • Conservative
    • observation fine in most cases 
      • majority experience remission within two years without treatment
  • Pharmacologic
    • topical preferred when possible (skin, eyes, nasal, airway, etc)
    • corticosteroids
    • indications
      • extra-pulmonary manifestations: cardiac, neurologic, or uveitis
      • symptomatic hepatosplenomegaly
      • significant hypercalcemia
      • only if refractory to observations
Prognosis
  • Relapsing/remitting course with 50% resolving spontaneously
  • Progression
    • Stage I - bilateral hilar lymphadenopathy
    • Stage II - bilateral hilar lymphadenopathy + upper lobe infiltrates
    • Stage III - lung infiltrates only
    • Stage IV - lung fibrosis

Please rate topic.

Average 5.0 of 16 Ratings

Questions (11)
Evidence (9)
VIDEOS & PODCASTS (1)
EXPERT COMMENTS (19)
Private Note