SARCOIDOSIS

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SARCOIDOSIS

• Rheumatology Module
• Anna Mae Smith, MPAS, PA-C

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Definition

• Multisystem granulomatous disorder
• Whats granulomatous???
• Mass or nodule composed of chronically inflamed
  tissue.
• Non-infectious multisystem disease of unknown
  cause, commonly affecting young and middle-age
  adults.

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Presentation

• Bilateral symmetrical hilar lymphadenopathy
• Pulmonary infiltrates
• Skin lesions
• Eye lesions(uveitis)
• Other organs may be involved, including liver,
  spleen, lymph nodes, heart, and central nervous
  system.

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Immunopathology

• Sarcoid granulomas consist of focal accumulations
  of epitheloid cells, macrophages, lymphocytes,
  mainly T cells!
• T lymphocytesslightly B cells in the
  circulatory system.

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Epidemiology

• Increased prevalence found in Scandinavians,
  Japanese, Irish females, and African American
  women
• 30-80 per 100,000
• Predominant age 20-60 years
• Women gt men
• Course worse in African Americans than whites

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Differential Diagnosis

• Infectious granulomatous disease such as
  tuberculosis and fungal infections
• Foreign body reactions
• Lymphoma
• Other malignancies associated with
  lymphadenopathy
• Berylliosis

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Presentation

• Present with pulmonary symptoms or found on
  x-ray!
• Fatigue
• Weight loss

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Presentation

• Cough
• Shortness of breath
• Skin (new lesions)
• Pain or irritation of eyes
• General fatigue, malaise
• Fever
• Night sweats
• Bell's palsy

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Bilateral hilar lymph adenopathy

• Characteristic feature of sarcoid
• Often symptomless
• but may have associated dull, ache in
  chest, malaise fever

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Pulmonary Infiltration

• More progressive leads to dyspneacor
  pulmonaledeath
• Honeycomb appearance on chest x-ray!
• PFTs restrictive pattern

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Skin

• Erythema nodosum
• Bilateral symmetrical hilar lymphadenopathy
  erythema nodosum sarcoidosis!!!

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Bone Joint

• Arthralgias
• Bone cysts (especially in the digits)
• Swelling

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Acute Sarcoid Arthritis

• Migratory, Additive, Symmetric Polyarthritis
  (usu. Ankles knees)
• Monoarthritis very rare
• Minimal effusion
• Minimal Inflammation
• Synovial Bx- Mild inflammation

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Cardiac

• Ventricular dysrhythmias
• Conduction defects
• Cardiomyopathy with CHF
• Hypercalcemia

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labs

• X-ray
• CBC - normochromic, normocytic anemia,
  Lymphopenia, leukopenia
• Chem20 - calcium hypergammaglobulinemia
• Bronchial Biopsy
• ACE
• PFT

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CXR staging

• Routine chest roentgenograms are staged using
  Scadding's classification
• Stage 0 normal
• Stage 1 hilar adenopathy alone
• Stage 2 hilar adenopathy plus parenchymal
  infiltrates
• Stage 3 parenchymal infiltrates alone
• Stage 4 pulmonary fibrosis

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Special Tests

• Abnormal liver function, especially increased
  alkaline phosphatase is frequently encountered
• Hypercalciuria occurs in up to 10 of patients,
  with hypercalcemia less frequent
• Kveim-Siltzbach skin test can be performed. This
  test is fairly sensitive (approximately 80) and
  highly specific (gt 95)

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Be careful...

• Prednisone will lower serum-angiotensin
  converting enzyme
• Hyperthyroidism and diabetes will increase serum
  angiotensin converting enzyme level

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Treatment

• Hilar lymphadenopathy alone with no evidence of
  lung parenchymal involvementNO treatment!
• Steroids!! 30mg qd x 6 wks than 15mg qd x 6-12
  months
• Topical too!

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Alternative Drugs

• Methotrexate 10 mg/wk
• hydroxychloroquine (Plaquenil) 100-200 mg per day
• azathioprine 50-100 mg per day
• chlorambucil 0.1-0.2 mg/kg.

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Prognosis

• If bilateral hilar lymphadenopathy80 remission
  rate
• 80 of patients will have spontaneous resolution
  within two years
• 10 will have significant fibrosis but no further
  worsening of disease after 2 yrs
• 10 (higher in some populations, including
  African Americans) will have chronic dz.