SARCOIDOSIS

A multisystem granulomatous disorder of unknown etiology.

Clinical manifestations

a.      Mostly young adults.

b.     Any organ may be involved with the lungs, lymphatics, skin, liver, eyes most commonly affected in decreasing order. Some manifestations to watch for:
- Myocardial involvement (possible conduction disturbances)
- Uveitis
- Cranial nerve VII involvement
- Erythema nodosa

c.      PFTs: Decreased volumes, compliance, DLCO, hypoxemia.

d.     BAL: Predominance of T-lymphocytes and macrophages.

e.      Immunology: Activated T-lymphocytes secrete IL-2 which, as a monocyte chemotactic factor, recruit monocytes and thus contribute to granuloma formation.

f.       CXR:
- Stage 0  Clear
- Stage I   Bilateral hilar adenopathy
- Stage II  Hilar adenopathy and parenchymal infiltrates
- Stage III Parenchymal infiltrates only
- State IV Extensive fibrosis and distortion of lung architecture

g.     Gallium-67 Imaging: increased pulmonary uptake with alveolitis.

h.     Hypercalcemia (10-15%), hypercalciuria (20-30%)

i.        Elevated ACE level (nonspecific); however, ACE may be followed for therapeutic response.

j.        Hyperglobulinemia, impaired delayed hypersensitivity.

Diagnosis: Transbronchial lung biopsy showing non-caseating granulomas with compatible clinical picture.

Therapy: Corticosteroids

a.      Indications for prednisone therapy: active alveolitis with severe symptoms, uveitis, liver disease (marked), cardiac disease, CNS disease, hypercalcemia.

b.     Secondary agent: methotrexate, antimalarial agents controversial.

Transplantation

a.      Recurrent sarcoid granulomata noted in allografts; immunosuppression for transplantation may attenuate granulomatous responses in patients having recurrent disease.