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
- 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
- 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.
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