ABSTRACT
The role of pathology in the diagnosis of sarcoidosis is identification of granulomas
in tissue specimens and performance of studies to exclude known causes of granulomatous
inflammation. The granulomas of sarcoidosis are nonspecific lesions that, by themselves
and in the absence of an identifiable etiologic agent, are not diagnostic of sarcoidosis
or any other specific disease.
Among the diseases to be excluded are mycobacterial, fungal, and parasitic infections,
chronic beryllium disease and other pneumoconiosis, hypersensitivity pneumonitis,
and Wegener's granulomatosis. Even after extensive workup a substantial number of
granulomas will remain unclassified. Not every disease that features nonnecrotizing
granulomas of undetermined etiology is sarcoidosis.
The granulomas of sarcoidosis may exhibit focal necrosis of minimal amount. In cases
with granulomas that exhibit a greater degree of necrosis an infectious or other nonsarcoid
etiology should be strongly suspected.
Strict clinical, radiological, and pathological criteria must be used for diagnosis.
In cases that exhibit necrotizing granulomas with more than minimal, focal necrosis,
extrathoracic involvement only, and/or incompatible clinical and radiological findings,
the diagnosis of sarcoidosis should be approached with great caution. The diagnosis
is most secure when compatible clinical and radiological findings are supported by
the demonstration of microorganism-negative, nonnecrotizing granulomas in a biopsy
specimen accompanied by biopsy evidence or strong clinical evidence of multisystem
involvement, and negative cultures for bacteria, mycobacteria, and fungi. A positive
Kveim-Siltzbach test provides strong support for the diagnosis of sarcoidosis.
KEYWORDS
Sarcoidosis - pathology - granuloma - biopsy - diagnosis
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Yale RosenM.D.
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