We evaluated the clinical and myopathological features of all patients
with granulomas in muscle biopsy specimens identified over a 5-year p
eriod (1992-1996) at the Washington University Medical Center. Ten pat
ients were found to have granulomas in their muscle biopsy specimens.
Of these, eight patients had myopathic changes. Seven had dysphagia as
a major functional difficulty during the course of their disease. Non
e had elevated levels of serum creatine kinase (CK). Four of the patie
nts with myopathy had systemic sarcoidosis and relatively severe proxi
mal weakness with functional disability. Treatment with corticosteroid
s was followed by marked improvement in strength and functional disabi
lity. The four other patients with myopathy had no systemic signs of s
arcoidosis. Weakness was especially prominent distally in three of the
se patients. The two patients in this group treated with corticosteroi
ds did not improve. The final two patients, who had granulomas in musc
le but no myopathic changes, had clinical syndromes of mononeuritis mu
ltiplex and eosinophilic fasciitis (Shulman syndrome). We conclude tha
t granulomatous myopathy, in the presence or absence of systemic sarco
idosis, is commonly associated with dysphagia (87%) and a normal serum
CK. Clinical features in patients with sarcoidosis included severe pr
oximal weakness with functional disability that often responded to cor
ticosteroid treatment. Granulomatous myopathy without systemic sarcoid
osis was associated with milder, but more predominantly distal weaknes
s.