Granulomatous Myopathy in Patients with Sarcoidosis and Myasthenia Gravis
Zámečník J.1, Ambler Z.2, Ehler E.3, Šimková L.4, Mazanec R.5, Schützner J.6
1 Ústav patologie a molekulární medicíny UK 2. LF a FN Motol, Praha 2 Neurologická klinika LF UK a FN, Plzeň 3 Neurologické oddělení, Krajská nemocnice, Pardubice 4 Neurologická klinika UK 1. LF a VFN, Praha 5 Neurologická klinika UK 2. LF a FN Motol, Praha 6 III. chirurgická klinika UK 1. LF a FN Motol, Praha |
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Summary:
Granulomatous myopathies are extremely rare. The finding of epithelioid granulomas in muscle
biopsy indicates mostly an involvement of the skeletal muscle in systemic sarcoidosis. In this
report we provide description of five patients with previously diagnosed sarcoidosis (pulmonary
or cutaneous), in which a clinically significant muscle weakness developed. We aim at
demonstrating the value of muscle biopsy for further treatment of the patients, since clinically
indistinguishable myopathies can arise not due to the involvement of muscles in the
granulomatous process, but due to the corticosteroid-induced changes (chronic steroid myopathy).
The demonstration of a selective atrophy of type II muscle fibers can provide the clue for
distinguishing the patients, in which the corticosteroid treatment should continue, from those, in
which the treatment should be modified. Further, we discuss a rare finding of granulomas in
muscle biopsies of two patients with myasthenia gravis (MG) associated with thymoma. Although
it is difficult to explain the pathogenesis of this event, MG should be considered in the differential
diagnosis of granulomatous myopathies. Moreover, the finding of granulomas along with
lymphocytic infiltration in MG muscle should lead to a search for an underlying thymic neoplasm.
Key words:
myopathy – granulomas – sarcoidosis – myasthenia gravis
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