Macrophagic myofasciitis

Macrophagic myofasciitis: A most unusual inflammatory myopathy, first descr ibed by Germmad had been reported with increasing frequency since 1993 in t he leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis. Clinical features: By November 1999, 70 cases of macrophagic myofasciitis h ad been recorded since our first description. The first 22 patients (seu ra tio M/F = 1:3) referred with the presumptive diagnosis of polymyositis (n = 11), polymyalgia rheumatica (n = 5), mitochondrial cytopathy (n = 4), and congenital myopathy or muscle dystropy (n = 1 each). Symptoms included myal gia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (450%), and fever (32%). Laboratory findings: Abnormal laboratory findings included elevated CK leve ls (50%), markedly increased erythrocyte sedimentation rate (37%), and myop athic EMC; (35%. Muscle biopsy showed a unique myopathological pattern char acterized by: i) centripetal infiltration of epimysium, perimysium and peri fascicular endomysium by sheets of large cells of the moncyte/macrophage li neage (CD68+ CD 1a-,S 100-, with a PAS-positive content; ii) absence of nec rosis, of both epitheloid and giant cells, and of mitotic figures; iii) pre sence of occasional CD8+ T-cells; iv) unconspicuous muscle fiber damage. Th e picture was easily distinguishable from sarcoid myopathy and fasciitis-pa nniculitis syndromes. The infectious diseases know to be associated with re active histiocytes, including Whippleis disease, Mycobacterium avium intrac ellulare infection and malakopalakia, could not be documented. Patients imp roved under corticosteroid therapy and/or immunomodulatory therapeutic Conclusion: A new inflammatory muscle disorder, characterized by a distinct ive pathological pattern of macrophagic myofasciitis is emerging in France.