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[Macrophagic myofasciitis. Study and Research Group on Acquired and Dysimmunity-related muscular diseases (GERMMAD)].
- Source :
-
Presse medicale (Paris, France : 1983) [Presse Med] 2000 Feb 05; Vol. 29 (4), pp. 203-8. - Publication Year :
- 2000
-
Abstract
- Unlabelled: MACROPHAGIC MYOFASCIITIS: A most unusual inflammatory myopathy, first described by Germmad had been reported with increasing frequency since 1993 in the leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis.<br />Clinical Features: By November 1999, 70 cases of macrophagic myofasciitis had been recorded since our first description. The first 22 patients (sex ratio 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 dystrophy (n = 1 each). Symptoms included myalgia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%).<br />Laboratory Findings: Abnormal laboratory findings included elevated CK levels (50%), markedly increased erythrocyte sedimentation rate (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathological pattern characterized by: i) centripetal infiltration of epimysium, perimysium and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-, with a PAS-positive content; ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; iii) presence of occasional CD8+ T-cells; iv) inconspicuous muscle fiber damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases know to be associated with reactive histiocytes, including Whippleís disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients improved under corticosteroid therapy and/or immunomodulatory therapeutic<br />Conclusion: A new inflammatory muscle disorder, characterized by a distinctive pathological pattern of macrophagic myofasciitis is emerging in France.
- Subjects :
- Adult
Aged
Biopsy
Clinical Enzyme Tests
Creatine Kinase blood
Diagnosis, Differential
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Muscle Weakness etiology
Muscle, Skeletal pathology
Muscular Diseases etiology
Muscular Diseases pathology
Pain etiology
Fasciitis diagnosis
Fasciitis etiology
Fasciitis pathology
Macrophages
Muscular Diseases diagnosis
Subjects
Details
- Language :
- French
- ISSN :
- 0755-4982
- Volume :
- 29
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Presse medicale (Paris, France : 1983)
- Publication Type :
- Academic Journal
- Accession number :
- 10705901