{"orpha":247718,"name":"Inflammatory myopathy with abundant macrophages","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IMAM"],"definition":"A rare idiopathic inflammatory myopathy characterized by diffuse destructive infiltration of CD68+ macrophages into the fascia rather than muscle fibers in muscle biopsies, proximal muscle weakness and myalgia with or without scaly dermatomyositis-like or atypical non-dermatomyositis-like skin lesions, elevation of creatine kinase levels and thickening of muscle fascia in muscle MRI.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-247718/","orphanet_url":"https://www.orpha.net/en/disease/detail/247718","names":{"fr":{"name":"Myopathie inflammatoire avec abondance de macrophages","synonyms":["IMAM","MIAM"],"definition":"Une myopathie inflammatoire rare caractérisée par une infiltration destructrice diffuse de macrophages CD68+ dans le fascia plutôt que dans les fibres musculaires, observable à la biopsie musculaire. Elle est associée à une faiblesse et une myalgie des muscles proximaux avec ou sans lésions cutanées squameuses de type dermatomyosite ou des lésions atypiques de type non dermatomyosite, à une élévation des taux de créatine kinase et à un épaississement du fascia musculaire visible en imagerie par résonance magnétique (IRM) musculaire."},"de":{"name":"Inflammatorische Myopathie mit überzähligen Makrophagen","synonyms":["IMAM"],"definition":"Eine seltene idiopathische entzündliche Myopathie, die durch eine diffuse destruktive Infiltration von CD68+ Makrophagen in die Faszie, aber nicht in die Muskelfasern in Muskelbiopsien, proximale Muskelschwäche und Myalgie mit oder ohne Dermatomyositis-ähnliche oder atypische nicht-Dermatomyositis-ähnliche schuppende Hautläsionen, erhöhte Kreatinkinasewerte und Verdickung der Muskelfaszie im Muskel-MRT gekennzeichnet ist."},"es":{"name":"Miopatía inflamatoria con abundancia de macrófagos","synonyms":["IMAM"],"definition":"Es una miopatía inflamatoria idiopática poco frecuente caracterizada por infiltración destructiva difusa de macrófagos CD68+ en la fascia en lugar de en las fibras musculares, patente en biopsias musculares, debilidad muscular proximal y mialgia con o sin lesiones cutáneas escamosas tipo dermatomiositis o lesiones atípicas no similares a dermatomiositis, elevación de los niveles de creatinquinasa y engrosamiento de la fascia muscular en la resonancia magnética."},"it":{"name":"Miopatia infiammatoria con eccesso di macrofagi","synonyms":["IMAM"],"definition":"La miopatia infiammatoria con eccesso di macrofagi è una miopatia infiammatoria rara, caratterizzata, sulla biopsia muscolare, da una infiltrazione distruttiva diffusa di macrofagi CD68+ nella fascia (anziché nelle fibre muscolari), debolezza dei muscoli prossimali e mialgia con o senza lesioni cutanee desquamative, che ricordano la dermatomiosite, o atipiche, che non la ricordano, aumento dei livelli di creatin-chinasi e ispessimento della fascia muscolare apprezzabile alla RMN dei muscoli."},"nl":{"name":"Inflammatoire myopathie met abundante macrofagen","synonyms":["IMAM"],"definition":"Een zeldzame idiopathische inflammatoire myopathie, gekarakteriseerd door diffuse destructieve infiltratie van CD68+ macrofagen in fascie eerder dan spiervezels in spierbiopten, proximale spierzwakte en myalgie met of zonder schilferende dermatomyositis-achtige of atypische niet-dermatomyositis-achtige huidlaesies, verhoogde gehaltes van creatinekinase, en verdikking van spierfascie op MRI van spier."},"pt":{"name":"Miopatia inflamatória com macrófagos abundantes"},"pl":{"name":"Miopatia zapalna z licznymi makrofagami","synonyms":["IMAM"]},"cs":{"name":"Zánětlivá myopatie s nadbytkem makrofágů","synonyms":["IMAM"]},"uk":{"name":"Запальна міопатія з великою кількістю макрофагів","synonyms":["ІМАМ"],"definition":"A rare idiopathic inflammatory myopathy characterized by diffuse destructive infiltration of CD68+ macrophages into the fascia rather than muscle fibers in muscle biopsies, proximal muscle weakness and myalgia with or without scaly dermatomyositis-like or atypical non-dermatomyositis-like skin lesions, elevation of creatine kinase levels and thickening of muscle fascia in muscle MRI."},"zh":{"name":"炎症型肌病伴大量巨噬细胞","synonyms":["IMAM"],"definition":"炎症性肌病伴大量巨噬细胞是一种罕见的炎症性肌病，其特征是肌活检中发现筋膜中而不是肌纤维中出现CD68+巨噬细胞弥漫性破坏性浸润，近端肌无力和肌痛伴或不伴鳞状皮肌炎样或非典型非皮肌炎样皮损，肌肉MRI中肌酸激酶水平升高和肌筋膜增厚。"},"tr":{"name":"Bol makrofajlar ile seyreden inflamatuar miyopati","synonyms":["IMAM"]}},"xrefs":[{"source":"ICD-10","code":"G72.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016609","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707791","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98482,"name":"Idiopathic inflammatory myopathy","classifications":[181,196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}