raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:247718

Inflammatory myopathy with abundant macrophages

Synonyms: IMAM

Type
Disease
Group
Disorder
Inheritance
Not applicable
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enInflammatory myopathy with abundant macrophagesIMAM
frMyopathie inflammatoire avec abondance de macrophagesIMAM · MIAM
deInflammatorische Myopathie mit überzähligen Makrophagen= en
esMiopatía inflamatoria con abundancia de macrófagos= en
itMiopatia infiammatoria con eccesso di macrofagi= en
nlInflammatoire myopathie met abundante macrofagen= en
ptMiopatia inflamatória com macrófagos abundantes
plMiopatia zapalna z licznymi makrofagami= en
csZánětlivá myopatie s nadbytkem makrofágů= en
ukЗапальна міопатія з великою кількістю макрофагівІМАМ
zh炎症型肌病伴大量巨噬细胞= en
trBol makrofajlar ile seyreden inflamatuar miyopati= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10G72.4NTBTattributedvalidated
MONDO0016609Evalidated
UMLSC4707791Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card