Inclusion body myositis
Synonyms: IBM · Sporadic inclusion body myositis · sIBM
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare degenerative inflammatory disorder of skeletal muscles characterized by late onset weakness, starting in either the quadriceps or finger flexors and slowly progressing to include other groups of limb muscles. Distinctive histopathological features include inflammatory and degenerative features.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Inclusion body myositis | IBM · Sporadic inclusion body myositis · sIBM |
| fr | Myosite à inclusions | IBM · Myosite à inclusions sporadique · sIBM |
| de | Einschlusskörper-Myositis | Einschlusskörper-Myositis, sporadische · IBM · sIBM |
| es | Miositis por cuerpos de inclusión | IBM · Miositis por cuerpos de inclusión esporádica · sIBM |
| it | Miosite da corpi di inclusione | IBM · Miosite sporadica da corpi di inclusione · sIBM |
| nl | Inclusielichaammyositis | IBM · Inclusion body myositis · Sporadische inclusielichaammyositis · Sporadische inclusion body myositis · sIBM |
| pt | Miosite de corpos de inclusão | |
| pl | Wtrętowe zapalenie mięśni | IBM · sIBM · Sporadyczne wtrętowe zapalenie mięśni |
| cs | Myozitida s inkluzními tělísky | IBM · sIBM · Sporadická myozitida s inkluzními tělísky |
| uk | Міозит зі включеними тільцями | IBM · sIBM · Спорадичний інфільтративний міозит тіла |
| zh | 包涵体肌炎 | 散发性包涵体肌炎 |
| tr | İnklüzyon cisimcik miyoziti | IBM · sIBM · Sporadik inklüzyon cisimcikli miyoziti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 147421 | E | validated | |
| ICD-10 | M60.8 | NTBT | attributed | validated |
| ICD-11 | 4A41.20 | NTBT | index | validated |
| MONDO | 0007827 | E | validated | |
| MeSH | D018979 | E | validated | |
| UMLS | C0238190 | E | validated | |
| GARD | 3896 | E | validated | |
| MedDRA | 10066407 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98482Idiopathic inflammatory myopathy
rare neurological diseases; rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
7 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 1 000 000 | 0.5 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2012[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 2.5 / 100 000 | Sweden | Value and class | Validated | 35596584[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.49 / 100 000 | Netherlands | Value and class | Validated | 11087787[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.2 / 100 000 | Australia | Value and class | Not yet validated | 10842277[PMID]_18815046[PMID]_ ORPHANET |
| Point prevalence | 1-9 / 100 000 | 1.1 / 100 000 | United States | Value and class | Validated | 11552086[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.1 / 100 000 | Turkey | Value and class | Validated | 21842592[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.2 / 100 000 | Sweden | Value and class | Validated | 35596584[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
14 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004303 | Abnormal muscle fiber morphology | |
| HP:0002960 | Autoimmunity | |
| HP:0003457 | EMG abnormality | |
| HP:0003236 | Elevated circulating creatine kinase concentration | |
| HP:0009071 | Inflammatory myopathy | |
| HP:0003701 | Proximal muscle weakness | |
| HP:0003731 | Quadriceps muscle weakness | |
| HP:0003200 | Ragged-red muscle fibers | |
| HP:0003805 | Rimmed vacuoles | |
| HP:0003202 | Skeletal muscle atrophy | |
| Frequent (79-30%) | ||
| HP:0034153 | Anti-cytosolic-5-nucleotidase-1A antibody positivity | |
| HP:0008872 | Feeding difficulties in infancy | |
| HP:0001315 | Reduced tendon reflexes | |
| Occasional (29-5%) | ||
| HP:0003326 | Myalgia | |
Run the differential on the 10 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/611
- This record as JSON: /data/d/611.json