immune-mediated necrotizing myopathy
MONDO:0016098Mondo
Findings
No curated finding names immune-mediated necrotizing myopathy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Necrotizing autoimmune myopathy (NAM) is a rare form of idiopathic inflammatory myopathy characterized clinically by acute or subacute proximal muscle weakness, and histopathologically by myocyte necrosis and regeneration without significant inflammation.
Definition from the Mondo Disease Ontology (MONDO:0016098), read 2026-09-29. CC BY 4.0.
Features
24 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Autoimmune antibody positivityHPOHP:0030057
- Very frequent (80% to 99% of cases)
- Elevated circulating creatine kinase activityHPOHP:0003236
- Very frequent (80% to 99% of cases)
- EMG: myopathic abnormalitiesHPOHP:0003458
- Very frequent (80% to 99% of cases)
- Fatiguable weakness of proximal limb musclesHPOHP:0030200
- Very frequent (80% to 99% of cases)
- Muscle fiber necrosisHPOHP:0003713
- Very frequent (80% to 99% of cases)
- MyopathyHPOHP:0003198
- Very frequent (80% to 99% of cases)
- Anti-3-hydroxy- 3-methylglutaryl-coA reductase antibody positivityHPOHP:0033708
- Frequent (30% to 79% of cases)
- Anti-signal recognition particle antibody positivityHPOHP:0033713
- Frequent (30% to 79% of cases)
- DysphagiaHPOHP:0002015
- Frequent (30% to 79% of cases)
- MyalgiaHPOHP:0003326
- Frequent (30% to 79% of cases)
- MyositisHPOHP:0100614
- Frequent (30% to 79% of cases)
- Proximal lower limb muscle weaknessHPOHP:0008994
- Frequent (30% to 79% of cases)
Show the remaining 12
- Skeletal muscle atrophyHPOHP:0003202
- Frequent (30% to 79% of cases)
- Abnormal pulmonary interstitial morphologyHPOHP:0006530
- Occasional (5% to 29% of cases)
- Axial muscle weaknessHPOHP:0003327
- Occasional (5% to 29% of cases)
- Chest painHPOHP:0100749
- Occasional (5% to 29% of cases)
- MyocarditisHPOHP:0012819
- Occasional (5% to 29% of cases)
- NeoplasmHPOHP:0002664
- Occasional (5% to 29% of cases)
Where it sits
Other names
6 names
Resolves to: immune-mediated necrotizing myopathy
- Also called
- anti-HMG-CoA myopathyanti-SRP myopathyautoimmune necrotizing myositisimmune myopathy with myocyte necrosisIMNMNAM