Rare diseases · Sign or symptom

Anti-Mi2 antibody positivity

HP:0033554

What it means

The presence of autoantibodies in the blood circulation that react against the Mi-2 antigen.

The Mi-2 antigen is a nuclear antigen, component of the Nucleosome Remodelling-Deacetylase (NuRD) complex involved in transcription regulation. Autoantibodies targeting the Mi-2 nuclear antigen are a serologic hallmark of idiopathic inflammatory myopathies, with a diagnostic sensitivity and specificity of approximately 4-18% and 98-100%, respectively. They are most strongly associated with dermatomyositis and correlate with HLA-DR7.

Rare diseases that can present with this1

Sometimes5–29%

1

The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.

Part of the broader category

Anti-Mi2 antibody positivity

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This page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.