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Inflammatory myopathies (polymyositis and dermatomysotis) occur in distinct subsets based on antibody profiles. Antisynthetase syndrome is one of them.
Anti Jo-1 is by far the most common antisynthetase antibody but there are others.
Antisynthetase syndrome has a distinct clinical profile based on extramuscular manifestations.
Extramuscular manifestations (e.g. ILD, polyarthritis) may dominate the clinical picture or be the initial manifestation.
ILD is the most important extramuscular manifestation. Its histology (NIP) differs from that of IPF and is somewhat responsive to steroids and other immunomodulatory agents.
Pulmonary hypertension may be out of proportion to parenchymal lung involvement.
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