Inflammatory myopathies & the antisynthetase syndrome

Great discussion on inflammatory myopathies with Dr. Kazi during today’s CUH morning report!

Did you know that patients with inflammatory myopathies such as dermatomyositis/polymyositis with antisynthetase antibodies (e.g., Anti-Jo-1) have high risk of developing interstitial lung disease?

Antisynthetase syndrome is defined as:

  • Presence of at least 1 anti-synthetase Ab
    • AND 
  • ≥1 of the following: 1) inflammatory myopathy; 2) inflammatory polyarthritis; 3) interstitial lung disease. 

Other features to support the diagnosis include: “mechanic’s hands”, Raynaud’s, fevers

Certain antisynthetase antibodies (Anti-PL-12) are associated with ILD as the dominant clinical feature.

Links to a couple NEJM articles below:


Inflammatory Muscle Diseases – NEJM, 2015

NEJM Case: A 21-Year-Old Man with Fevers, Arthralgias, and Pulmonary Infiltrates