Anti-synthetase syndrome (anti-Jo1)

Case contributed by Matt A. Morgan


Long standing history of diffuse symmetric shoulder and pelvic girdle muscle pain, presenting with a long history of dyspnea, cyanosis, and ineffective cough.

Patient Data

Age: 45 years
Gender: Female

Extensive interstitial changes, including fibrosis with honeycombing, small interstitial nodules, prominent peribronchoarterial thickening, and traction bronchiectasis. There are parenchymal changes as well, with ground glass attenuation and patchy areas of consolidation (likely organizing pneumonia).

Case Discussion

Dermatomyositis and polymyositis are associated with the development of antibodies to nuclear and cytoplasmic antigens. One group of these autoantibodies, the antisynthetases, is associated with interstitial lung disease (80% of the patients with associated interstitial lung disease have these autoantibodies).  The most common antisynthetase antibody is Anti-Jo1, which was positive in this patient.

Antisythetase lung disease results in a nonspecific diffuse interstitial lung disease with superimposed consolidations from organizing pneumonia.

The 5-year mortality rate in one series of 182 patients who had dermatomyositis followed in Japan was 27%. The most common causes of death were malignancy and interstitial lung disease.

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