Antisynthetase syndrome (anti-Jo1)

Case contributed by Dr Matt A. Morgan

Presentation

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: 45-50Y
Gender: Female
Modality: CT

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.

PlayAdd to Share

Case Information

rID: 31899
Case created: 4th Nov 2014
Last edited: 10th Sep 2015
System: Chest
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.