Antisynthetase syndrome (anti-Jo1)
Long standing history of diffuse symmetric shoulder and pelvic girdle muscle pain, presenting with a long history of dyspnea, cyanosis, and ineffective cough.
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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).
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.
- 1. Fraser RS, Muller NL, Colman NC et-al. Fraser and Pare's diagnosis of diseases of the chest. Saunders. ISBN:0721661947. Read it at Google Books - Find it at Amazon
- 2. Hidano A, Torikai S, Uemura T et-al. Malignancy and interstitial pneumonitis as fatal complications in dermatomyositis. J. Dermatol. 1992;19 (3): 153-60. Pubmed citation