Presentation
Worsening dyspnea in a patient known to have anti-synthetase syndrome.
Patient Data
HRCT imaging performed in the prone position shows bilateral peripheral and basal regions of ground glass opacification in a peribronchial distribution, with mild architectural distortion, representing organizing pneumonia presentation of pulmonary involvement in a patient with antisynthetase syndrome (Anti-JO-1 Ab positive).
Case Discussion
Autoimmune inflammatory myositis which commonly has pulmonary involvement with interstitial lung disease (ILD).
Constellation of clinical findings including hand deformity (mechanic's hands), Raynaud's phenomenon and polyarthritis.
The pattern of ILD can be NSIP, organizing pneumonia or UIP, with a basal and peripheral distribution, which responds to steroid therapy.
Patient's can develop pulmonary hypertension.
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