Anti-synthetase syndrome

Case contributed by Dr Rachael O'Rourke

Presentation

Worsening dyspnoea in a 51 year-old male patient known to have anti-synthetase syndrome.

Patient Data

Age: 51
Gender: Male
Modality: CT

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 organising 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, organising pneumonia or UIP, with a basal and peripheral distribution, which responds to steroid therapy. 

Patient's can develop pulmonary hypertension.

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Case Information

rID: 48398
Case created: 5th Oct 2016
Last edited: 7th Oct 2016
System: Chest
Inclusion in quiz mode: Excluded

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