SESSION TYPE: ILD Student/Resident Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
PURPOSE: In myositis, the lung is commonly involved with associated interstitial lung disease (ILD). Anti synthetase syndrome (ASS) consists of ILD, arthritis, myositis, fever, mechanic’s hands and Raynaud’s phenomenon in the presence of anti aminoacyl-tRNA-synthetase antibodies (anti-ARS). We present a case of atypical fibrotic lung disease in a polymyositis patient.
METHODS: A 38-year-old female with no past medical history until six months ago when she had pneumonia related acute respiratory distress syndrome (ARDS), requiring nine days of mechanical ventilation. She was treated with a steroid taper. She had two subsequent hospitalizations for pneumonia. Cultures grew MAI and RSV. When being tapered off steroids, she had worsening dyspnea, productive cough, dysphagia along with muscle pain and weakness. On exam, she was afebrile with cushingoid features, saturating well on 2LPM oxygen, had bi-basal mid inspiratory fine rales and mild hand joint synovitis.
RESULTS: Chest imaging showed scarring at lung bases reflecting post ARDS change with possible superimposed infection. She was treated for possible aspiration pneumonia. She was positive for ANA and anti Jo-1 with an elevated creatine kinase (CK). Patient refused open lung biopsy. A presumptive diagnosis of antisynthetase syndrome was made and she was started on high dose steroids and then tacrolimus. Patient responded favorably with resolution of both muscle weakness and dyspnea.
CONCLUSIONS: ASS has no defined diagnostic criteria. Median age of onset is 51 years with female predominance. Antibodies described include anti Jo-1 (15-20%), PL-7(5-10%), PL-12(<5%), EJ, OJ, KS, Zo and YRS. ILD was more prominent with anti PL-12 and myositis was more common in anti Jo-1. Our patient was anti Jo-1 positive, had CK elevation and arthritis but did not have Raynauds or mechanic’s hands. Chest imaging was suggestive of possible post-residual ARDS but no defined ILD pattern. Clinical improvement with immunosuppression proves to be indicative of antisynthetase syndrome related ILD.
CLINICAL IMPLICATIONS: We propose that future studies could provide further insight into the pathogenesis and clinical consequences of ASS and define diagnostic criteria.
DISCLOSURE: The following authors have nothing to disclose: Dheeraj Dhotre, Muneeruddin Mohammed, John Youssef, Nevin Uysal-Biggs
No Product/Research Disclosure InformationMedical College of Wisconsin, Milwaukee, WI