Raynaud Phenomenon in Antisynthetase Syndrome Treated With Epoprostenol.

T opoisomerase syndrome comprises a series of recently identified series of rheumatologic syndromes due to autoantibodies directed to aminoacyl-tRNAs synthetases. Probably the most well known of these syndromes are the idiopathic inflammatory myopathies driven by the Jo-1 antibody, directed against the histidyltRNA synthetase and representing up to approximately 30% of cases. Several similar autoimmune syndromes have been discovered as a continuum of “antisynthetase syndrome,” which target these molecules. In a rare subtype, antibodies target anti–threonyltRNA synthetase or anti–PL-7, which presents as myositis, ILD, arthritis, and Raynaud phenomenon. A 74-year-old white woman with a medical history of hypothyroidism, atherosclerosis of the aorta, osteoporosis, hyperlipidemia, and spondylosis of the lumbar spine presented to her primary care provider at an outside hospital in June 2018 for shortness of breath. She was prescribed doxycycline and sent home with a diagnosis of bronchitis, but her shortness of breath did not improve. During the same period, she also developed painful tingling in her fingers, difficulty swallowing, and hyperkeratosis with fissuring of her hands (mechanic's hands). Further workup with a computed tomography scan showed a “diffuse thickening of the intralobular septa throughout the lungs most pronounced in the lung bases with developing honeycombing,” consistent with pulmonary fibrosis. She underwent a bronchoscopy and biopsy, which demonstrated pathologic findings consistent with bronchiolitis obliterans organizing pneumonia (Fig. A and B). Laboratory tests were significant for erythrocyte sedimentation rate of 9, C-reactive protein less than 1.0, negative ANAwith normal C3 and C4, and a negative anti-Ro/anti-La. She was found to be positive for the anti-PL7 antibody (antithreonyl tRNA synthetase). Further autoimmune and thrombotic workup did not show significant findings. She was started on steroids but failed to improve, and by August 2018, she presented for continued ischemia with associated dry gangrene in the distal digits of her arms. Transthoracic echocardiogram showed an increased systolic pulmonary artery pressure to 40 mm Hg, tricuspid annular plane systolic excursion of 2 cm, tricuspid annular systolic velocity of 13 cm/s with normal left heart function, and a trivial pericardial effusion. Computed