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A3046 - Rapidly Fatal Anti Synthetase Syndrome Presenting with Pneumonia
Author Block: A. Nayfeh1, N. Addasi1, j. Macaraeg1, N. Jagan2, I. Berim1; 1Creighton University Medical Center, Chi Health Clinic, Omaha, NE, United States, 2Creighton University, Omaha, NE, United States.
Anti-synthetase syndrome (AS) is a rare systemic autoimmune syndrome characterized by an inflammatory myositis associated with interstitial lung disease (ILD) and anti-synthetase antibody.
49 years old male with past medical history of Hypertension and left leg osteosarcoma 30 years ago, presented to the ED with dyspnea, cough, fever and weight loss. He had two courses of oral antibiotics and steroids without improvement. In the ED, he was hypoxic. CXR showed bilateral lower lobe pneumonia. Blood workup showed elevated WBC at 32.5 k/ul (4-12), elevated creatinine at 1.4 mg/dl (0.6-1.3), elevated CPK at 785 u/l (39-308 u/l). Patient was started on broad spectrum antibiotics, oxygen, and IV fluid. Three days later, respiratory pathogen screen came positive for Enterovirus D68, and antibiotics were discontinued. Patient remained hypoxic, and chest CT showed bilateral air space disease. He improved and was eventually discharged. After 10 days, he came back to the ED with similar symptoms. CXR showed same changes. Blood tests showed WBC 32, CPK 2435, creatinine 1.9, elevated ESR, and CRP. The patient was admitted. Bronchoscopy showed mixed inflammatory cells. Rheumatological disease was suspected and work up showed positive ANA. However, ANCA, ENA, Anti-DNA, Anti Jo-1, GBM antibody, and complement level were within normal limits. Given his mild muscle weakness, muscle MRI was done and showed mild inflammation. Right vastus lateralis Muscle biopsy showed necrotizing myopathy with perivascular inflammation. He was started on prednisone 60 mg daily. Patient's cough and muscle weakness improved, but he remained hypoxemic. Lung biopsy showed chronic interstitial and organizing pneumonia. Anti-synthetase syndrome was suspected and further workup showed positive SSA, anti PL-7, and the diagnosis was confirmed. The patient was started on Mycophenolate, as a steroid sparing agent, considering the lack of significant response to steroids in two months. Unfortunately, his condition deteriorated rapidly, he developed diffuse alveolar hemorrhage, and died.
ILD is the major determinant of morbidity and mortality in patients with AS. Among patients with AS, 86% were reported to have ILD. Diagnosis depends on high index of clinical suspicion, and presence of anti-synthetase antibody. Anti- Jo1 positive patients more often develop severe myositis and joint manifestation, it usually presents 15-20% of the cases. Anti-PL7/PL12 positive patients more commonly experience early and severe ILD as in our patient. It usually presents in 5-10 % of the cases. Treatment of AS is challenging. However, glucocorticoids are the first line agent for patients with AS associated ILD.