Clinical Analysis Of17Cases Of Anti-Jo-1Antibody Syndrome And Literature Review

Objective: A retrospective clinical study of17cases of anti-Jo-1antibodysyndrome was made in the field of its clinical features, diagnosis, pathogenesis andtreatments, to elucidate the importance of earlier diagnosis and earlier treatment foranti-Jo-1antibody syndrome.Methods:17patients with definite anti-Jo-1antibody syndrome wereretrospectively studied, and the clinical date about their constitutional symptoms,clinical manifestations, supplementary exams and therapy were reviews andanalyzed.Results: Anti-Jo-1ASS affected females (11/16,64.7%) and males (6/17,35.3%)aged between33and69years, with average disease duration of25.7±29.9months.Disease recurrence was observed in4patients.8cases showed myositis atdisease onset and6showed articular symptoms.2patients showed bothforementioned symptoms. The clinical manifestation was relatively homogeneouswith one or more of the following features: myositis, interstitial pulmonary diseaseand articular involvement. The presence of fever, Reynaud’s phenomenon and"mechanical hands" may also be observed. All the cases had interstitial pulmonarydisease. Pulmonary hypertension was usually observed in cardiac involvement cases.The presence of anti-Ro antibody （11/17,64.7%）was associated to pulmonaryfibrosis in ASS. Only one patient was accompanied by nasopharyngeal carcinoma. Conclusions: Patients with anti-Jo-1antibody syndrome usually develope mildmyositis or arthritis at disease on set, and are usually misdiagnosed as rheumatoidarthritis. In anti-Jo-1antibody syndrome, the incidence of interstitial pulmonarydisease without myositis is high and is the leading cause of death. High-resolutionCT(HRCT) chest examination offers additional details to confirm ILD. The presenceof anti-Ro antibody was associated to pulmonary fibrosis in anti-Jo-1ASS. Patientswith anti-Jo-1antibody carried a low risk for malignancies.Treatment ofcorticosteroid plus immunosuppressive drugs is effective.