Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease

Abstract

ObjectiveThis study aimed to analyze the clinical features and prognostic factors of imaging progression and survival in patients with antisynthetase syndrome (ASS) complicated by interstitial lung disease (ILD) in a large Chinese cohort.MethodsMedical records, imaging, and serological data of 111 patients with ASS-ILD (positive for at least one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ) from the Affiliated Yantai Yuhuangding Hospital of Qingdao University database were retrospectively investigated. According to the changes in high-resolution computed tomography (HRCT) outcomes at 1 year follow-up, Patients were categorized into three groups: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate the possible prognostic factors of ILD outcome and death, and multivariate analysis was performed to determine the independent predictors of ASS-ILD outcome and death by logistic regression.ResultsThe number of CD3-CD19+ cells and initial glucocorticoid dosage were correlated with imaging progression, and may be independent risk factors for ILD deterioration. Dyspnea as the first symptom, hypohemoglobinemia, the serum ferritin level, oxygen partial pressure at diagnosis, and different treatment types were important factors affecting survival, and the initial serum ferritin level may be an independent risk factor for survival.ConclusionsThe clinical characteristics of patients with ASS-ILD with different antisynthetase antibody subtypes are different. An increase in the CD3-CD19+ cell level is an independent risk factor for the deterioration of HRCT imaging. Early intensive treatment with high-dose glucocorticoids can effectively improve imaging prognosis of ILD. Patients with significantly elevated serum ferritin levels should be treated intensively.

Patient With Pulmonary Symptoms, Dysphagia, and Raynaud Disease

“…Antisynthetase syndrome is a rare autoimmune disease classified as a subgroup of idiopathic inflammatory myopathic disorders . A characteristic feature of antisynthetase syndrome is presence of an autoantibody to one of the aminoacyl–transfer RNA (tRNA) synthetases, which are intracellular enzymes that attach amino acids to tRNA during protein synthesis . Antisynthetase syndrome has been associated with HLA haplotype DRB1*0301 and with exposures to tobacco, cleaning chemicals, mold, bird droppings, and airborne particles from the September 11, 2001, World Trade Center attack .…”

“…The classic triad of antisynthetase syndrome consists of interstitial lung disease (ILD), myositis, and nonerosive arthritis. Although these 3 conditions develop in up to 90% of patients with antisynthetase syndrome, approximately 25% present only with symptoms of arthritis, 25% present with myositis, and 15% to 30% have ILD as their presenting manifestation . Other conditions associated with antisynthetase syndrome include Raynaud phenomenon, gastroesophageal reflux disease, fever, and fissured eruptions on the hands (“mechanic hands”) …”

“…1 A characteristic feature of antisynthetase syndrome is presence of an autoantibody to one of the aminoacyl-transfer RNA (tRNA) synthetases, which are intracellular enzymes that attach amino acids to tRNA during protein synthesis. 2,3 Antisynthetase syndrome has been associated with HLA haplotype DRB1*0301 and with exposures to tobacco, cleaning chemicals, mold, bird droppings, and airborne particles from the September 11, 2001, World Trade Center attack. 2 Of the 10 identified antiaminoacyl-tRNA synthetases, the most common are anti-Jo-1 antibody (60%), anti-PL-12 (17%), and anti-PL-7 (12%).…”

“…2 Of the 10 identified antiaminoacyl-tRNA synthetases, the most common are anti-Jo-1 antibody (60%), anti-PL-12 (17%), and anti-PL-7 (12%). 2,3 The classic triad of antisynthetase syndrome consists of interstitial lung disease (ILD), myositis, and nonerosive arthritis. Although these 3 conditions develop in up to 90% of patients with antisynthetase syndrome, approximately 25% present only with symptoms of arthritis, 25% present with myositis, and 15% to 30% have ILD as their presenting manifestation.…”

“…Although these 3 conditions develop in up to 90% of patients with antisynthetase syndrome, approximately 25% present only with symptoms of arthritis, 25% present with myositis, and 15% to 30% have ILD as their presenting manifestation. 2,3 Other conditions associated with antisynthetase syndrome include Raynaud phenomenon, gastroesophageal reflux disease, fever, and fissured eruptions on the hands ("mechanic hands"). 2,4,5 ILD affects approximately 70% to 95% of patients with antisynthetase syndrome and is the most common cause of morbidity and mortality.…”