Clinical characteristics of interstitial lung diseases positive to different anti-synthetase antibodies

Abstract

To analyze the clinical, serological, and imaging characteristics of patients with interstitial lung diseases (ILD) positive to different anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies. The clinical data, serological indexes, pulmonary high-resolution computed tomography (HRCT) imaging features and pulmonary functions, and bronchoalveolar lavage fluid of 84 ILD patients with anti-ARS antibody positive in Beijing Chao-yang Hospital, Capital Medical University were reviewed. (1) Anti-ARS antibodies included anti-Jo-1 (42.86%), anti-PL-7 (26.19%), anti-PL-12 (10.71%), anti-EJ (14.29%), and anti-OJ (5.95%). (2) Nonspecific interstitial pneumonia was the main type of patients with ILD positive to antibodies of anti-Jo-1, anti-PL-7, and anti-EJ, organizing pneumonia was the main type of patients with ILD positive to anti-PL-12 antibody and usual interstitial pneumonia was the main type of patients with ILD positive to anti-OJ antibody. (3) Only 14.29% of the patients had typical “triad syndrome” (interstitial pneumonia, myositis, and non-erosive arthritis). Myositis mainly occurred in patients with ILD positive to antibodies of anti-PL-7, anti-Jo-1, and anti-EJ. The incidence of arthritis in ILD patients with anti-Jo-1 was higher than that in ILD patients with anti-PL-12 and anti-EJ ( P < .05). The incidence of mechanic's hand in ILD patients with anti-Jo-1 was higher than that in ILD patients with anti-PL-12 ( P < .05). ILD positive to anti-Jo-1 antibody is associated with multiple organ involvement, mainly manifested as myositis, mechanic's hand, and arthritis. As other clinical manifestations of some ILD patients are relatively hidden, ILD patients should pay attention to the screening of the anti-ARS antibodies and guard against anti-synthetase syndrome.

Autoimmune pulmonary alveolar proteinosis exacerbated by steroid therapy due to misdiagnosis as anti-aminoacyl-tRNA synthetase (ARS) antibody positive- interstitial pneumonia: a case report

“…Anti-ARS antibodies, known to be associated with dermatomyositis and polymyositis, are also detected in 6.0-7.6% of patients with idiopathic interstitial pneumonia [5,6]. Among the anti-ARS antibodies detected in patients with idiopathic interstitial pneumonia, anti-PL-7 antibody is the second most common, after anti-Jo-1 [11]. Anti-ARS antibody-positive interstitial pneumonia is known to respond well to treatment with steroid and immunosuppressive agents [12].…”

A Rare Case of Anti-glycyl transfer RNA (tRNA) Synthetase Antibody-Related Non-specific Interstitial Pneumonia

“…In another study, the prevalence of NSIP reached 94.4% [ 9 ]. In a retrospective study conducted on 84 patients, an anti-EJ antibody was found in 14.29% of patients, and NSIP was the main type of clinical manifestation in the patients positive for anti-EJ antibody [ 6 ]. Taken together, these findings support the early consideration of anti-ARS antibodies when evaluating patients with clinical manifestations of ILD, which could prompt early diagnosis and appropriate treatment, potentially leading to better clinical outcomes.…”

“…In patients who are positive for anti-ARS antibody, the clinical manifestations of ILD are not well established. However, NSIP has been reported in patients with anti-Jo-1, anti-PL-7, and anti-EJ antibodies [ 6 ]. The present manuscript presents the clinical, laboratory, and radiological findings of a patient with anti-EJ antibodies to add to the current knowledge regarding the possible presentations of this syndrome.…”

A multidisciplinary approach to the diagnosis of antisynthetase syndrome

“…The classical triad of ILD, myositis, and arthritis was reported at first presentation (defined as the development of all three features within 3 months of first symptom onset) in only 5% of the large international American and European Network of Antisynthetase Syndrome (AENEAS) cohort, including anti-Jo1, anti-PL7, anti-PL12, anti-EJ, and anti-OJ antibody-positive patients and 15% of a Chinese cohort of antisynthetase antibody-positive ILD patients ( 16 , 17 ). In the AENEAS study, the onset was predominantly a single triad feature in all included antibody subgroups, suggesting the condition can frequently present as isolated ILD, myopathy, or arthritis.…”