Abstract
Background: Antisynthetase syndrome (ASyS) is a rare autoimmune disease characterized by inflammatory myopathy, arthritis, fever, and interstitial lung disease (ILD). Pulmonary involvement in ASyS significantly increases morbidity and mortality and, therefore, requires prompt and effective immunosuppressive treatment. Owing to the rarity of ASyS, limited data exists on progression and prognosis of ILD under immunosuppression.Objectives: The objective of the study was to evaluate the radiological progression and outcome measures of ILD with immunosuppressive therapy in patients with ASyS.Methods: Twelve patients with ASyS-associated ILD (ASyS-ILD) were included. Demographic and clinical data, including organ involvement, pulmonary function tests (PFT), laboratory parameters, imaging studies, and treatment regimens were retrospectively analyzed from routinely collected data. The extent of ground glass opacities, fibrotic changes and honeycombing was analyzed and scored using high-resolution chest computed tomography (HRCT) scans. HRCT findings were compared between baseline and follow-up examinations. In addition, patients were stratified depending on whether they had received rituximab (RTX) or not.Results: Pulmonary function tests revealed stable lung function and follow-up HRCT scans showed an improvement of radiological alterations in the majority of ASyS patients under immunosuppressive therapy. We did not detect significant differences between the RTX- and non-RTX-treated groups, but the RTX-treated patients more frequently had myositis and relapsing disease.Conclusions: Radiographic alterations in ASyS-associated ILD respond to immunosuppressive treatment. RTX is a feasible treatment option with similar clinical and radiographic outcomes in patients with relapsing disease and clinically apparent myositis.
Highlights
Antisynthetase syndrome (ASyS) is a rare autoimmune disease, belonging to the idiopathic inflammatory myopathies (IIM) [1]
In this single-center cohort study, we studied the effect of immunosuppression on high-resolution chest computed tomography (HRCT) findings in the course of ASyS-interstitial lung disease (ILD), focusing particular on RTX
We evaluated the presence and spectrum of specific abs, organ involvement, laboratory parameters, pulmonary function tests (PFT), and imaging procedures in each patient
Summary
Antisynthetase syndrome (ASyS) is a rare autoimmune disease, belonging to the idiopathic inflammatory myopathies (IIM) [1]. Due to frequent extramuscular manifestations, including fever, Raynaud’s syndrome, arthritis, mechanic’s hands and interstitial lung disease (ILD) [2, 3], ASyS is classified among the overlap myositis [4]. ASyS-ILD is the most important prognostic factor in these patients and lung involvement is associated with an increased risk of mortality, requiring prompt immunosuppressive treatment [7, 8]. Antisynthetase syndrome (ASyS) is a rare autoimmune disease characterized by inflammatory myopathy, arthritis, fever, and interstitial lung disease (ILD). Pulmonary involvement in ASyS significantly increases morbidity and mortality and, requires prompt and effective immunosuppressive treatment. Owing to the rarity of ASyS, limited data exists on progression and prognosis of ILD under immunosuppression
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
