Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center

IntroductionIdiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involv...

Full description

Bibliographic Details
Main Authors: Josefin Marklund, Balsam Hanna, Tao Jin, Rille Pullerits
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-03-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2024.1340310/full
_version_ 1797238498477998080
author Josefin Marklund
Balsam Hanna
Tao Jin
Tao Jin
Rille Pullerits
Rille Pullerits
Rille Pullerits
author_facet Josefin Marklund
Balsam Hanna
Tao Jin
Tao Jin
Rille Pullerits
Rille Pullerits
Rille Pullerits
author_sort Josefin Marklund
collection DOAJ
description IntroductionIdiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involvement and distinct myositis autoantibodies in individuals with IIM in a single-center cohort.MethodsPatients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features.ResultsSeventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM (n = 23), DM (n = 21), ASS (n = 23), and IBM (n = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA (n = 53), myositis-associated antibodies, MAA (n = 33), both MSA + MAA (n = 24), MSA only (n = 29), MAA only (n = 9), no MSA, or MAA (n = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected.Discussion:The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.
first_indexed 2024-04-24T17:36:36Z
format Article
id doaj.art-d4fce541226f41159d1c672f021f0e3e
institution Directory Open Access Journal
issn 2296-858X
language English
last_indexed 2024-04-24T17:36:36Z
publishDate 2024-03-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Medicine
spelling doaj.art-d4fce541226f41159d1c672f021f0e3e2024-03-28T05:07:01ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2024-03-011110.3389/fmed.2024.13403101340310Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single centerJosefin Marklund0Balsam Hanna1Tao Jin2Tao Jin3Rille Pullerits4Rille Pullerits5Rille Pullerits6Department of Rheumatology, Sahlgrenska University Hospital, Gothenburg, SwedenDepartment of Rheumatology, Sahlgrenska University Hospital, Gothenburg, SwedenDepartment of Rheumatology, Sahlgrenska University Hospital, Gothenburg, SwedenDepartment of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy at University of Gothenburg, Gothenburg, SwedenDepartment of Rheumatology, Sahlgrenska University Hospital, Gothenburg, SwedenDepartment of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy at University of Gothenburg, Gothenburg, SwedenDepartment of Clinical Immunology and Transfusion Medicine, Sahlgrenska University Hospital, Gothenburg, SwedenIntroductionIdiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involvement and distinct myositis autoantibodies in individuals with IIM in a single-center cohort.MethodsPatients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features.ResultsSeventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM (n = 23), DM (n = 21), ASS (n = 23), and IBM (n = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA (n = 53), myositis-associated antibodies, MAA (n = 33), both MSA + MAA (n = 24), MSA only (n = 29), MAA only (n = 9), no MSA, or MAA (n = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected.Discussion:The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.https://www.frontiersin.org/articles/10.3389/fmed.2024.1340310/fullidiopathic inflammatory myopathymyositis-associated antibodymyositis-specific antibodyanti-Ro/SSA52lung involvement
spellingShingle Josefin Marklund
Balsam Hanna
Tao Jin
Tao Jin
Rille Pullerits
Rille Pullerits
Rille Pullerits
Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
Frontiers in Medicine
idiopathic inflammatory myopathy
myositis-associated antibody
myositis-specific antibody
anti-Ro/SSA52
lung involvement
title Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
title_full Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
title_fullStr Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
title_full_unstemmed Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
title_short Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis − a cohort study of 70 adult patients with myositis in a single center
title_sort myositis associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis a cohort study of 70 adult patients with myositis in a single center
topic idiopathic inflammatory myopathy
myositis-associated antibody
myositis-specific antibody
anti-Ro/SSA52
lung involvement
url https://www.frontiersin.org/articles/10.3389/fmed.2024.1340310/full
work_keys_str_mv AT josefinmarklund myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT balsamhanna myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT taojin myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT taojin myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT rillepullerits myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT rillepullerits myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter
AT rillepullerits myositisassociatedantibodiespredicttheseverityoflunginvolvementinadultpatientswithinflammatorymyositisacohortstudyof70adultpatientswithmyositisinasinglecenter