Ro52, Myositis, and Interstitial Lung Disease

McHugh, Neil

doi:10.3899/jrheum.221067

Cited by 8 publications

(5 citation statements)

References 20 publications

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“…Die Prävalenz ist bei durch ASyS-Ak charakterisierten Patienten mit 40–70 % noch höher. Vielfach wurde berichtet, dass Anti-Ro-52-Ak dabei mit häufigerer und/oder rascher progredienter ILD, häufigeren Rezidiven und/oder höherer Mortalität assoziiert sind [ 10 ]. Somit zeigt der Nachweis von Anti-Ro-52 zusätzlich zu einem IIM-Antikörper eine generell schwerere (Lungen‑)Erkrankung an.…”

Section: Zusätzliche Iim-modifizierende Autoantikörperunclassified

Autoantibody diagnostics in idiopathic inflammatory myopathy

Biesen,

Schneider,

Lindae

et al. 2024

Z Rheumatol

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ZusammenfassungIdiopathische inflammatorische Myopathien (IIM) sind eine Gruppe seltener und heterogener Systemerkrankungen, die sich nicht nur an der Muskulatur, sondern auch an der Haut, den Gelenken und der Lunge manifestieren. Die Erstsymptome können isoliert und variabel auftreten, und so stellt die Diagnosefindung verschiedene Fachgruppen vor Herausforderungen. Da Autoantikörper mitunter die einzigen spezifischen Befunde sind, die zur Diagnose und einer angepassten Therapie führen, sind grundlegende Kenntnisse über diese unabdingbar. In dieser Arbeit erklären wir verfügbare Testsysteme, benennen die klinischen notwendigen Indikationen zur Veranlassung einer Autoantikörperdiagnostik, geben Informationen zu Etymologie, Antigenen, Synonymen und Erstbeschreibern, beschreiben die durch Myositisantikörper induzierte indirekte Immunfluoreszenz auf HEp-2-Zellen und geben die klinisch-serologischen Assoziationen wieder. Der Abgleich des Autoantikörperbefundes mit der klinischen Symptomatik und laborchemischen Befunden ermöglicht im Sinne einer Plausibilitätsprüfung, falsch positive oder auch falsch negative Laborbefunde zu identifizieren.

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Section: Zusätzliche Iim-modifizierende Autoantikörperunclassified

Autoantibody diagnostics in idiopathic inflammatory myopathy

Biesen,

Schneider,

Lindae

et al. 2024

Z Rheumatol

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“…This antibody not only commonly serves more frequently served as a serological marker for various autoimmune diseases, including Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus but also detected in patients with IIM. [16][17][18] Conversely, MSAs were specific to IIM. However, many individuals who lack typical characteristics, such as rash, arthritis, and myositis, often went unrecognized due to the absence of anti-ARS antibody testing.…”

Section: Discussionmentioning

confidence: 99%

“…The anti‐Ro52 antibody, recognized as one of the myositis‐related antibodies, was found in 76 out of 119 patients with ASS (63.9%) in our study. This antibody not only commonly serves more frequently served as a serological marker for various autoimmune diseases, including Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus but also detected in patients with IIM 16–18 . Conversely, MSAs were specific to IIM.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Zhang,

Wang,

Zhou

et al. 2023

Immunity Inflam & Disease

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ObjectivesTo identify the differences of clinical characteristics, laboratory findings, and the long‐term outcomes in patients with anti‐synthetase syndrome (ASS) of different anti‐aminoacyl‐transfer RNA synthetase antibodies.MethodsWe retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS.ResultsThe frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)‐ILD. The highest incidence of RP‐ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non‐ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti‐Jo1 antibody‐positive group were significantly higher than anti‐Jo1 antibody‐negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti‐nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP‐ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti‐Ro52 antibody‐positive group than the anti‐Ro52 antibody‐negative patients with ASS (all p < .05).ConclusionsPatients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti‐Ro52 antibody provides valuable insights for managing and predicting disease progression and long‐term outcomes.

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“…A study on pulmonary functions in PPF demonstrated an increased risk of developing PPF in patients with FVC <50% or DLCO SB% <35 ( 30 ). In addition to the aforementioned predictors, anti-Ro-52 and anti-MDA5 antibodies have also been recognized as risk factors for poorer prognosis ( 10 , 11 ). MDA5 is encoded by the interferon induced helicase C domain containing protein 1 gene and functions as an intracellular sensor for double-stranded RNA viral replication intermediates or byproducts.…”

Section: Discussionmentioning

confidence: 99%

“…Antifibrotic drugs such as pirfenidone or nintedanib are frequently added to slow down the rate of progression in this subset of patients. Several factors have been identified as predictors of a poor prognosis in patients with IIM-ILD, including acute onset of ILD, lower forced vital capacity (FVC) % predicted at ILD onset, positive anti-Ro-52 antibody, positive anti-MDA5 antibody, and the presence of usual interstitial pneumonia (UIP) (8)(9)(10)(11). The clinical characteristics of progressive fibrosis in patients with myositis-specific antibody-positive interstitial pneumonia (MSA-IP) have been described by clustering in our previous studies and demonstrated that anti-MDA5 positive and acute or subacute of ILD patients were prone to interstitial pneumonia progression (12).…”

Section: Introductionmentioning

confidence: 99%

Progressive pulmonary fibrosis in myositis-specific antibody-positive interstitial pneumonia: a retrospective cohort study

Wang,

Sun

et al. 2024

Front. Med.

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ObjectivesIdiopathic inflammatory myopathy (IIM) frequently coexists with interstitial pneumonia (IP) and is commonly the initial or sole manifestation accompanied by positive myositis-specific autoantibodies (MSAs), even in the absence of meeting diagnostic criteria. This study aims to evaluate the proportion of progressive pulmonary fibrosis (PPF) and identify potential predictors influencing the progression of pulmonary fibrosis in patients with MSA-IP.MethodsThis descriptive study employed a retrospective cohort design, enrolling patients diagnosed with interstitial pneumonia and positive MSAs at Beijing Chao-Yang Hospital in a sequential manner. Clinical data were systematically collected from the patients’ medical records during regular follow-up visits conducted every 3 to 6 months. Cox regression analysis was utilized to identify independent predictors of PPF in patients with positive MSAs and interstitial pneumonia.ResultsA total of 307 patients were included in the study, with 30.6% of them developing PPF during a median follow-up period of 22 months. Kaplan–Meier survival curves demonstrated a significantly lower survival in the PPF patients compared to the non-PPF patients (median 11.6 months vs. 31 months, p = 0.000). An acute/subacute onset of interstitial pneumonia (HR 3.231, 95%CI 1.936–5.392, p = 0.000), lower diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted (HR 6.435, 95%CI 4.072–10.017, p = 0.001), and the presence of diffuse alveolar damage (DAD) on high-resolution computed tomography (HRCT) (HR 8.679, 95%CI 1.974–38.157, p = 0.004) emerged as independent predictors of PPF. Notably, the implementation of triple therapy comprising glucocorticoids, immunosuppressants, and antifibrotic drugs was associated with a reduced risk of developing PPF (HR 0.322, 95%CI 0.115–0.899, p = 0.031).ConclusionApproximately 30.6% of patients with MSA-IP may develop PPF within the follow-up period. Patients presenting with an acute/subacute onset of interstitial pneumonia, lower predicted DLCO SB% and evidence of DAD on HRCT are more susceptible to developing PPF. Conversely, the administration of triple therapy appears to serve as a protective factor against the development of PPF in patients with MSA-IP.

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Ro52, Myositis, and Interstitial Lung Disease

Cited by 8 publications

References 20 publications

Autoantibody diagnostics in idiopathic inflammatory myopathy

Autoantibody diagnostics in idiopathic inflammatory myopathy

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Progressive pulmonary fibrosis in myositis-specific antibody-positive interstitial pneumonia: a retrospective cohort study

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