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Background Cancer‐associated myositis (CAM) has poor prognosis and causes higher mortality. In general, myositis‐specific autoantibodies (MSAs) and myositis‐associated autoantibodies (MAAs) have been shown to be useful biomarkers for its diagnosis. Methods In the present study, focus was given in assessing the presence, prevalence, and diagnostic values of myositis autoantibodies in Chinese patients diagnosed with CAM. The sera collected from 49 CAM patients, 108 dermatomyositis/polymyositis (DM/PM) patients without cancer, 105 disease controls, and 60 healthy controls were detected for the presence of 16 autoantigens (Jo‐1, OJ, EJ, PL‐7, PL‐12, MDA5, TIF1γ, Mi‐2α, Mi‐2β, SAE1, NXP2, SRP, Ku, PM‐Scl75, PM‐Scl100, and Ro‐52) using a commercial Euroline assay. Results The frequency of anti‐TIF1γ was significantly higher in CAM patients than in DM/PM patients without cancer (46.9% vs 14.8%, P < .001). Importantly, the sensitivity and specificity for this MSA were 46.9% and 85.2%, respectively. These helped to differentiate CAM patients from DM/PM patients without cancer. However, there was no difference in other MSAs and MAAs between CAM and DM/PM patients without cancer. Conclusion The present study indicates that anti‐TIF1γ levels can serve as important biomarkers for CAM diagnosis and help in distinguishing between CAM and DM/PM patients without cancer.

Background Anti‐signal recognition particle (anti‐SRP) antibodies are myositis‐specific autoantibodies associated with immune‐mediated necrotizing myopathy. This study was undertaken to better understand how patients with anti‐SRP antibodies have been managed at our tertiary centre and to assess the spectrum of clinical features and outcomes in routine clinical practice. Methods We conducted a retrospective evaluation of 25 patients with anti‐SRP antibodies identified via line‐blot immunoassay at a tertiary care centre (2019–2024). Demographic, clinical, serological, and imaging data were reviewed. Results The group of patients had a mean age of 60.1 years, with a female‐to‐male ratio of 10:15. Eight patients (32%) were diagnosed with myositis, primarily presenting with proximal muscle weakness. Interstitial lung disease was observed in 53% of the group of patients, and 50% of the subset of patients with myositis. Coexisting myositis‐specific autoantibodies were present in 32%, and 48% had positive antinuclear antibody titres (≥ 1:400). Cardiac involvement was reported in two myositis patients. Corticosteroids, often combined with mycophenolate mofetil or other immunosuppressants, formed the basis of treatment. Conclusion Anti‐SRP antibodies are associated with a heterogeneous clinical spectrum, with many patients lacking myositis features. There was a high prevalence of coexisting autoantibodies. Further studies are needed to elucidate the pathogenic role of anti‐SRP and optimise management strategies.

Dermatomyositis (DM) is a systemic disease characterized by chronic inflammation in the skin and muscle. A variety of myositis‐specific autoantibodies (MSAs) are detected in patients with DM. These antibodies are associated with unique clinical subsets in DM. Panniculitis is a rare cutaneous manifestation of DM that most commonly presents as tender, erythematous subcutaneous nodules on the thighs, arms, and buttocks. We herein describe the clinical features of two DM‐associated panniculitis patients with anti‐TIF1 antibodies. Both are female patients and showed characteristic cutaneous features, including heliotrope rashes and Gottron's signs with muscle involvement. While one patient had a posterior mediastinum tumor, another had no cancer. Furthermore, to elucidate the clinical significance of anti‐TIF1 antibodies in DM patients with panniculitis, we analyzed all cases with detailed information on the clinical features, including MSAs. A PubMed search of keywords “panniculitis” and “DM” was conducted. We found 23 cases with the detection of MSAs. A review of these cases and our two cases revealed a predominance of female gender (80%) with a median age of 48 years. Anti‐MDA5 antibodies were detected in 11 patients (11/23, 48%), and anti‐TIF1 antibodies were observed in four patients, including the current cases (4/23, 17%). Anti‐Mi‐2 antibodies were observed in four patients (4/23, 17%). Each patient had anti‐NXP‐2 antibodies or anti‐SAE antibodies (2/23; 9%). Although the pathogenesis of panniculitis in DM remains unknown, differences in pathogenesis of DM‐associated panniculitis according to MSAs may exist. Accumulation of additional cases is required to clarify the relationship between panniculitis and MSAs. Panniculitis is a rare cutaneous manifestation of DM. We herein describe the clinical features of two DM‐associated panniculitis patients with anti‐TIF1 antibodies. Although the pathogenesis of panniculitis in DM remains unknown, differences in pathogenesis of DM‐associated panniculitis according to MSAs may exist.

[This corrects the article DOI: 10.3389/fimmu.2025.1674437.].

Background Cancer is a significant complication contributing to increased mortality in idiopathic inflammatory myopathies (IIMs), and the association between IIMs and cancer has been extensively reported. Myositis-specific autoantibodies (MSAs) can help to stratify patients into more homogeneous groups and may be used as a biomarker for cancer-associated myositis. In this study, we aimed to systematically define the cancer-associated MSAs in IIMs. Methods Serum from 627 patients with IIMs was tested for MSAs. The cancer risk with different MSAs was estimated by standardized incidence ratio (SIR). Paraneoplastic manifestation, such as the close temporal relationship between myositis onset and cancer diagnoses in patients with different MSAs, was also evaluated. Results Compared with the general Chinese population, patients with IIMs and anti-transcriptional intermediary factor (TIF1)-γ antibodies (SIR = 17.28, 95% CI 11.94 to 24.14), anti-nuclear matrix protein (NXP2) antibodies (SIR = 8.14, 95% CI 1.63 to 23.86), or anti-SAE1 antibodies (SIR = 12.92, 95% CI 3.23 to 32.94), or who were MSAs-negative (SIR = 3.99, 95% CI 1.96 to 7.14) faced increased risk of cancer. There was no association between specific MSAs subtypes and certain types of cancer. Paraneoplastic manifestations were observed in the patients carrying anti-TIF1-γ, as well as other MSAs. There were no prognostic differences among the patients with cancer-associated myositis (CAM) from different MSAs subgroups. However, in comparison to those with cancer unrelated to myositis, CAM had a worse prognosis, with an age-adjusted and sex-adjusted Cox hazard ratio (HR) of 10.8 (95% CI 1.38-84.5, p  = 0.02) for all-cause mortality. Conclusions Our study demonstrates in what is, to our knowledge, the largest population examined to date, that anti-SAE1, and previously reported anti-TIF1-γ and anti-NXP2 antibodies, are all associated with an increased risk of cancer in patients with IIMs. Moreover, our data suggest that in some cases, anti-HMGCR, anti-Jo-1 and anti-PL-12 antibody production might also be driven by malignancy. This can aid in the etiologic research of paraneoplastic myositis and clinical management.

ObjectivesThe aim of this study is to analyze the prevalence of myositis-specific autoantibodies (MSAs) and to elucidate their associations with clinical features in Chinese patients with polymyositis (PM) and dermatomyositis (DM).MethodsTwelve subsets of MSAs including anti-Mi-2, anti-TIF1-γ, anti-MDA5, anti-NXP2, anti-SAE1, anti-SRP, anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, and anti-HMGCR antibodies were tested. Four hundred and ninety-seven PM/DM patients were enrolled. Clinical features and laboratory data were collected. The frequency of MSAs and the correlations with clinical phenotypes were calculated by SPSS 21.0.ResultsMSAs were present in 65.4% in PM/DM patients. Anti-TIF1-γ (14.3%), anti-MDA5 (12.5%), and anti-Jo-1 (10.1%) were the three commonest MSAs. Anti-SAE1 (OR 14.877, 95% CI 1.427–155.074), anti-SRP (OR 4.339, 95% CI 1.529–12.312) and anti-TIF1-γ (OR 2.790, 95% CI 1.578–4.935) were associated with dysphagia. In contrast, anti-MDA5 (OR 0.356, 95% CI 0.148–0.856) might decrease the frequency of this manifestation. Interstitial lung disease (ILD) was observed more frequently in patients carrying anti-EJ (OR 14.202, 95% CI 1.696–118.902), anti-Jo-1 (OR 11.111, 95% CI 3.306–37.335), and anti-MDA5 (OR 3.109, 95% CI 1.578–6.128). On the contrary, anti-Mi-2 (OR 0.180, 95% CI 0.055–0.589), anti-TIF1-γ (OR 0.163, 95% CI 0.080–0.333), and anti-HMGCR (OR 0.058, 95% CI 0.007–0.451) were protective factors against developing ILD. Anti-TIF1-γ was an independent risk factor for cancer-associated myositis (OR 4.237, 95% CI 1.712–10.487).ConclusionsPM/DM patients had high frequencies of MSAs. Several MSAs were independent factors in determining unique clinical phenotypes.

Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. The management of PM-/DM-/CADM-associated ILD (PM/DM/CADM-ILD) requires careful evaluation of the disease severity and clinical subtype, including the ILD forms (acute/subacute or chronic), because of the substantial heterogeneity of their clinical courses. Recent studies have highlighted the importance of myositis-specific autoantibodies’ status, especially anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl tRNA synthetase (ARS) antibodies, in order to evaluate the clinical phenotypes and treatment of choice for PM/DM/CADM-ILD. Because the presence of the anti-MDA5 antibody is a strong predictor of a worse prognosis, combination treatment with glucocorticoids (GCs) and calcineurin inhibitors (CNIs; tacrolimus (TAC) or cyclosporin A (CsA)) is recommended for patients with anti-MDA5 antibody-positive DM/CADM-ILD. Rapidly progressive DM/CADM-ILD with the anti-MDA5 antibody is the most intractable condition, which requires immediate combined immunosuppressive therapy with GCs, CNIs, and intravenous cyclophosphamide. Additional salvage therapies (rituximab, tofacitinib, and plasma exchange) should be considered for patients with refractory ILD. Patients with anti-ARS antibody-positive ILD respond better to GC treatment, but with frequent recurrence; thus, GCs plus immunosuppressants (TAC, CsA, azathioprine, and mycophenolate mofetil) are often needed in order to achieve favorable long-term disease control. PM/DM/CADM-ILD management is still a therapeutic challenge for clinicians, as evidence-based guidelines do not exist to help with management decisions. A few prospective clinical trials have been recently reported regarding the treatment of PM/DM/CADM-ILD. Here, the current knowledge on the pharmacologic managements of PM/DM/CADM-ILD was mainly reviewed.

This study aimed to investigate the profiles of myositis-specific autoantibodies (MSA) and their correlation with distinct clinical features in patients with idiopathic inflammatory myopathy (IIM) in southern China. We retrospectively analyzed the medical records of 208 IIM patients, collecting data on their demographic variables, clinical manifestations, comorbidities, and MSA test results. Of the 208 patients, 185 were positive for MSAs. 69 patients were anti-MDA5 positive, 61 patients were anti-ARS positive followed by anti-SRP (34), anti-TIF1-γ (26), anti-Mi-2β (10), anti-NXP2 (10), anti-HMGCR (9), anti-Mi-2α (6), anti-cN-1A (6), and anti-SAE1 (1). Distinct clinical phenotypes were strongly associated with specific antibodies. Anti-MDA5 positive patients had shorter disease duration, less muscle involvement, but higher rates of rash, alopecia, arthritis, fever, and ILD with poorer prognosis. Anti-ARS positive patients had longer disease duration, mechanic’s hands, arthritis, fever, and ILD, but better prognosis. Both anti-MDA5 and anti-ARS antibodies were independent risk factors for developing ILD. Anti-TIF1-γ and anti-Mi-2 were most detected in IIM patients combined with malignancies, and nasopharyngeal carcinoma was the most common malignant tumor. Furthermore, hyperlipidemia and elevated cardiac biomarkers were frequently observed, particularly in patients positive for anti-SRP. The 3-month survival rate for anti-MDA5 positive patients was 87.8%, with all deaths attributed to rapidly progressive-ILD (RP-ILD). In contrast, other antibody positive patients had a 100% survival rate. This comprehensive analysis of a southern Chinese IIM cohort underscores that MSA profiles can effectively stratify patients into clinically distinct subgroups, which is crucial for predicting specific organ involvement, prognosis, and developing tailored treatment strategies.

The clinical features of interstitial lung disease (ILD) in patients with dermatomyositis (DM) and negative myositis autoantibodies had not been exactly demonstrated previously. This study aimed to describe and expand the phenotype of interstitial lung disease (ILD) in this cohort of patients. A total of 1125 consecutive Chinese patients with idiopathic inflammatory myopathies (IIM) between 2006 and 2020 were screened retrospectively. All proven cases of isolated ILD with both negative myositis-specific autoantibodies (MSA) and negative myositis-associated autoantibodies (MAA) were selected for inclusion. The clinical features and outcome among this group, MDA5 DM (DM patients with positive anti-MDA5 antibody) and ASS (patients with positive anti-aminoacyl tRNA synthetases antibodies were recorded and compared. Of 1125 IIM patients with an average follow-up of 6 years, 154 DM patients with negative MSA and MAA (MSA/MAA) were identified, with an ILD incidence of 46.8%. DM-ILD Patients with negative MSA/MAA presented younger age at onset (p<0.001), lower incidence of elevated CA153 (p=0.03) and fever (p=0.04)than those ILD patients with MDA5 DM and ASS.The estimated high-resolution computed tomography patterns of ILD showed non-specific interstitial pneumonia (66.6%), followed by organizing pneumonia in patients with negative MSA/MAA. OP pattern was more common in patients with MDA5 DM (69.7%), and the ratios of the OP (48.7%) and NSIP (51.3%) patterns were almost equal in patients with ASS. Of these DM-ILD patients with negative MSA/MAA, 25% developed rapidly progressive interstitial lung disease (RP-ILD). Patients with RP-ILD had a shorter disease duration (p=0.002), higher percentage of positive ANA(p=0.01) and organizing pneumonia patterns (p=0.04), elevated CYFRA211(p=0.04) and decreased FiO2/PaO2 (p<0.001) than those with chronic progressive ILD. The incidence of OP pattern in RP-ILD patients with negative MSA/MAA was lower than in those RPILD patients with MDA5 DM (75%) and ASS (89%) (p=0.006). The cumulative 5- and 10-year survival rates in the DM-ILD patients with negative MSA/MAA were 91% and 88%, respectively, during the long-term follow-up study. And they had more favorable survival rate compared with ILD patients with MDA5 DM and ASS (p<0.001). An independent prognostic factor was identified as decreased PaO2/FiO2 (hazard ratio, 0.97; p=0.004]. This study indicates DM-ILD patients with negative MSA/MAA had favorable long-term outcomes. Decreased baseline PaO2/FiO2 acted as an independent prognostic factor for this group of patients.

Background The increased availability of myositis autoantibodies represents new possibilities and challenges in clinical practice (Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76:1955–64. https://doi.org/10.1136/annrheumdis-2017-211468 .). The aim of this study was to perform a retrospective data analysis of patient cases with positive myositis autoantibodies to analyse their significance in routine rheumatology practice. Methods A monocentric analysis of all the orders used to determine myositis autoantibodies from July 2019 to May 2022 in the Department of Rheumatology, Krankenhaus Porz am Rhein, Cologne, Germany, was carried out. Results In the defined time interval, a total of 71,597 laboratory values for the antibodies mentioned above were obtained. A total of 238 different positive autoantibodies ​​were detected in 209 patients. Idiopathic inflammatory myopathy was diagnosed in 37 patients (18%), and inflammatory rheumatic diseases other than idiopathic inflammatory myopathy were diagnosed in 90 patients (43%). No inflammatory rheumatic disease was diagnosed in 82 patients (39%). General clusters of clinical manifestations were observed. Conclusions In our cohort, we were able to show that a relevant proportion of patients with positive myositis antibodies did not have idiopathic inflammatory myopathies or inflammatory rheumatic diseases. This finding indicates the importance of myositis autoantibodies in this group of patients. However, further studies on the course of symptoms and examination results in patients without inflammatory rheumatic diseases and with positive myositis antibodies are necessary.