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Interstitial Lung Disease (ILD) is one of the most common causes of mortality in idiopathic Inflammatory Myopathies (IIM). Despite these conditions being commonly associated with proximal weakness, skin rashes and arthritis, ILD can be the first or the sole clinical feature in up to 60% of patients, potentially leading to incorrect diagnosis. The early recognition of an underlying IIM in ILD patients can allow for prompt treatment, which could potentially stabilize or even improve the lung disease, also avoiding the development of other clinical features associated with the condition. The objective of this review is to describe the clinical, serological and radiological features associated with IIM-ILD, mainly focusing on dermatomyositis and antisynthetase syndrome. [ABSTRACT FROM AUTHOR]

Objectives Anti-synthetase syndrome (ASS) is a chronic autoimmune condition, with interstitial lung disease (ILD) being a key feature. This systematic literature review (SLR; CRD42023416414) aimed to summarize treatments and outcomes of ILD associated with ASS (ASS-ILD). Methods Databases were searched for articles discussing ASS-ILD management and outcomes, published 1946–September 2023. Screening and data extraction were performed by two reviewers. Meta-analysis, using a random effects model, and paired t -tests were undertaken where appropriate to evaluate post-treatment-change in pulmonary function tests. Results Ten articles were included, comprising 514 patients: 67.8% female, mean age 52.4 years (s. d. 4.6). Baseline high-resolution CT was documented in 447 patients (86.9%); the most common pattern was non-specific interstitial pneumonia (n  = 220; 49.2%). The most common myositis-associated autoantibody was anti-Jo1 (48%), with 27.8% having associated anti-Ro52 antibodies. Pooled estimate, after meta-analysis, for baseline forced vital capacity (FVC) was 60.8% predicted (s. e. 2.1) and for diffusion capacity of lungs for carbon monoxide (DLco) was 49.8% (s. e. 3.5). After 1 year, pooled improvement in FVC was 14.1% from baseline (s. e. 3.1) and in DLco was 15.1% (s. e. 2.8). Paired t -test demonstrated significant overall improvement in FVC (P  = 0.007) and DLco (P  = 0.002). Patients receiving rituximab had 12.2% improvement in FVC and 2.9% increase in DLco at 1 year; for patients receiving CYC, there was 17% improvement and 6.3% increase, respectively. Twenty-eight deaths were reported. Conclusion Our SLR, the first to summarize management and outcomes of ASS-ILD, found no conclusive difference between effectiveness of treatments. More robust trials are required to reduce morbidity and mortality resulting from ASS-ILD. [ABSTRACT FROM AUTHOR]

Objective Anti-synthetase syndrome (ASyS) patients have heterogeneous clinical manifestations with different initial presentations, complications and outcomes. This study aimed to assess the clinical characteristics and complications in patients with ASyS, and to identify factors that were associated with the survival of ASyS patients. Methods This was a retrospective multicentre longitudinal study. Patients fulfilling either Connor's criteria or Solomon's criteria for ASyS were recruited. Electronic health records were reviewed until October 2022. Multivariate Cox regression analysis was used to determine the independent prognostic factors. Auto-antibodies were checked by commercial immunoassays. Results A total of 205 patients (anti-Jo1 49.3%, anti-PL7 19.0%, anti-EJ 11.2%, anti-PL12 10.2% and anti-OJ 3.4%) were included. The median follow-up time was 4 years. The time from symptoms onset to diagnosis was significantly longer for non-anti-Jo1 patients (median 5 vs 3 months). Common initial presentations included myositis (56.1%), arthritis (54.6%) and interstitial lung disease (ILD) (54.1%). Patients with anti-Jo1 had significantly higher muscle enzyme levels and more arthritis. All patients with anti-EJ would develop ILD on follow-up and malignancy was noted in 28.6% of the anti-OJ positive patients; 15.6% of the patients died and pulmonary diseases (ILD or pneumonia) were the major causes. Age at diagnosis, malignancy and rapidly progressive ILD were independently associated with mortality, while joint manifestation was a protective factor. Conclusion In view of the heterogeneity of clinical presentation of ASyS, a high index of suspicion and early checking of specific autoantibodies might help prompt diagnosis of ASyS and detection of related complications. [ABSTRACT FROM AUTHOR]

Purpose of Review: This review aims to provide a comprehensive and updated overview of autoimmune myopathies, with a special focus on the latest advancements in understanding the role of autoantibodies. We will begin by examining the risk factors and triggers associated with myositis. Next, we will delve into recent research on how autoantibodies contribute to disease pathogenesis. Finally, we will explore the latest innovations in treatment strategies and their implications for our understanding of myositis pathogenesis. Recent Findings: Recent research has revealed that myositis-specific autoantibodies can infiltrate muscle cells and disrupt the function of their target autoantigens, playing a crucial role in disease pathogenesis. Significant advances in treatment include CD19 CAR-T cell therapy, JAK-STAT inhibitors, and novel strategies targeting the type 1 interferon pathway in dermatomyositis. Additionally, the ineffectiveness of complement inhibitors in treating immune-mediated necrotizing myositis has challenged established views on disease mechanisms. Summary: Autoimmune myopathies are a collection of disorders significantly influenced by specific autoantibodies that drive disease pathogenesis. This review highlights the critical role of autoantibody research in deepening our understanding of these conditions and discusses recent therapeutic advancements targeting key pathogenic pathways. [ABSTRACT FROM AUTHOR]

Anti-synthetase syndrome constitutes a dynamically evolving subset of Idiopathic Inflammatory Myopathy, however, the nomenclature and abbreviations for this syndrome are plagued by heterogeneity, leading to lack of consistency in literature. The objective of this study is to evaluate existing diversity in disease names and abbreviations, with a future goal to develop consensus on the nomenclature. A scoping review format was used for analysis. A comprehensive PUBMED search was conducted from January 1, 1984 (the initial description of anti-synthetase autoantibodies) to November 30, 2023, encompassing all pertinent articles published within this timeframe. Search terms included, ((antisynthetase syndrome) OR (anti synthetase syndrome)) OR (anti-synthetase syndrome)). The articles were screened for presence of terminology and abbreviations used. The search yielded 936 items with the specified terms. After excluding 303 irrelevant articles and 58 non-English publications, the remaining n = 575 articles underwent detailed review of the abstract and full article. Out of n = 575, 54.7% (n = 314) used 'antisynthetase syndrome' and 43.4% (n = 249) preferred 'anti-synthetase syndrome' with few novel names also. Among these, 394 articles used abbreviations while 181 did not. Most utilized term was ASS; in 64.7% (n = 255), followed AS in 11.9% (n = 47), ASSD in 9.9% (n = 39) and ASyS in 7.6% (n = 30). A discordance in nomenclature is evident, with about half using antisynthetase syndrome and other half using anti-synthetase syndrome. Moreover, significant heterogeneity exists in abbreviation use aswell. There is a pressing need to bridge this disparity and establish a uniform identifier for the disease with an objective to develop greater coherence in future research, educational initiatives, and interdisciplinary collaboration. [ABSTRACT FROM AUTHOR]

Background: The presence of the rs35705950 variant in the MUC5B gene promoter is a critical genetic risk factor in idiopathic pulmonary fibrosis (IPF). It has been associated with usual interstitial pneumonia (UIP) in several interstitial lung diseases (ILDs). In antisynthetase syndrome (ASSD), most high-resolution computed tomography (HRCT) patterns are inflammatory, but up to 13% have UIP, leading to a worse prognosis. Methods: This single-center study included 60 patients with ASSD-ILD. We investigated whether carrying the MUC5B rs35705950 promoter variant was associated with UIP. To estimate the strength of the association between the genotype of the MUC5B rs35705950 promoter variant and the fibrotic pattern we used the odds ratio (cOR), and to assess the effect of confounding variables (age, evolution time, and sex), we performed a logistic regression to obtained the adjusted odds ratio (aOR). Results: The GT genotype of the MUC5B rs35705950 promoter variant is associated with up to a 4-fold increased risk of UIP (cOR 5.0, 95% CI 1.13–22.10), and the effect was even maintained after adjusting for potentially confounding variables such as sex, age, and time to progression (aOR 5.2, 95% CI 1.04–25.89). Conclusions: our study supports the role of MUC5B rs35705950 in ASSD-ILD with UIP. It reinforces that this polymorphism in our population could have a similar genetic basis to that already described in other ILDs that present predominantly fibrotic patterns. [ABSTRACT FROM AUTHOR]

Objective: Anti–histidyl‐transfer RNA synthetase (Jo‐1) antibodies are associated with myositis as well as different extramuscular organ complications comprising the anti‐synthetase syndrome. This study aimed to clarify the relationship between anti–Jo‐1 epitope recognition patterns and specific clinical features of this syndrome. Methods: B cell epitope mapping was performed via enzyme‐linked immunosorbent assay in 180 patients who were anti–Jo‐1 antibody‐positive using overlapping peptides/protein fragments spanning the amino‐terminal 151 amino acids of Jo‐1 as substrate antigens. Statistical associations with clinical features were assessed through rank‐sum, correlation, and cluster analyses. Results: The level of reactivity against subfragments spanning amino acids 1–151 of Jo‐1 paralleled that of full‐length Jo‐1, confirming the immunodominance of this amino‐terminal region. The corresponding frequencies of reactivity to peptides 1 (amino acids [aa] 1–21), 3 (aa 27–47), 4 (aa 40–60), 10 (aa 118–138), and 11 (aa 131–151) were 6.1%, 42.5%, 6.8%, 6.7%, and 20.3%. While anti–full‐length Jo‐1 antibodies were significantly associated with Raynaud phenomenon, anti‐fragment A2 (aa 1–60) and A3 (aa 1–90) antibodies were associated with proximal muscle weakness, Raynaud phenomenon, arthritis, and sicca syndrome. Anti‐fragment A4 (aa 1–120) and A5 (aa 1–151) antibodies were also associated with sicca syndrome. Peptide 1 (aa 1–21) antibodies were associated with Raynaud phenomenon and dysphagia. Whereas anti‐peptide 3 (aa 27–47) antibodies were also linked to Raynaud phenomenon, anti‐peptide 9 (aa 105–125) antibodies were associated with mechanic's hands. Conclusion: Autoantibodies targeting different amino‐terminal subfragments and/or peptides of Jo‐1 were associated with specific clinical features of the anti‐synthetase syndrome, demonstrating the biomarker potential of B cell epitope profiling in this disorder. [ABSTRACT FROM AUTHOR]

We present a case of interstitial lung disease arising in the course of antisynthetase syndrome (ASSD) in a patient with axial spondyloarthritis (ax-SpA) undergoing tumor necrosis factor-α (TNF-α) inhibitors therapy. Only two cases of ASSD in ax-SpA patients have been described in the literature, although with a different autoantibody profile. Only in one case, ASSD manifested with lung involvement, without the possible implication of TNF-α inhibitors in the pathogenesis, as it occurred concurrently with spondyloarthritis. Our case is the first to emphasize the coexistence of ASSD with anti-glycyl tRNA synthetase antibodies and ax-SpA, reminding us of the possible, although rare, adverse effects on the lungs with TNF-α inhibitors.

Objective: Identify the clinical characteristics and prognostic factors in patients with idiopathic infiammatory myopathy (IIM) combined with interstitial lung disease (ILD). Methods: IIM-ILD patients who were hospitalized at Guangxi Medical University from January 2017 to December 2022 were retrospectively analyzed and classified as having dermatomyositis (DM)-ILD or -ILD. Clinical and laboratory results were analyzed. Results: There were 39 males and 111 females, the mean age of disease onset was 50.4 ± 12.3 years, and the median disease duration was 3 months (range: 1–6). Ninety-seven patients had DM-ILD, and 53 had ASS-ILD. The DM-ILD group had 72% positivity for the anti-MDA5 antibody and 5.2% positivity for the anti-Mi-2 antibody; the ASS-ILD group had 67.9% positivity for the anti-Jo-1 antibody and 17% positivity for the anti-EJ antibody. Muscle symptoms, skin ulcers, rash, rapidly progressing interstitial lung disease (RP-ILD), and elevated levels of serum carcinoembryonic antigen were more common in DM-ILD patients (all p < 0.05). However, pericardial effusion and pleural effusion, elevated creatinine kinase, and elevated C-reactive protein were more common in ASS-ILD patients. After a median follow-up of 15.5 months, there were more deaths in the DM-ILD group (42.3% vs. 13.2%, p < 0.001). Multivariate Cox regression analysis showed that RP-ILD, dyspnea, and the usual interstitial pneumonia type of ILD had negative associations with overall survival (OS), and arthralgia had a positive association with OS (all p < 0.05). [ABSTRACT FROM AUTHOR]

Abstract Background Anti‐synthetase syndrome (ASyS) is a rare inflammatory myopathy associated with anti‐aminoacyl transfer RNA synthetase antibodies, and is characterized by a triad of interstitial lung disease, arthritis, and myopathy. ASyS has been considered to represent a subtype of dermatomyositis (DM), however it is not clear whether cutaneous findings overlap in both diseases. Objectives We aimed to characterize mucocutaneous features which should raise suspicion of ASyS and prompt early diagnostic testing. Methods We reviewed 28 cases of ASyS in our institution, and retrieved data from 43 articles reporting clinical features of ASyS in the literature. Results Dermatologic manifestations of ASyS can be categorized as: specific cutaneous signs strongly associated with ASyS (‘Mechanic's hands’, ‘Hiker's feet’); mucocutaneous signs overlapping with other collagenoses; nailfold changes (resembling those of DM and systemic sclerosis); and mucocutaneous signs of undetermined significance. Only 20% had pathognomonic DM signs. Conclusions Dermatologic manifestations of ASyS are heterogeneous and do not always reproduce the pattern seen in DM. Presence of palmar hyperkeratosis (‘Mechanic's hands’), cuticular overgrowth, skin ulcerations and nailfold capillary changes should prompt further diagnostic workup of ASyS. Limitations of our study include inconsistent reporting of dermatologic findings within our series between virtual and in‐person visits. Not all patients were seen in dermatology consultation.

Objective: Identify the clinical characteristics and prognostic factors in patients with idiopathic inflammatory myopathy (IIM) combined with interstitial lung disease (ILD). Methods: IIM‐ILD patients who were hospitalized at Guangxi Medical University from January 2017 to December 2022 were retrospectively analyzed and classified as having dermatomyositis (DM)‐ILD or ‐ILD. Clinical and laboratory results were analyzed. Results: There were 39 males and 111 females, the mean age of disease onset was 50.4 ± 12.3 years, and the median disease duration was 3 months (range: 1–6). Ninety‐seven patients had DM‐ILD, and 53 had ASS‐ILD. The DM‐ILD group had 72% positivity for the anti‐MDA5 antibody and 5.2% positivity for the anti‐Mi‐2 antibody; the ASS‐ILD group had 67.9% positivity for the anti‐Jo‐1 antibody and 17% positivity for the anti‐EJ antibody. Muscle symptoms, skin ulcers, rash, rapidly progressing interstitial lung disease (RP‐ILD), and elevated levels of serum carcinoembryonic antigen were more common in DM‐ILD patients (all p < 0.05). However, pericardial effusion and pleural effusion, elevated creatinine kinase, and elevated C‐reactive protein were more common in ASS‐ILD patients. After a median follow‐up of 15.5 months, there were more deaths in the DM‐ILD group (42.3% vs. 13.2%, p < 0.001). Multivariate Cox regression analysis showed that RP‐ILD, dyspnea, and the usual interstitial pneumonia type of ILD had negative associations with overall survival (OS), and arthralgia had a positive association with OS (all p < 0.05). Conclusion: DM‐ILD patients were more prone to secondary RP‐ILD and skin ulcers, had milder symptoms of myositis and less severe serositis, and had lower survival rates than the ASS‐ILD patients. RP‐ILD, dyspnea, and the usual interstitial pneumonia type of ILD had adverse effects on prognosis, but arthralgia was a protective factor. [ABSTRACT FROM AUTHOR]

Myasthenia gravis (MG) and idiopathic inflammatory myopathy (IIM) are autoimmune diseases of the nervous system, and their main clinical manifestation is muscle weakness. The concurrent presence of both conditions in the same patient is clinically rare and easily missed. Here, we report the case of a 74-year-old woman who went to the doctor with fluctuating weakness of the limbs and muscle pain. By analyzing the patient's history and the results of repeated frequency electrical stimulation, chest computed tomography, thigh muscle magnetic resonance imaging, serum antibody detection, lymph node biopsy, etc., she was finally diagnosed with MGconcomitant IIM with squamous cell carcinoma of the thymus. Acetylcholine receptor antibody, titin antibody, ryanodine receptor antibody, anti-JO-1 antibody, and Ro-52 antibody tests were positive. MG-concomitant IIM is often associated with thymoma. The immunopathology mechanism may be different from that of pure MG or IIM, which needs further research. [ABSTRACT FROM AUTHOR]

Objective This study aimed to establish a screening model for differentiating anti-synthetase syndrome (ASS) from other ANA-associated rheumatic diseases (AARDs) using a combination of cytoplasmic and non-cytoplasmic ANA (ncANA) patterns. Methods : This retrospective observational study included patients with AARDs such as SLE, SSc, SS, MCTD and PM/DM who underwent ANA screening between April 2012 and December 2021. Variables included age, sex, ANA patterns (Cytoplasmic and ncANA) and titres. Logistic regression analysis of Cytoplasmic and ncANA patterns was performed to differentiate ASS from other AARDs. Result : The 981 diagnosed cases of AARDs consisted of SS (n  = 451), SSc (n  = 264), SLE (n  = 201), PM/DM (n  = 104), MCTD (n  = 52) and ASS, including PM/DM (n  = 64). Of these, 155 patients had ≥2 overlapping diseases; however, there was no overlap between AARDs and ASS. ASS is more likely to occur when the cytoplasmic titre is positive and the ncANA <320. Receiver operating characteristic analysis of the Cytoplasmic and ncANA range revealed an area under the receiver operating characteristic curve of 0.885 (95% CI: 0.844–0.927). Conclusion : It is important to detect cytoplasmic patterns as an ANA screening test for ASS diagnosis, even if the titre is low. Additionally, combining the cytoplasmic and ncANA patterns yields more accurate ASS screening results. [ABSTRACT FROM AUTHOR]

Objectives To assess the ability of dual-energy X-ray absorptiometry (DXA) and hand-grip dynamometer to measure damage in inflammatory myopathies (IM). Methods Forty adult IM patients with a disease duration ≥12 months, low or no disease activity for ≥6 months, were prospectively enrolled. Thirty healthy age and sex-matched volunteers were enrolled as controls. Whole-body DXA and hand-grip dynamometer were used to measure muscle mass, grip strength and diagnose sarcopenia (EWGSOP2 criteria). Relationships between the results of strength in 12 muscles, functional tests, patient-reported disability, IMACS damage score, and history of the disease were assessed. The serum levels of potential molecular actors in the damage were measured. Results DXA and grip strength measurements took ≤20 min. Both muscle mass and grip strength were decreased in IM patients vs volunteers (−10% and −30%, respectively) with a dispersion that varied widely (interquartile range −24.3% to +7.8% and −51.3% to −18.9%, respectively). Muscle mass and grip strength were non-redundantly correlated (r up to 0.6, P  = 0.0001) with strength in 14 muscles (manual muscle test and hand-held dynamometer), functions (of limbs, respiratory and deglutition muscles), patient-reported disability, damage (extension and severity in muscular and extra-muscular domains) and blood levels of several myokines. Seven IM patients (17.5%) were sarcopenic. They had the worst damage, impaired functions, disability and history of severe myopathy. Decreased irisin and osteonectin levels were associated with sarcopenia (area under the curve 0.71 and 0.80, respectively). Conclusion DXA and hand-grip dynamometer are useful tools to assess damage in IM. Irisin and osteonectin may play a role in IM damage pathogenesis. [ABSTRACT FROM AUTHOR]

Objectives To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. Methods Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). Results In total 1054 patients were included (DM, n  = 405; ASyS, n  = 649). In the ASyS cohort, 31% (n  = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P  < 0.001), whereas higher frequency of any of four DM-type rashes—heliotrope rash (n  = 248, 61% vs n  = 90, 44%), violaceous rash (n  = 166, 41% vs n  = 57, 9%), V-sign (n  = 124, 31% vs n  = 28, 4%), and shawl sign (n  = 133, 33% vs n  = 18, 3%)—differentiated DM from ASyS-DMskin (all P  < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n  = 67, 17%) compared with ASyS (n  = 21, 3%) and ASyS-DMskin (n  = 7, 3%) cohorts (both P  < 0.001). Conclusion DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management. [ABSTRACT FROM AUTHOR]

There is a well-established relationship between different subsets of idiopathic inflammatory myopathies (IIMs, myositis) and interstitial lung disease (ILD), with lung complications sometimes presenting prior to myopathic manifestations. The subtypes of myositis include those that are strongly associated with ILD, such as polymyositis (PM) and dermatomyositis (DM). Research has shown that in certain patients, these can then be further divided into subtypes using myositis-specific antibodies (MSAs), which are specific for myositis, and myositis-associated antibodies (MAAs), which can be found in myositis in overlap syndromes with other connective tissue diseases (CTDs). Notably, certain MSAs and MAAs are associated with ILD in patients with myositis. The clinical presentations of ILD in patients with myositis can vary widely and can be insidious in onset and difficult to diagnose. As ILD can progress rapidly in some cases, it is essential that clinicians are able to identify and diagnose ILD in patients with myositis. For this reason, the aim of this review is to highlight the clinical features, diagnostic criteria, important histopathologic, laboratory, and radiographic features, and treatment modalities for those patients with myositis-associated ILD. [ABSTRACT FROM AUTHOR]

Objectives Myocarditis is an overlooked manifestation of anti-synthetase syndrome (ASS). Our study describes the clinical and instrumental features of ASS myocarditis and evaluates the performance of cardiac MRI (CMRI) with mapping techniques in assisting diagnosis of ASS myocarditis. Methods Data from patients with ASS were retrospectively analysed. CMRI data for patients diagnosed with myocarditis, including late gadolinium enhancement (LGE), T2 ratio, T1 mapping, extracellular volume (ECV) and T2 mapping, were reviewed. Myocarditis was defined by the presence of symptoms and/or signs suggestive for heart involvement, including increased high-sensitive troponin T (hs-TnT) and/or N-terminal pro-brain natriuretic peptide (NT-proBNP), and at least an instrumental abnormality. The clinical features of patients with ASS with and without myocarditis were compared. A P -value of <0.05 was considered statistically significant. Results Among a cohort of 43 patients with ASS [median age 58 (48.0–66.0) years; females 74.4%; anti-Jo1 53.5%], 13 (30%) were diagnosed with myocarditis. In 54% of those 13 patients, myocarditis was diagnosed at clinical onset. All patients with ASS with myocarditis had at least one CMRI abnormality: increased ECV in all cases, presence of LGE in 91%, and increased T1 and T2 mapping in 91%. The 2009 Lake Louise criteria (LLC) were satisfied by 6 patients, and the 2018 LLC by 10 patients. With the updated LLC, the sensitivity for myocarditis improved from 54.6% to 91.0%. Patients with ASS with myocarditis were more frequently males (53% vs 13%; P  = 0.009) with fever (69% vs 17%; P  = 0.001), and had higher hs-TnT [88.0 (23.55–311.5) vs 9.80 (5.0–23.0) ng/l; P  < 0.001], NT-proBNP [525.5 (243.5–1575.25) vs 59.0 (32.0–165.5; P  = 0.013) pg/ml; P  = 0.013] and CRP [7.0 (1.7–15.75) vs 1.85 (0.5–2.86) mg/l; P  = 0.011] compared with those without myocarditis. Conclusion In ASS, myocarditis is frequent, even at clinical onset. Patients with ASS with myocarditis frequently presented with fever and increased CRP, suggesting the existence of an inflammatory phenotype. The use of novel CMRI mapping techniques may increase diagnostic sensitivity for myocarditis in ASS. [ABSTRACT FROM AUTHOR]

Abstract Objective To assess the real-world, long-term effectiveness of rituximab (RTX) as a rescue therapy in patients with antisynthetase syndrome and progressive interstitial lung disease (ASS-ILD). Methods Multicentre observational retrospective longitudinal study of a cohort of patients with ASS-ILD that started treatment with RTX due to recurrent or ongoing progressive ILD despite therapy with glucocorticoids and immunosuppressants. Results Twenty-eight patients were analyzed. Examining the entire study population, before treatment with RTX the mean decline in %pFVC and %pDLCO from the ASS-ILD diagnosis to the initiation of RTX treatment (T0) was -6.44% and -14.85%, respectively. After six months of treatment, RTX reversed the decline in pulmonary function test (PFT) parameters: ∆%pFVC +6.29% (95% CI: -10.07 to 2.51; p=0.002 compared to T0) and ∆%pDLCO +6.15% (95% CI: -10.86 to -1.43; p=0.013). Twenty-four patients completed one year of therapy and 22 two years, maintaining the response in PFT: ∆%pFVC: +9.93% (95% CI: -15.61 to -4.25; p=0.002) and ∆%pDLCO: +7.66% (95% CI: -11.67 to -3.65; p

Abstract Objective To investigate the association of serum anti-Jo-1 antibody levels with the disease activity and prognosis in anti-Jo-1-positive patients with antisynthetase syndrome (ASS). Methods This study included 115 anti-Jo-1-positive patients with ASS who were admitted to China-Japan Friendship Hospital between 2009 and 2019. Anti-Jo-1 antibody serum levels at initial admission and follow-up were determined by enzyme-linked immunosorbent assay (ELISA). Global and organ disease activity was assessed at baseline and follow-up according to the International Myositis Assessment and Clinical Studies guidelines. Results Among enrolled patients, 70 (60.9%) patients initially presented with interstitial lung disease (ILD), and 46 (40%) patients presented with with muscle weakness at initial admission. At baseline, patients with ILD had lower levels of anti-Jo-1 antibodies than those without ILD (p = 0.012). Baseline anti-Jo-1 antibody levels were higher in patients with muscle weakness, skin involvement, and arthritis (all p

Objective: To evaluate whether anti-PL7 and anti-PL12 autoantibodies are associated with a greater extent of the fibrotic component of ILD in ASSD patients. Methods: Patients with ILD-ASSD who were positive for one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ were included. Clinical manifestations, CPK levels, pulmonary function tests, and HCRT assessments were prospectively collected according to the Goh index. The fibrotic, inflammatory, and overall extension of the Goh index and DLCO were assessed by multiple linear analyses and compared between ASSD antibody subgroups. Results: Sixty-six patients were included; 17 were positive for anti-Jo1 (26%), 17 for anti-PL7 (26%), 20 for anti-PL12 (30%), and 9 (14%) for anti-EJ. Patients with anti-PL7 and anti-PL12 had a more extensive fibrotic component than anti-Jo1. Anti-PL7 patients had a 7.9% increase in the fibrotic extension (cβ = 7.9; 95% CI 1.863, 13.918), and the strength of the association was not modified after controlling for sex, age, and time of disease evolution (aβ = 7.9; 95% CI 0.677, 15.076) and also was associated with an increase in ILD severity after adjusting for the same variables, denoted by a lower DLCO (aβ = − 4.47; 95% CI − 8.919 to − 0.015). Conclusions: Anti-PL7-positive ASSD patients had more extensive fibrosis and severe ILD than the anti-Jo1 subgroup. This information is clinically useful and has significant implications for managing these patients, suggesting the need for early consideration of concurrent immunosuppressive and antifibrotic therapy. [ABSTRACT FROM AUTHOR]

Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations. [ABSTRACT FROM AUTHOR]

Objective: To investigate the association of serum anti-Jo-1 antibody levels with the disease activity and prognosis in anti-Jo-1-positive patients with antisynthetase syndrome (ASS). Methods: This study included 115 anti-Jo-1-positive patients with ASS who were admitted to China-Japan Friendship Hospital between 2009 and 2019. Anti-Jo-1 antibody serum levels at initial admission and follow-up were determined by enzyme-linked immunosorbent assay (ELISA). Global and organ disease activity was assessed at baseline and follow-up according to the International Myositis Assessment and Clinical Studies guidelines. Results: Among enrolled patients, 70 (60.9%) patients initially presented with interstitial lung disease (ILD), and 46 (40%) patients presented with with muscle weakness at initial admission. At baseline, patients with ILD had lower levels of anti-Jo-1 antibodies than those without ILD (p = 0.012). Baseline anti-Jo-1 antibody levels were higher in patients with muscle weakness, skin involvement, and arthritis (all p < 0.05) compared to those without these manifestations. Baseline anti-Jo-1 antibody levels were positively correlated with skin visual analogue scale (VAS) scores (r = 0.25, p = 0.006), but not with disease activity in other organs. However, changes in anti-Jo-1 antibody levels were significantly positively correlated with the changes in PGA (β = 0.002, p = 0.001), muscle (β = 0.003, p < 0.0001), and pulmonary (β = 0.002, p = 0.013) VAS scores, but not with skin and joint VAS scores. Older age of onset (hazard ratio [HR] 1.069, 95% confidence interval [CI]:1.010–1.133, p = 0.022) and higher C-reactive protein (CRP) levels (HR 1.333, 95% CI: 1.035–1.717, p = 0.026) were risk factors for death. Conclusion: Anti-Jo-1 titers appear to correlate more with disease activity changes over time rather than with organ involvement at baseline, which provides better clinical guidance for assessing the disease course using anti-Jo-1 levels. [ABSTRACT FROM AUTHOR]

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Objectives To characterize clinically distinct subgroups among unselected patients with anti-synthetase antibodies using cluster analysis. Methods This study evaluated patients with anti-synthetase antibodies registered to two independent cohorts; 106 consecutive patients from a prospective, single-centre cohort of the Scleroderma/Myositis Centre of Excellence (SMCE) were used as a derivation cohort and 125 patients from the Multicentre Retrospective Cohort of Japanese Patients with Myositis-Associated Interstitial Lung Disease (JAMI) were used as a validation cohort. Anti-synthetase antibodies were identified by RNA immunoprecipitation. A multiple correspondence analysis followed by hierarchical clustering was performed to aggregate the patients into homogeneous subgroups. Subsequently, a simple-to-use classification tree was generated using classification and regression tree analysis. Results Three clusters were identified in the SMCE cohort: cluster 1 (n  = 48), the interstitial pneumonia with autoimmune features/amyopathic dermatomyositis cluster, associated with older age at diagnosis and a higher frequency of malignancy; cluster 2 (n  = 46), the DM cluster, corresponded to a younger age at diagnosis with a higher prevalence of myositis, arthritis, DM pathognomonic rashes, mechanic's hands and fever; and cluster 3 (n  = 12), the SSc cluster, characterized by chronic interstitial lung disease. There was no significant difference in overall survival or progression-free survival between the clusters. A simple classification tree using myositis and RP was created in the SMCE cohort. Clusters 1 and 2 were successfully reproduced and the classification tree demonstrated favourable performance in the JAMI cohort. Conclusion Patients with anti-synthetase antibodies were classified into three distinct phenotypes, indicating substantial heterogeneity within this patient group. [ABSTRACT FROM AUTHOR]

Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions. [ABSTRACT FROM AUTHOR]

Background: Antisynthetase syndrome (ASS) is a rare autoimmune disease characterized by the immune system attacking specific synthetase in the body. Due to the difficulty in clinical diagnosis, there is still a lack of effective treatment. Methods: We report a case of a 50-year-old man who presented with progressive, symmetric limb weakness, starting from the lower limbs and gradually affecting the upper limbs. He was admitted to the intensive care unit (ICU) for treatment due to recurrent fever and coma. When he was admitted to the ICU, his limbs were almost unable to move, and the levels of creatine phosphokinase and muscle glycogen were significantly elevated (2449 u/l and 1857 ng/ml). The electromyogram showed myogenic injury, and the anti-PL7 antibody, anti-SSA antibody, and anti-Ro52 antibody were positive. Pathological biopsy of the left biceps brachii showed striated muscle necrosis and macrophage infiltration. He was finally diagnosed with ASS and received treatment with methylprednisolone (subsequently changed to prednisone) and traditional Chinese medicine (Buzhongyiqi Decoction and Shenlingbaizhu powder). Results: After receiving 2 weeks of glucocorticoid and traditional Chinese medicine treatment, his muscle strength had basically recovered, reaching grade 5 in his limb muscles strength. During the 3-month follow-up period, his activity tolerance continued to improve. Conclusion: We present a case of severe anti-PL7 positive ASS with positive anti-SSA/Ro52 antibody. The disease was relieved by glucocorticoid and traditional Chinese medicine treatment. This provides an effective approach for managing ASS.

Introduction/Justification: Antisynthetase syndrome is an autoimmune pathology characterized by production of autoantibodies against aminoacyl tRNA synthetase, mainly anti-Jo-1. Report: Male patient presenting a clinical picture of polyarthritis in hands and wrists, periorbital edema, myositis and interstitial lung disease. FAN 1/320 (nuclear homogeneous), reactive anti Jo-1, elevated CPK, erythrocyte sedimentation rate and CRP. MRI presenting muscle edema, electroneuromyography compatible with myopathy and chest CT scan suggesting inflammatory/infectious pattern. Patient was diagnosed with antisynthetase syndrome and prednisone introduced. Later on course cyclophosphamide was added due to lung involvement. He evolved with respiratory and joint symptom improvement but with progressive worsening of muscle symptoms, characterized by proximal weakness (difficulty getting out of bed and car, daily life activities like brushing his teeths and eating). He also presented CPK rise, persistent subfebrile temperature and signs of inflammatory activity (leukocytosis and CRP rise), without any apparent focus and isolated episodes of dysphagia. Rituximabe was introduced and PET/CT scan was performed to search for the focus of the infection. PET/CT showed a diffuse pattern of muscle hypermetabolism, specially in right lower limb, suggesting a diffuse muscle inflammation without any infectious focus. Conclusion: Antisynthetase syndrome is a rare entity with few PET/CT reports in the literature. However PET scan appears to be very useful in the investigation of fever of unknown origin, diagnosing inflammatory activity and in response assessment evaluation.

Background: Antisynthetase syndrome (ASS) is a special presentation of autoimmune inflammatory myopathy (AID) characterized by specific anti-RNA polymerase antibodies. Its therapeutic care is similar to that of other MAI. It is a pathology often responsive to corticosteroid and conventional immunosuppressive treatments. In refractory cases, authors suggest the use of Rituximab (RTX). It is an anti-CD20 monoclonal antibody of the lymphocyte membrane. Methods: This is a retrospective description of an observation followed for an antisynthetase syndrome in our department for 46 months having received RTX. We subsequently carried out a bibliographic search of the different cases reported in the literature of SAS treated with RTX. Case report :A 44-year-old patient admitted for myalgia, dyspnea and asthenia with muscle deficit and myolysis. She was diagnosed with ASS based on muscular involvement, pulmonary involvement such as diffuse interstitial diffuse pneumonia and specific anti-JO-1 antibodies. She was treated with corticosteroid and several immunosuppressive drugs withoutimprovement. She had resistant and disabling muscle damage. Therefore, she was treated with RTX with a transient improvement in muscle damage for few months. Conclusion: RTX, despite a risk of therapeutic failure, remains a therapeutic alternative for refractory muscle damage in ASS. Further studies are needed to better assess its effectiveness. [ABSTRACT FROM AUTHOR]

Introduction: In a recent study, we have shown that atorvastatin is clinically safe for dermatomyositis (DM) and antisynthetase syndrome (ASS) patients with dyslipidemia. Herein, we showed in an unprecedented way, the safety of atorvastatin on the muscular tissues of these patients. Methods: Transcriptome analysis was performed on samples of the vastus lateralis muscle obtained at baseline and after 12 weeks of atorvastatin (20 mg/day) intervention in DM or ASS patients with dyslipidemia [6DM and 5ASS received atorvastatin, and 2DM and 3ASS received placebo]. The results were analyzed considering differences in expression fold change before and after treatment. Histological and histochemical analyses were also performed. Results: In both groups, no significant changes were observed in genes related to the mitochondrial, oxidative, insulin, lipid, and fibrogenic pathways. Histological analysis showed a slight variability in the fiber size that was preserved after the intervention. In addition, the mosaic of muscle fibers was preserved in the internal architecture of the fibers and all histological regions. No fiber necrosis or atrophy, focal failures, subsarcolemmal accumulation, lipids, areas of fibrosis, or alterations in mitochondrial activity were observed. All muscle fibers were labeled for MHC I. Conclusion: Atorvastatin did not promote significant changes in the expression of genes related to mitochondrial, oxidative, insulin, lipid, and fibrogenic pathways in the muscle tissues of DM and ASS patients with dyslipidemia. Atorvastatin did not also promote histological and histochemical changes in muscle tissues. Our results reinforce the safety of the administration of atorvastatin to treat dyslipidemia in patients with DM and ASS. [ABSTRACT FROM AUTHOR]

Abstract Background Anti-Jo-1 autoantibodies represent essential markers in the diagnosis of antisynthetase syndrome (ASS). In this retrospective study, we aimed to investigate whether their concentrations and fluctuations could both respectively reflect the severity and evolution of ASS. Methods Between 2015 and 2020, clinical and biological features of ASS patients with at least one positive measure of anti-Jo-1 autoantibody were collected. At each serum sampling, we assessed myositis activity by using the Myositis Intention to Treat Activities Index (MITAX) and compared anti-Jo-1 concentrations with ASS severity, anti-Jo-1 concentrations between patients with and without active disease, and changes in anti-Jo-1 concentrations with disease activity. Results Forty-eight patients with ASS had at least one positive determination of anti-Jo-1 concentration. Among them, twenty-nine patients had at least two determinations of anti-Jo-1 autoantibody in their follow-up. We showed that these autoantibody concentrations were significantly correlated with MITAX (r = 0.4, p = 0.03) and creatine kinase concentration (r = 0.34, p = 0.002) and that they were significantly higher in patients with active disease than in those with inactive disease (91.7 IU/L vs 44.4 IU/L, p = 0.016). During follow-up, we found a significant correlation between fluctuations of anti-Jo-1 autoantibody concentrations and MITAX score (r = 0.7, p

Abstract Background Antisynthetase syndrome is an inflammatory myopathy that is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies. Only 30% of those who suffer from the disease can be identified. We present three Hispanic cases of antisynthetase syndrome with unusual clinical pictures were extended myositis panel results enable disease diagnosis and treatment. Case presentation A 57-year-old Hispanic/Latino female with an erythematous scaly plaque, unresolved fever and non-immune haemolytic anaemia in whom inpatient work-up for fever of unknown origin was positive for anti-PL12 positive myositis extended panel. A 72-year-old Hispanic/Latino male with amyopathic weakness syndrome and mechanic hands in whom impatient work-up was relevant for proximal muscle uptake and anti-PM75 and AntiPL-12 myositis extended panel. And a 67-year-old Hispanic/Latino male with progressive interstitial lung disease and unresolved fever ended in myositis extended panel positive for antiPL-7. After systemic immunosuppressor treatment, patients had favourable clinical and paraclinical responses during outpatient follow-up. Conclusions The high variability of the antisynthetase syndrome in these cases demonstrates the importance of identification through an expanded panel and highlights the probability that this is a variable disease and that we need to include emerging molecular tests to promote the timely treatment of patients.

Background: This report presents the influence of immunosuppression by new rheumatological therapies on hepatitis E virus infection in a 54-year-old male patient with an anti-synthetase syndrome and treatment with methotrexate and rituximab. Case description: The patient arrived at the Emergency Department with epigastric pain, vomiting and dark urine. Initial examination revealed signs of inflammation and hepatic dysfunction. Subsequent laboratory tests and imaging confirmed acute hepatitis E infection in the context of recent initiation of rituximab therapy. Despite initial suspicion of pancreatitis, subsequent investigations ruled out pancreatic involvement. Treatment with ribavirin, along with supportive measures, led to significant clinical improvement with resolution of jaundice, ascites, and oedema. Conclusions: This case underscores the importance of considering hepatitis E in patients with autoimmune conditions, especially when initiating immunosuppressive therapies, a situation that is not well described in scientific literature and is increasingly common, necessitating proper recognition.

Les myopathies inflammatoires (MI) sont des pathologies. auto-immunes rares. L'incidence de ces pathologies a été mieux précisée (8,22/million/an). Ces pathologies ont en commun une inflammation du muscle squelettique mais sont hétérogènes en termes de présentation clinique, d'auto-anticorps, de physiopathologie et de pronostic. La scléromyosite est une entité émergeant dans la classification des MI qui se distingue sur la base de caractéristiques cliniques sérologiques et histopathologiques. Sa reconnaissance est cruciale pour la surveillance et l'adaptation de la corticothérapie. Des avancées importantes ont été faites dans la caractérisation sérologique des MI. En particulier, des premières recommandations pour l'utilisation des anticorps « classiques » ont été publiées, des nouveaux anticorps antisynthétases ont été identifiés et des nouveaux auto-anticorps aident à préciser le risque de cancer associé aux dermatomyosites. L'étude de la biopsie du muscle des patients a permis de mettre en lumière de nouveaux acteurs physiopathologiques, en particulier, le lymphocyte B (au cours du syndrome des antisynthétases), les auto-anticorps, l'IFN-1 et la mitochondrie (au cours de la dermatomyosite). Au plan thérapeutique, une spécialité d'immunoglobulines intraveineuses a obtenu une AMM pour les dermatomyosites (une première dans les MI), les données de l'essai Recital placent le rituximab avant le cyclophosphamide dans le traitement des pneumopathies associées aux MI et les premières données de tolérance et d'efficacité des CD19 CAR-T cells dans les syndromes des antisynthétases réfractaires sont très encourageantes. Inflammatory myopathies (IM) are rare autoimmune pathologies whose incidence has been refined (8.22/million/year). These pathologies share an inflammation of the skeletal muscle but they are heterogeneous in terms of clinical presentation, autoantibodies, pathophysiology and prognosis. Scleromyositis is an emerging entity within MI spectrum, that can be delineated based on clinical, serological and histopathological characteristics. Its recognition is crucial for patient monitoring and adapting corticosteroid therapy. Significant advances have been made in the serological characterization of MI. In particular, the first recommendations for the use of "classical" autoantibodies have been published, new antisynthetases have been identified and new autoantibodies help to clarify the risk of cancer associated with dermatomyositis. In-depth studies of the muscle of IM patients highlighted new physiopathological actors, in particular, the B lymphocyte (in antisynthetase syndrome), autoantibodies, IFN-1 and the mitochondria (in dermatomyositis). A speciality of intravenous immunoglobulins has obtained marketing authorization for dermatomyositis (first drug to be approved for IM), data from the Recital trial placed rituximab before cyclophosphamide in the treatment of interstitial lung disease associated with MIs and the first data regarding tolerance and efficacy of CD19 CAR-T cells in refractory antisynthetase syndromes are very encouraging. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The association of neuromyelitis optica concurrently with two other autoimmune diseases is rare. Neuromyelitis optica should be taken into consideration when evaluating the symptoms of the patient as a differential diagnostic aspect. [ABSTRACT FROM AUTHOR]

Background: Antisynthetase syndrome (ASSD) is a multisystem autoimmune disease characterized by the presence of antibodies against aminoacyl-transfer RNA synthetases, most common being anti-Jo1 antibody. It is an under-recognized entity with a significant delay in diagnosis, both due to lack of awareness of this condition and presentation as “incomplete” form more often than the complete form, characterized by the presence of triad of interstitial lung disease (ILD), myositis, and arthritis. We aimed to study the clinical spectrum and outcomes of anti-Jo1 antibody-positive ASSD at a single tertiary care referral hospital in North India. Methods: Anti-Jo1 positive ASSD patients diagnosed according to the Connors et al. criteria were included in this observational study conducted over 3 years by the departments of Rheumatology and Pulmonary Medicine at a single tertiary care hospital in North India. Results: The clinical spectrum and treatment outcomes of 17 patients diagnosed with anti-Jo1 positive ASSD were studied. Only 2 of the 17 patients presented with the “complete” form at the onset. While 12 patients improved with treatment with steroids and immunosuppressives (mycophenolate, azathioprine, or methotrexate), five patients died, four due to sepsis and one due to progressive lung disease. Conclusion: ASSD is an under-recognized disease, often presenting as an incomplete form rather than the classic triad of arthritis, ILD, and myositis. Infections are the major cause of death contributing to high mortality in Indian patients with ASSD.

AimTo characterize the lung microbiome in the bronchoalveolar lavage fluid (BALF) of patients with Antisynthetase Syndrome (ASSD) according to anti-Jo1 autoantibody positivity and evaluate the correlation with differential cell count and other bacterial genera in BALF.MethodsWe sequenced the 16S ribosomal RNA gene in the BALF of anti-Jo1-positive (JoP, n=6) and non-Jo1-positive (NJo, n=17) patients, and the differential cell count in BALF was evaluated. The Spearman’s correlation was calculated for the quantitative variables and abundance of bacterial species.ResultsThe Veillonella genus showed a significant decrease (p

• Anti-PL7 or PL12 antisynthetase syndromes are very rare but very severe diseases • In our cohort, 20% of patients died during follow-up, 67% had lung fibrosis at last visit and 20% received oxygen • Repeated screening of lung involvement is required to discuss antifibrotic therapies • Neoplasms should be screened at diagnosis or during the year after diagnosis • Pericardial effusion and pulmonary hypertension should be closely monitored Antisynthetase syndrome (ASyS) is a rare autoimmune disease. We aimed to determine clinical, biological, radiological, and evolutive profiles of ASyS patients with anti-PL7 or anti-PL12 autoantibodies. We performed a retrospective study that included adults with overt positivity for anti-PL7/anti-PL12 autoantibodies and at least one Connors' criterion. Among 72 patients, 69% were women, 29 had anti-PL7 and 43 anti-PL12 autoantibodies, median age was 60.3 years, and median follow-up period was 52.2 months. At diagnosis, 76% of patients had interstitial lung disease, 61% had arthritis, 39% myositis, 25% Raynaud's phenomenon, 18% mechanic's hands, and 17% had fever. The most frequent pattern on initial chest computed tomography was non-specific interstitial pneumonia and 67% had fibrosis at last follow-up. During follow-up, 12 patients had pericardial effusion (18%), 19 had pulmonary hypertension (29%), 9 (12.5%) had neoplasms, and 14 (19%) died. Sixty-seven patients (93%) received at least one steroid or immunosuppressive drug. Patients with anti-PL12 autoantibodies were younger (p =0.01) and more frequently exhibited anti-SSA autoantibodies (p =0.01); patients with anti-PL7 autoantibodies had more severe weakness and higher maximum creatine kinase levels (p =0.03 and 0.04, respectively). Initial severe dyspnoea was more common in patients from the West Indies (p =0.009), with lower predicted values of forced vital capacity, forced expiratory volume in 1s, and total lung capacity (p =0.01, p =0.02, p =0.01, respectively) contributing to a more severe 'respiratory' initial presentation. The high mortality and significant numbers of cardiovascular events, neoplasms and lung fibrosis in anti-PL7/12 patients justify close monitoring and question addition of antifibrotic drugs. [ABSTRACT FROM AUTHOR]

Antisynthetase syndrome is a rare idiopathic inflammatory multisystem disorder, which can lead to serious postoperative complications. Due to its low incidence, there is little literature on its anesthetic management. However, patients with this disease can suffer from serious complications secondary to muscle weakness and respiratory complications. Although the intraoperative and the immediate postoperative periods may be uneventful, complications may appear later. The characteristics of the disease can lead to a misdiagnosis in the case of respiratory acute failure. The objective of this clinical report is to discuss the perioperative management of patients suffering from antisynthetase syndrome, assess the usefulness of postoperative monitoring, and evaluate alternatives that could have been carried out to prevent the fatal outcome reported in this narrative. [ABSTRACT FROM AUTHOR]

Objective. To determine the clinical phenotype and outcomes of patients with idiopathic inflammatory myopathies (IIMs) and myocarditis. Methods. Using the Johns Hopkins Myositis Center Research Registry, we identified 31 adult patients with IIM--out of a total of 3082 with confirmed or suspected muscle disease--with an encounter code of myocarditis from 2004 to 2021. Of these, 14 adult patients with IIM were adjudicated to have clinical myocarditis. Information about demographics, autoantibodies, and clinical outcomes was retrospectively collected and analyzed. Results. Of 14 patients with IIM with clinical myocarditis, the median age at IIM diagnosis was 49 (IQR 35-56) years, and the median age at myocarditis diagnosis was 54 (IQR 36-61) years. The median duration between IIM diagnosis and myocarditis was 3 (IQR 2-9) years. The majority of patients were female (8/14, 57%) and Black (10/14, 71%). Antisynthetase syndrome was the most common IIM subtype (9/14, 64%). Anti-Jo1 (n = 4) and anti-PL12 (n = 3) were the most frequent autoantibodies. At myocarditis diagnosis, most patients (11/14, 79%) had active myositis, defined as elevated creatine kinase and/or muscle weakness; required hospitalization (13/14, 93%); and had reduced left ventricular ejection fraction (LVEF < 50%; 10/14, 71%). Despite intensification of immunosuppression, the 5-year overall survival rate from IIM diagnosis was 84%, and the 5-year overall survival rate from myocarditis diagnosis was 53%. Systolic dysfunction (LVEF < 40%) at final evaluation was observed in all expired patients (n = 6). Conclusion. Clinical presentations of myocarditis in this select cohort of patients with IIM were severe and heterogeneous with poor outcomes despite intensification of immunosuppression, potentially reflecting late detection of myocarditis. [ABSTRACT FROM AUTHOR]

Background: Antisynthetase syndrome is an inflammatory myopathy that is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies. Only 30% of those who suffer from the disease can be identified. We present three Hispanic cases of antisynthetase syndrome with unusual clinical pictures were extended myositis panel results enable disease diagnosis and treatment. Case presentation: A 57-year-old Hispanic/Latino female with an erythematous scaly plaque, unresolved fever and non-immune haemolytic anaemia in whom inpatient work-up for fever of unknown origin was positive for anti-PL12 positive myositis extended panel. A 72-year-old Hispanic/Latino male with amyopathic weakness syndrome and mechanic hands in whom impatient work-up was relevant for proximal muscle uptake and anti-PM75 and AntiPL-12 myositis extended panel. And a 67-year-old Hispanic/Latino male with progressive interstitial lung disease and unresolved fever ended in myositis extended panel positive for antiPL-7. After systemic immunosuppressor treatment, patients had favourable clinical and paraclinical responses during outpatient follow-up. Conclusions: The high variability of the antisynthetase syndrome in these cases demonstrates the importance of identification through an expanded panel and highlights the probability that this is a variable disease and that we need to include emerging molecular tests to promote the timely treatment of patients. [ABSTRACT FROM AUTHOR]