Search

Objectives: Rapidly progressive interstitial lung disease (RP-ILD) is a life-threatening form of idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD). We aimed to assess the combination of 18 F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET/CT) and high-resolution computed tomography (HRCT) for the quantification of IIM-ILD activity and risk stratification for RP-ILD., Method: Patients with IIM and undergoing 18 F-FDG PET/CT were included in this retrospective study. Pulmonary FDG uptake was assessed using the maximum standardized uptake value (SUV lung ) and visual score (PET score). HRCT was evaluated using visual analysis (HRCT score). Multivariable logistic regression was used to identify risk factors for RP-ILD., Results: Seventy-three patients with IIM (17 with RP-ILD, 38 with non-RP-ILD, and 18 without ILD) were included. SUV lung , PET score, and HRCT score were significantly higher in RP-ILD than in non-RP-ILD. Strong positive correlations were observed between SUV lung , PET score, and the HRCT parameters. The area under the curve (AUC) of the PET score to differentiate between RP-ILD and non-RP-ILD (AUC = 0.860) was higher than that of the SUV lung (AUC = 0.802) and HRCT scores (AUC = 0.806). We developed a risk score based on the number of positive risk factors (PET score > 18, HRCT score > 140, and positive anti-melanoma differentiation-associated gene 5 (MDA5) antibody) to differentiate between RP-ILD and non-RP-ILD (AUC = 0.955). Patients with higher risk scores had significantly worse prognoses., Conclusions: 18 F-FDG PET/CT is useful for assessing disease activity in patients with IIM-ILD. The combination of PET score, HRCT score, and anti-MDA5 antibody can be used to identify patients at increased risk of RP-ILD and with poor prognoses., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Pancreatic cancer is only rarely associated with myopathy. We present a case of a 69-year-old male with recently diagnosed pancreatic cancer, who presented himself with a paraneoplastic myositis of both legs. MRI and EMG contributed to this diagnosis. Treatment was started with high dose corticosteroids followed by urgent laparoscopic pancreaticoduodenectomy. Postoperatively there was a rapid normalisation of the creatinine kinase levels with gradual increase of the muscle strength. The anatomopathology of the biopsy specimen showed a large cell neuroendocrine carcinoma. Paraneoplastic myositis associated with pancreatic cancer may be treated successfully with cancer specific treatment., (© Acta Gastro-Enterologica Belgica.)

Arthritogenic alphaviruses are mosquito-borne arboviruses that include several re-emerging human pathogens, including the chikungunya (CHIKV), Ross River (RRV), Mayaro (MAYV), and o'nyong-nyong (ONNV) virus. Arboviruses are transmitted via a mosquito bite to the skin. Herein, we describe intradermal RRV infection in a mouse model that replicates the arthritis and myositis seen in humans with Ross River virus disease (RRVD). We show that skin infection with RRV results in the recruitment of inflammatory monocytes and neutrophils, which together with dendritic cells migrate to draining lymph nodes (LN) of the skin. Neutrophils and monocytes are productively infected and traffic virus from the skin to LN. We show that viral envelope N-linked glycosylation is a key determinant of skin immune responses and disease severity. RRV grown in mammalian cells elicited robust early antiviral responses in the skin, while RRV grown in mosquito cells stimulated poorer early antiviral responses. We used glycan mass spectrometry to characterize the glycan profile of mosquito and mammalian cell-derived RRV, showing deglycosylation of the RRV E2 glycoprotein is associated with curtailed skin immune responses and reduced disease following intradermal infection. Altogether, our findings demonstrate skin infection with an arthritogenic alphavirus leads to musculoskeletal disease and envelope glycoprotein glycosylation shapes disease outcome. IMPORTANCE Arthritogenic alphaviruses are transmitted via mosquito bites through the skin, potentially causing debilitating diseases. Our understanding of how viral infection starts in the skin and how virus systemically disseminates to cause disease remains limited. Intradermal arbovirus infection described herein results in musculoskeletal pathology, which is dependent on viral envelope N-linked glycosylation. As such, intradermal infection route provides new insights into how arboviruses cause disease and could be extended to future investigations of skin immune responses following infection with other re-emerging arboviruses.

Currently, no standard treatment strategy has been established for immune-mediated necrotizing myopathy (IMNM). Here we present a case of IMNM which was successfully treated with intensive combined therapy with high-dose glucocorticoids, tacrolimus, and intravenous immunoglobulins. Her muscle weakness was rapidly progressive and severe so that she became bedridden one week after admission. She was complicated with dysphagia and had serum myogenic enzymes elevation, ventricular diastolic dysfunction, and interstitial lung disease. Serum anti-SRP antibody was positive and her muscle biopsy revealed many necrotic fibers with minimal inflammation. Further histological analysis demonstrated infiltration of phagocytic macrophages with deposition of membrane attack complex (C5b-9) in the necrotic muscle fibers, suggesting activation of complement pathway and macrophages as a pathomechanism of this disease. She was diagnosed as IMNM and was immediately initiated a combination therapy described above, which led to dramatic clinical improvements. Recent studies suggest that intravenous immunoglobulins and tacrolimus can inhibit the activation of complement pathway and macrophages. Our present case suggests that early initiation of intensive combined therapy including intravenous immunoglobulins and tacrolimus might be effective for preventing irreversible muscle damages by disrupting a pathogenic activation of complement and macrophages in IMNM.

Objective: To investigate the prevalence, describe the radiological features, and consider the clinical sequelae of COVID-19- associated shoulder girdle calcific myositis., Methods: All patients who underwent a CT pulmonary angiogram study at our institution (Queen Alexandra Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, United Kingdom) in April and May 2020, January 2021, and July 2021 were included. A total of 1239 CT pulmonary angiogram studies for 1201 patients were reviewed. Patients with COVID-19 and associated shoulder girdle calcific myositis were identified. Their electronic patient records were reviewed. The patients' demographics, serum inflammatory markers, and proning history were recorded., Results: Of the 364 patients in Wave 1, 71 patients (19.5%) had COVID-19, and of those, 2 patients (2.8%) had shoulder girdle calcific myositis. Of the 521 patients in Wave 2, 354 patients (67.9%) had COVID-19, and of those, 3 patients (0.8%) had shoulder girdle calcific myositis. Of the 316 patients in Wave 3, 37 patients (11.7%) had COVID-19, and of those, 1 patient (2.7%) had shoulder girdle calcific myositis. The overall prevalence was 1.3%. The most common site of calcific myositis was within the subscapularis muscle., Conclusion: COVID-19-associated shoulder girdle calcific myositis is a rare extrapulmonary musculoskeletal manifestation of COVID-19. Early recognition and increased awareness of this disease entity, in our experience, aids in reducing patient morbidity and improving long-term functional outcome., Advances in Knowledge: We have reported a novel disease entity associated with COVID-19, in the form of shoulder girdle calcific myositis. We have described the common imaging features and discussed our experience of management and clinical sequelae.

Objective: To investigate the prognostic significance of videofluorographic swallowing study (VFSS)-confirmed oropharyngeal dysphagia in idiopathic inflammatory myopathies (IIMs)., Method: We reviewed the medical records of patients who were diagnosed with IIM between 2009 and 2020 at Seoul St Mary's Hospital. All oropharyngeal dysphagia cases were limited to VFSS-confirmed dysphagia found during the initial diagnostic work-up for IIM. We described the findings on VFSS and the course of the dysphagic symptoms. Logistic regression and survival analyses were performed to evaluate the risk of pneumonia and mortality, respectively., Results: We found 88 patients with IIM who met the criteria. Among them, 17 patients (19%) had oropharyngeal dysphagia. Except for two cases lost to follow-up and one deceased case, all of the patients with dysphagia (14 of 14) had swallowing function restored within 6 months. The risk of pneumonia within 3 months from the diagnosis of IIM was significant [odds ratio = 4.49, 95% confidence interval (CI) 1.07-18.88]. The median follow-up duration was 34 and 27 months for the groups without and with dysphagia, respectively. The survival analysis failed to demonstrate that the presence of oropharyngeal dysphagia increased the risk of death (hazard ratio = 0.77, 95% CI: 0.085-7.00)., Conclusions: Oropharyngeal dysphagia found at the initial diagnosis of IIM improved within 3-6 months in nearly all cases. Furthermore, IIM patients who had oropharyngeal dysphagia at the initial diagnosis of IIM were not likely to have shorter survival, even if the risk of pneumonia was increased in the short term.

The term "connective tissue diseases" (CTDs) refers to a heterogeneous group of autoimmune disorders, including systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis, antisynthetase syndrome, and mixed connective tissue disease. Chest high-resolution computed tomography (HRCT) is the imaging method of choice for evaluating patients with known or suspected CTD-related interstitial lung disease (CTD-ILD), a complication accounting for substantial morbidity and mortality. While specific HRCT patterns and signs of CTD-ILD have been extensively described (hence the designation "the usual suspects"), the knowledge of various, less frequent conditions involving the lungs in patients with CTD would help the radiologist produce a clinically valuable report, thus potentially influencing patient management. This paper aims to provide an up-to-date review of various unusual pulmonary CTD-related conditions the radiologist should be aware of; namely, acute exacerbation of CTD-ILD, CTD-related interstitial lung abnormalities, lung amyloidosis, MALT lymphoma, antisynthetase syndrome, pleuroparenchymal fibroelastosis-like lesion, drug-induced ILD, combined pulmonary fibrosis and emphysema, and pulmonary hypertension. For each condition, the chest HRCT appearance and the key histopathological and clinical features are resumed, helping the radiologist participate actively in the multidisciplinary discussion of complex clinical cases., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Coronavirus disease 2019 (COVID-19) typically manifests with respiratory symptoms and can ultimately progress to severe multiorgan failure. Viral myositis, systemic capillary leak syndrome, and arteriovenous thrombosis are atypical manifestations of COVID-19. We present a case of a 33-year-old woman, fully vaccinated against COVID-19, who developed myositis and shock. She ultimately required bilateral lower extremity fasciotomies secondary to compartment syndrome, presumably from COVID-19 myositis. Although compartment syndrome from COVID-19 myositis has been reported for ocular, hand, and thigh compartment syndromes, this is the first case report showing bilateral lower extremity compartment syndrome secondary to COVID-19 myositis in a fully vaccinated individual. As we learn more about COVID-19 and its extrapulmonary effects, it is imperative to consider all working diagnoses when working up patients. Providers must be aware of extrapulmonary effects of COVID-19, particularly in individuals who might deviate from traditional symptoms.

Interstitial lung disease can be the first sign of systemic autoimmune disease. If associated with myositis, interstitial lung disease may be the only symptom, with no presence of muscular weakness or other extramuscular manifestations. ANA-testing performed with indirect immune fluorescence may be negative. Testing for myositis antibodies should be considered as a step in the diagnostic process when strong clinical suspicion of interstitial lung disease of unknown origin is present. If interstitial lung disease is suspected, the patients should be referred to a specialist clinic for further investigation and, if possible, for discussion within a multidisciplinary team. Early suspicion of systemic inflammatory disease, rapid diagnosis and early start of treatment are crucial for future prognosis, quality of life and survival.

Anti-synthetase syndrome (ASyS)-associated myositis is a major subgroup of the idiopathic inflammatory myopathies (IIM) and is characterized by disease chronicity with musculoskeletal, dermatological and pulmonary manifestations. One of eight autoantibodies against the aminoacyl-transferase RNA synthetases (ARS) is detectable in the serum of affected patients. However, disease-specific therapeutic approaches have not yet been established.To obtain a deeper understanding of the underlying pathogenesis and to identify putative therapeutic targets, we comparatively investigated the most common forms of ASyS associated with anti-PL-7, anti-PL-12 and anti-Jo-1. Our cohort consisted of 80 ASyS patients as well as healthy controls (n = 40), diseased controls (n = 40) and non-diseased controls (n = 20). We detected a reduced extent of necrosis and regeneration in muscle biopsies from PL-12 + patients compared to Jo-1 + patients, while PL-7 + patients had higher capillary dropout in biopsies of skeletal muscle. Aside from these subtle alterations, no significant differences between ASyS subgroups were observed. Interestingly, a tissue-specific subpopulation of CD138 + plasma cells and CXCL12 + /CXCL13 + CD20 + B cells common to ASyS myositis were identified. These cells were localized in the endomysium associated with alkaline phosphatase + activated mesenchymal fibroblasts and CD68 + MHC-II + CD169 + macrophages. An MHC-I + and MHC-II + MxA negative type II interferon-driven milieu of myofiber activation, topographically restricted to the perifascicular area and the adjacent perimysium, as well as perimysial clusters of T follicular helper cells defined an extra-medullary immunological niche for plasma cells and activated B cells. Consistent with this, proteomic analyses of muscle tissues from ASyS patients demonstrated alterations in antigen processing and presentation. In-depth immunological analyses of peripheral blood supported a B-cell/plasma-cell-driven pathology with a shift towards immature B cells, an increase of B-cell-related cytokines and chemokines, and activation of the complement system. We hypothesize that a B-cell-driven pathology with the presence and persistence of a specific subtype of plasma cells in the skeletal muscle is crucially involved in the self-perpetuating chronicity of ASyS myositis. This work provides the conceptual framework for the application of plasma-cell-targeting therapies in ASyS myositis., (© 2022. The Author(s).)

Chronic graft-versus-host disease (cGVHD) is the most important complication resulting in the death of bone marrow transplantation (BMT) survivors. It is also a relatively rare cause of inflammatory myopathy (IM). We report the case of a 46-year-old woman who developed severe cGVHD-related IM after BMT for myelodysplastic syndrome. She presented with severe muscle pain and weakness with cGVHD-related symptoms in other organs. Myopathological analysis showed moderate cell infiltration with remarkable necrotic and regenerative fibers. Sarcoplasm and capillaries expressed C5b9 and myxovirus resistance protein 1. Non-necrotic fibers in perifascicular regions expressed MHC-II. Steroid therapy did not sufficiently control cGVHD-related IM, and the patient was concurrently treated with an immunosuppressant. Our findings show that IM is a key manifestation of cGVHD and that the expression of interferon-inducible proteins in muscle pathology is useful for identifying cGVHD-related IM., (© 2022 Japanese Society of Neuropathology.)

Background/objective: Patients classified as interstitial pneumonia with autoimmune features (IPAF) have interstitial lung disease (ILD) and features of autoimmunity but do not fulfill criteria for connective tissue diseases (CTDs). Our goal was to identify patients classifiable as IPAF, CTD-ILD, and idiopathic pulmonary fibrosis (IPF) from a preexisting pulmonary cohort and evaluate the prognosis of patients with IPAF., Methods: We reviewed the medical records of 456 patients from a single-center pulmonary ILD cohort whose diagnoses were previously established by a multidisciplinary panel that did not include rheumatologists. We reclassified patients as IPAF, CTD-ILD, or IPF. We compared transplant-free survival using Kaplan-Meier methods and identified prognostic factors using Cox models., Results: We identified 60 patients with IPAF, 113 with CTD-ILD, and 126 with IPF. Transplant-free survival of IPAF was not statistically significantly different from that of CTD-ILD or IPF. Among IPAF patients, male sex (hazard ratio, 4.58 [1.77-11.87]) was independently associated with worse transplant-free survival. During follow-up, only 10% of IPAF patients were diagnosed with CTD-ILD, most commonly antisynthetase syndrome., Conclusion: Despite similar clinical characteristics, most patients with IPAF did not progress to CTD-ILD; those who did often developed antisynthetase syndrome, highlighting the critical importance of comprehensive myositis autoantibody testing in this population. As in other types of ILD, male sex may portend a worse prognosis in IPAF. The routine engagement of rheumatologists in the multidisciplinary evaluation of ILD will help ensure the accurate classification of these patients and help clarify prognostic factors., Competing Interests: M.D.G. is supported by the National Institutes of Health Grant K23 AR073931-01 and has received consulting fees from AbbVie and research support from Bristol-Myers Squibb and GlaxoSmithKline for unrelated work. M.F.B. is supported by National Institutes of Health grant U01 HL152970, by The Robert L Mayock and David A Cooper Endowed Professorship, and via unrestricted funds from the Perelman School of Medicine Department of Medicine Research Fund. M.K.P. is supported by The William “Dutch” Dyson Fund. The other authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To investigate the impact of dysphagia on long-term survival and swallowing function outcomes in patients with idiopathic inflammatory myopathy other than inclusion body myositis., Methods: We retrospectively evaluated consecutive patients with idiopathic inflammatory myopathy other than inclusion body myositis to investigate the impact of dysphagia and its severity assessed using the Food Intake LEVEL Scale on survival and swallowing function outcomes. Time-to-event analyses were used, including Kaplan-Meier curves with log-rank (trend) test, cumulative incidence with Gray's test, and Cox proportional hazards models., Results: Of the 254 patients, 26 were dysphagic, including eight severe (Food Intake LEVEL Scale [FILS] score 2, 3) and six most severe (FILS score 1) cases; 210 were non-dysphagic, and 18 were indeterminate cases. During the 5 years after myositis diagnosis, 15 (57.7%) dysphagic and 31 (14.8%) non-dysphagic patients died, and dysphagic patients had significantly shorter survival. However, multivariate analysis showed that shorter survival was significantly associated with baseline age-adjusted Charlson Comorbidity Index (hazard ratio [HR] 1.57, 95% confidence interval [CI] 1.36-1.82]), but not with dysphagia (HR 1.46, 95% CI 0.69-3.10). Dysphagia severity was significantly associated with delayed recovery of dysphagia. In 20 non-severe or severe dysphagic cases, 19 restored swallowing function within 1 year. The most severe cases had a significantly higher cumulative probability of death before recovery from dysphagia than severe cases., Conclusion: The poor survival of dysphagic myositis patients was largely confounded by advanced age and comorbid malignancies. However, patients with the most severe dysphagia had a significantly worse swallowing function and survival prognosis than those with milder dysphagia., (© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: To investigate the clinical characteristics and risk factors for thromboembolic events in patients with idiopathic inflammatory myopathy (IIM)., Methods: We retrospectively analyzed 1144 consecutive patients with IIM for arterial and venous thromboses and compared them with age- and sex-matched IIM patients without thrombosis. Logistic regression analysis was used to analyze risk factors for thrombosis., Results: Twenty-four (2.1%) patients had arterial or venous thromboses (mean age, 62.6 ± 11.6 years; range, 33-81 years). Thromboembolic events occurred in 54.2% (13/24) of patients within 6 months before or after IIM diagnosis. Thrombosis patients had a higher Cutaneous Dermatomyositis Disease Area and Severity Index score (p = 0.028), higher myositis disease activity assessment visual analogue scale score (MYOACT) (p < 0.001), and a greater proportion of them had varicose veins (p = 0.001), surgical history in the past 3 months (p = 0.039), malignancy (p = 0.018), and infection (p < 0.001). The manual muscle test 8 score (p < 0.001) and albumin level (p = 0.003) were lower in thrombosis patients. There was no significant difference between the two groups in glucocorticoid pulse therapy; however, intravenous immunoglobulin therapy was more commonly used in thrombosis patients (p = 0.04). In multivariable regression models, malignancy, infection, longer duration of glucocorticoid treatment, and higher MYOACT were risk factors for thrombosis. The cumulative survival time of IIM patients with thrombosis was significantly shorter than that of controls., Conclusions: Malignancy, infection, longer duration of glucocorticoid use, and increased myositis disease activity are risk factors for thrombosis. Patients with these risk factors should undergo screening for thrombosis. Key Points • To investigate the clinical characteristics and risk factors for thromboembolism events in patients with IIM, we performed a retrospective study with IIM patients who experienced a thromboembolic event. • We found that malignancy, infection, longer duration of glucocorticoid treatment, and a higher level of myositis disease activity were risk factors for thrombosis. • The results suggest that patients with the above risk factors should undergo screening for thrombosis., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Objective: Heterogeneity exists among patients with myositis who have antinuclear matrix protein 2 (anti-NXP2) antibodies, although they usually present with severe muscle weakness. This study aimed to investigate the differences in phenotypes and prognoses among adult patients with myositis who have anti-NXP2 antibodies., Methods: Adult patients with myositis who have anti-NXP2 antibodies were enrolled from January 2010 to December 2019. Their clinical features and laboratory data were recorded retrospectively. We followed up on their survival status until June 30, 2020. A hierarchical cluster analysis, Kaplan-Meier curves, and classification and regression trees were used to analyze the data., Results: A total of 70 adult patients with myositis who have anti-NXP2 antibodies were enrolled. All patients experienced muscle weakness. A total of 11 patients did not present with rashes during disease progression, and 43 patients developed dysphagia. In total, 21 patients had interstitial lung disease (ILD), whereas no patients had rapidly progressive ILD. Hierarchical cluster analysis identified 2 clusters. Patients in cluster 1 were younger at disease onset, had a higher incidence of subcutaneous calcification, and had a lower incidence of V sign and shawl sign. Patients in cluster 2 had a higher frequency of ILD, accompanied by lower levels of lymphocytes and higher levels of serum ferritin. Moreover, patients in cluster 2 had worse prognoses., Conclusion: Patients with myositis who have anti-NXP2 antibodies may present with different phenotypes that are characterized by unique features and prognoses., (Copyright © 2022 by the Journal of Rheumatology.)

A patient in her mid-30s presented to hospital at 25 weeks' gestation with acute onset of leg pain.Routine investigations were performed to rule out the common causes of leg and back pain in pregnancy, which were grossly normal. Piriformis pyomyositis was diagnosed on MRI and a collection was drained. Following an initial response to antibiotic therapy, the patient delivered by elective caesarean section, but the pain returned on postnatal day 2 and muscle inflammation was diagnosed again, requiring a repeat course of antibiotics.This case highlights a rare cause of leg pain in a pregnant patient, and the additional complexities of managing cases in the obstetric population., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

This paper introduced the following information on the purpose of the consensus, process, writing format, formation of expert opinions, and the issues that remain to be solved and reflections, hoping to provide reference for readers to understand the content of this consensus and rational choice of application.

Idiopathic inflammatory myopathies (IIM), a group of heterogenous autoimmune diseases, are characterized by proximal, skeletal muscle involvement of chronic immunological pathogenesis. IIM associated interstitial lung diseases (IIM-ILDs) are the most important causes of morbidity or mortality for IIM patients. Patients with IIM-ILDs always exhibit diversity of diseases manifestations including onset pattern, diseases progress, therapeutic respond and prognosis. Hence, the diagnosis and evaluation of IIM-ILDs usually need multidisciplinary consultations. And we have to face some dilemmas, such as sophisticated choice of immunosuppressants due to limited prospective research data, and personnel and different responses to therapeutic remedy. It is an urgent task to develop a consensus on the diagnosis and treatment of IIM-ILDs for guiding the routine practice of clinicians. Experts from pulmonology, rheumatology, and radiology were organized by Respiratory Council of Chinese Research Hospital Association, and developed a consensus based on previous studies and the experts ' experiences. This consensus mainly illustrated the epidemiology, clinical characteristics, diagnosis and evaluation, therapeutic remedy for IIM-ILDs. After full discussion and voting by the expert group, a total of 18 recommendations were formed, including 7 recommendations on diagnosis and evaluation, and 11 recommendations on the choice of therapeutic strategies. This consensus is intended to facilitate the standardization of diagnosis and treatment of patients with IIM-ILD and promote the development of relevant clinical and basic research in China.

A man in his 50s was admitted with 4 months of myalgia, headaches, hypercalcaemia and declining renal function on a background of lung transplantation for cystic fibrosis 5 years prior. MRI confirmed myositis and a muscle biopsy revealed invasive muscular microsporidial infection. Positron emission tomography(PET)/CT revealed widespread dissemination of the infection. Albendazole was commenced and after a 1 week systemic inflammatory response syndrome, the patient made a significant recovery and was discharged home. PCR testing confirmed the species as Anncaliia algerae , which is known to infect mosquitoes, larvae and contaminate water supplies. This case highlights the need to relentlessly pursue a diagnosis and to consider atypical pathology in immune compromised patients. A tissue sample yielded highly beneficial and unexpected results. A multispecialty approach was essential given the varied infection manifestations, which included myositis, keratitis and possible central nervous system, vocal cord, parapharyngeal and renal involvement., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND Staphylococcus aureus (SA) is a rare cause of prostatic abscess. Risk factors include genito-urinary instrumentalization and immunocompromised states. Because of the lack of guidelines on the diagnosis, management, and follow-up of SA prostate abscess, the diagnosis can sometimes be challenging. Our patient was a 60-year-old man who initially presented with lower back pain and was diagnosed with a methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia, prostate abscess, osteomyelitis, and myositis. CASE REPORT A 60-year-old man presented with lower back pain. He had a past medical history of incompletely treated MSSA cervical osteomyelitis with epidural abscess, alcohol use disorder, intravenous drug use (IVDU), and poorly controlled diabetes mellitus (DM). He was afebrile and hemodynamically stable. Laboratory test results revealed leukocytosis and an elevated C reactive protein (CRP). Lumbar spine magnetic resonance imaging (MRI) showed vertebral osteomyelitis and right psoas myositis. Blood cultures isolated MSSA. The patient was treated with vancomycin and piperacillin-tazobactam. On day 5, our patient reported having fever, chills, flank pain, and dysuria. Computed tomography (CT) revealed a 4.0×4.9 cm prostatic abscess. CT-guided percutaneous abscess drainage was performed, and fluid culture revealed MSSA. Both antibiotics were discontinued and cefazolin was started following sensitivities. Post-drainage pelvic ultrasound (US) showed resolution of the abscess. CONCLUSIONS This case highlights the importance of a rapid diagnosis of SA prostate abscess in patients with documented risk factors and characteristic symptoms. Timely management with antibiotics and drainage as indicated are imperative to avoid further complications from the underlying bacteremia, including sepsis and metastatic infections.

Background: Inflammatory cardiomyopathy (IC) is a syndrome with chronic myocarditis and cardiac remodeling. This study aimed to explore predicting factors of adverse outcomes in patients with IC secondary to idiopathic inflammatory myopathy (IIM-IC)., Methods: By means of a single-center retrospective study, 52 patients with IIM-IC at Peking Union Medical College Hospital were identified from January 1999 to June 2019. Electrocardiogram and echocardiography data were analyzed for the primary outcome (defined as all-cause death) and secondary outcomes (defined as re-hospitalization of heart failure and all-cause death), using regression and survival analysis., Results: The prevalence of atrial fibrillation, ventricular tachycardia, Q-wave abnormality, left ventricular conduction abnormalities, and reduced left ventricular ejection fraction (LVEF) (≤40%) were 65.4%, 67.3%, 67.3%, 61.6%, and 50.5%. After a median follow-up of 2 years (IQR 0.8-3.0), 26 cases were readmitted due to heart failure. Twenty-two deaths were recorded, including 20 cardiogenic deaths. Among the patients with adverse events, the incidence of poor R-wave progression, low-voltage of the limb leads, Q-wave abnormality, QRS duration >130 ms, left ventricular enlargement, and impaired systolic function were higher. Kaplan-Meier analysis showed that Q-wave abnormality, limb leads low-voltage, LVEF ≤40%, and left ventricular end-diastolic dimension >60 mm were correlated with shorter survival. However, multivariate Cox regression analysis revealed that only Q-wave abnormality (HR = 12.315) and LVEF ≤40% (HR = 5.616) were independent risk factors for all-cause death., Conclusion: Q-wave abnormality and reduced LVEF are predictive of poor prognosis in patients with IIM-IC., (© 2022 The Authors. Annals of Noninvasive Electrocardiology published by Wiley Periodicals LLC.)

Objective: Myositis-specific antibodies (MSAs) facilitate grouping children with juvenile dermatomyositis (DM) into distinct phenotypes. The first aim of this study was to investigate the link between anti-p155/140 and lipodystrophy as determined by dual x-ray absorptiometry (DXA) assessment of fat distribution. The second aim was to examine the relationship between anti-p155/140 and damage to the nailfold capillary system., Methods: Children with juvenile DM followed for a minimum of 5 years were included. The study population was divided into 3 groups (anti-p155/140, other MSA, and MSA negative). Lipodystrophy was assessed by physician assessment and DXA fat distribution (trunk-to-leg fat ratio). Documentation of nailfold capillary end row loops (ERLs) was obtained at diagnosis., Results: A total of 96 subjects (44% anti-p155/140, 23% other MSA, 33% MSA negative) were included. There was no significant difference in age, disease activity scores, or lipodystrophy between the 3 groups. The trunk-to-leg fat ratios were similar among the 3 groups at different time points. However, the anti-p155/140 group had significantly decreased ERL counts (P = 0.006) at baseline as well as a prolonged duration of untreated disease at diagnosis (P = 0.027). Also, the anti-p155/140 group had fewer patients with a monophasic disease course than the other 2 groups (P = 0.008)., Conclusion: Generalized lipodystrophy frequency was equivalent in all 3 groups based on physician assessments and trunk-to-leg fat ratios. The anti-p155/140 group had a greater loss of ERLs, suggesting that this MSA may impact the vascular component of juvenile DM., (© 2020 American College of Rheumatology.)

To study the demographic, clinical and serologic characteristics of anti-MDA5-positive DM from two geographically and ethnically disparate inception cohorts in India. To identify the clinical and serological parameters at inception that could predict mortality among these individuals. Individuals with anti-MDA5 antibody-positive DM diagnosed between 2017 and 2020 from two centres in India were prospectively followed up. The clinical and serological characteristics at baseline and the treatment outcome at follow-up were assessed for this study. Anti-MDA5 antibody was positive in 25 (7.5%) out of the 330 individuals with myositis. These 25 (21 adults, 4 juvenile) patients were followed up for a median duration of 14 months. Among adults, a majority had cutaneous manifestations 21 (84%) followed by, arthritis 17 (80%), and interstitial lung disease 12 (ILD, 57.1%). Four (19%) had rapidly progressive ILD (RP-ILD). Eight (38%) presented as clinically amyopathic DM. Among cutaneous manifestations, majority (62%) had classic features (gottron's papules/sign, heliotrope rash) while 8 (38%) had cutaneous ulceration and 2 each had periorbital edema and tendon rupture. Eight (38%) were positive for anti-Ro-52 antibody. Out of 21 adults, 8 (38%) succumbed to the diseases. RP-ILD (n = 4; 19%), ulcerative gottron's (n = 5) and anti-Ro-52 (n = 8) were significantly associated with mortality (p < 0.05). Upon binary logistic regression, positive anti-Ro-52 antibody predicted mortality [HR 17.3 (95%CI 1.4-210, p = 0.025)]. All juvenile anti-MDA5-positive DMs had classic cutaneous features with 2 of them having ulcerative gottron's. None of the juvenile patients had ILD and everyone survived till the last follow-up. Indian adults with anti-MDA5 DM have high mortality. Rarer atypical features like tendon rupture or periorbital edema could assist in diagnosis. Ulcerative gottron's, positive anti-Ro 52 antibodies, and RP-ILD are valuable clinical-serological markers that portend poor prognosis., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

We performed a systematic review of the clinical manifestations and complementary exams of patients with myopathies and systemic sclerosis overlap syndrome (MyoSScOS). Systematic review from January 1976 to November 2021 according PRISMA protocol on three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appears in the title; written in English, Portuguese, or Spanish; and addresses MyoSScOS. Brief communications, reviews, studies that addressed myopathies in children, congress proceedings, monographs, and dissertations were excluded. Thirty-five articles were selected. MyoSScOS seems to be more common in women. It also commonly affects the esophagus and joints with symmetrical and bilateral muscle involvement, Raynaud's phenomenon, and impairment of forced vital capacity. Concerning SSc, the most common subtype was the diffuse form. Cardiovascular and pulmonary complications are an important cause of death. Anti-centromere, anti-PM/Scl, anti-Scl70, anti-RNA polymerase III, anti-Ku, and anti-RNP were more correlated with this entity, and muscle biopsies may present a more aggressive pattern. Electroneuromyography patterns are quite similar to those found in inflammatory myopathies. The absence of studies with robust methodologies and the large number of case reports and series make more robust statistical analyses such as meta-analyses unfeasible. The characterization of MyoSScOS is important for the formulation of therapeutic measures and specific treatments aiming at better quality of life and prognosis. Greater and better theoretical contributions are necessary to better characterize it., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Background: Melioidosis caused by Burkholderia pseudomallei is an emerging infection in Sri Lanka with a high case fatality rate. The disease usually manifests as pneumonia, however multisystem involvement is common. Myositis is an extremely rare occurrence and this is the only documented case where the initial presentation of melioidosis has been myositis and later complicated to myonecrosis., Case Presentation: A 45-year-old gentleman with pre-existing diabetes presented with a tender, right thigh lump for 1 week duration without any history of trauma or infection. Investigations revealed neutrophil leukocytosis, high erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels whilst ultrasonography showed focal myositis of right quadriceps. The patient went into sepsis amidst antibacterial treatment which warranted urgent surgery. At surgery, a large intramuscular abscess with myonecrosis was observed within vastus medialis which was completely drained and pus was taken for culture which eventually isolated Burkholderia pseudomallei. Melioidosis was diagnosed and intravenous meropenem was prescribed for 3 weeks. Following complete recovery, the patient was discharged on doxycycline and trimethoprim sulfamethoxazole for 3 months., Conclusions: Melioidosis, an endemic disease in south east Asia and northern Australia, is an emerging infection in Sri Lanka. Myositis is a rare presentation of the disease that can lead to myonecrosis and abscess formation which can cause rapid disease escalation and sepsis. Early surgical intervention may be life-saving in such cases where antibiotic therapy alone may not suffice., (© 2022. The Author(s).)

»: COVID-19 is a disease that is challenging science, health-care systems, and humanity. An astonishingly wide spectrum of manifestations of multi-organ damage, including musculoskeletal, can be associated with SARS-CoV-2., »: In the acute phase of COVID-19, fatigue, myalgia, and arthralgia are the most common musculoskeletal symptoms., »: Post-COVID-19 syndrome is a group of signs and symptoms that are present for >12 weeks. The associated musculoskeletal manifestations are fatigue, arthralgia, myalgia, new-onset back pain, muscle weakness, and poor physical performance., »: Data on COVID-19 complications are growing due to large absolute numbers of cases and survivors in these 2 years of the pandemic. Additional musculoskeletal manifestations encountered are falls by the elderly, increased mortality after hip fracture, reduced bone mineral density and osteoporosis, acute sarcopenia, rhabdomyolysis, Guillain-Barré syndrome, muscle denervation atrophy, fibromyalgia, rheumatological disease triggering, septic arthritis, adhesive capsulitis, myositis, critical illness myopathy, onset of latent muscular dystrophy, osteonecrosis, soft-tissue abscess, urticarial vasculitis with musculoskeletal manifestations, and necrotizing autoimmune myositis., »: A wide range of signs and symptoms involving the musculoskeletal system that affect quality of life and can result in a decrease in disability-adjusted life years. This powerful and unpredictable disease highlights the importance of multimodality imaging, continuing education, and multidisciplinary team care to support preventive measures, diagnosis, and treatment., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSREV/A841)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Idiopathic inflammatory myositis (IIM) is a group of rare diseases of unknown etiology, with a pathognomonic muscular deficiency. Antisynthetase syndrome is a subtype of IIM with an associated interstitial lung disease (ILD), characterized by pulmonary inflammation and fibrosis mediated by TGF-β. Pirfenidone is a new molecule with anti-inflammatory and anti-fibrotic properties, used for the treatment of idiopathic ILD, but has never been assessed in IIM. The aim of our study is to evaluate the effect of pirfenidone on IIM-associated ILD. Thirty-two BALB/c male mice were divided into three groups: Sham, IIM-untreated (IIM), and IIM pirfenidone-treated (IIM + PIR). IIM was induced by intramuscular injections of guinea pig muscle myosin extract and intraperitoneal injections of Pertussis toxin. Pirfenidone was given orally at a dose of 30 mg kg -1  day -1 for two months. Muscle force, blood and bronchoalveolar lavage fluid samples, as well as muscle and lung tissues, were analyzed. Progressive deterioration of muscle force and infiltration of the muscular tissue by inflammatory cells were observed with IIM. Auto-immune antibodies specific to the antisynthetase syndrome were also increased in IIM mice. Pirfenidone attenuated IIM-associated ILD with anti-inflammatory properties evidenced by decreased peribronchial inflammation and TGF-β1 in bronchoalveolar lavage fluid. Likewise, pirfenidone attenuated pulmonary fibrosis by fine-tuning TGF-β1-mediated epithelial-to-mesenchymal and fibrotic signaling pathways; pro-fibrotic SMAD3, ZEB2 and STAT1 expression and activation were decreased, whereas anti-fibrotic SMAD2 activation was increased. This study unravels for the first time that pirfenidone has the potential to fine-tune TGF-β1 fibrotic signaling in IIM-associated ILD., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Inclusion body myositis (IBM) is a slowly progressive muscle disease affecting ageing individuals. IBM presents with a distinctive pattern of weakness involving the quadriceps and finger flexor muscles, although other muscles including pharyngeal muscles become affected over time. Pathological hallmarks of IBM include autoimmune features, including endomysial infiltration by highly differentiated T cells, as well as degenerative features marked by intramyofibre protein aggregates organised into inclusion bodies. Despite some progress in understanding the cellular pathways involved in IBM, it remains untreatable, and the progression of the disease leads to progressive weakness, disability, wheelchair dependency and loss of independence. Therefore, there is an urgent need to improve our understanding of the underlying mechanisms and pathways involved in this disease to identify new treatment targets. Here, we discuss the current understanding of aetiopathogenesis, the interrelationship between autoimmunity and degeneration, and how ageing is a major influencer of both these features., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Inflammatory myopathies are heterogeneous clinico-serological syndromes, with variable clinical manifestations. Interstitial lung disease (ILD) is a major cause of morbidity and mortality in patients with myositis. The clinical manifestation of myositis-ILD is heterogeneous, e.g., with acute-on-chronic presentations, as well as the chronic aftermath of acute disease. Here, we have largely divided myositis-ILD into three main prognostic groups which require different treatment approaches: mild-moderate (subacute), severe or progressive (acute or subacute) and rapidly progressive, life-threatening. In current clinical practice, the treatment of myositis-ILD involves immunomodulation in an induction-maintenance treatment paradigm. There is now an option to add antifibrotics to slow the progression of established fibrosis in selected cases with chronic progressive phenotype. Here, we describe current concepts in myositis-ILD and aim to provide a practical guide for clinicians on how to approach assessment, including early identification of ILD, phenotyping of patients according to clinical trajectory and likely prognosis and stratified management adopting multi-disciplinary cross-speciality expertise, with close collaboration between rheumatology and respiratory physicians., (Copyright © 2022 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

We have reported a case of a 44-year-old woman with anti-signal recognition particle (SRP) antibody-positive immune-mediated necrotizing myopathy triggered by human parvovirus B19 (PVB19) infection. She was admitted to the hospital because of lower leg edema and muscle weakness after erythema infectiosum. Magnetic resonance imaging of the lower extremities revealed high signals in the proximal muscles and subcutaneous edema on STIR. Muscle biopsy showed myofiber regenerative changes and variation in fiber size. A myositis-specific autoantibody profile indicated a positive result for anti-SRP antibodies. We diagnosed the patient with immune-mediated necrotizing myopathy (IMNM). Muscle strength and subcutaneous edema improved gradually in 3 months following immunotherapy. This is the first case report of an IMNM associated with PVB19 infection.

Objective: To determine whether histopathological, electromyographic and laboratory markers correlate with clinical measures in inclusion body myositis (IBM)., Methods: We reviewed our electronic medical records to identify patients with IBM according to European Neuromuscular Center (ENMC) 2011 criteria, seen between 2015 and 2020. We only included patients who had a muscle biopsy and needle electromyography (EMG) performed on the same muscle (opposite or same side). We used a detailed grading system [0 (normal) to 4 (severe)] to score histopathological and EMG findings. Clinical severity was assessed by the modified Rankin scale (mRS), muscle strength sum score (SSS), quadriceps strength and severity of dysphagia on swallow evaluation. Serum markers of interest were creatine kinase level and cN-1A antibodies., Results: We included 50 IBM patients, with a median age of 69 years; 64% were males. Median disease duration at diagnosis was 51 months. On muscle biopsy, endomysial inflammation mainly correlated with dysphagia, and inversely correlated with mRS. Vacuoles and congophilic inclusions did not correlate with any of the clinical measures. On EMG, the shortness of motor un it potential (MUP) duration correlated with all clinical measures. Myotonic discharges, and not fibrillation potentials, correlated with the severity of inflammation. Serum markers did not have a statistically significant correlation with any of the clinical measures., Conclusions: Dysphagia was the main clinical feature of IBM correlating with endomysial inflammation. Otherwise, inclusion body myositis clinical measures had limited correlation with histopathological features in this study. The shortness of MUP duration correlated with all clinical measures., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: 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., Methods: 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., Results: 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]., Conclusions: 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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Chen, Wang, Zhang, Zuo, Ye, Wang and Shu.)

Objectives: This study aimed to quantify nailfold capillary (NFC) abnormalities in anti-melanoma differentiation-associated gene 5 (MDA5) -positive DM patients and to evaluate the association with clinical parameters, including serum biomarkers. In addition, we aimed to clarify the period leading to remission of NFC abnormalities during immunosuppressive treatment in patients with DM., Methods: A prospective observational study was conducted including patients (n = 10) who first visited Hiroshima University Hospital and were diagnosed with DM or clinically amyopathic DM with anti-MDA5 antibodies. We compared the NFC abnormalities detected by nailfold-video capillaroscopy (NVC), physical findings, blood tests, respiratory function tests, and vascular-related growth factors measured using a LEGENDplexTM Multi-Analyte Flow Assay Kit., Results: NFC abnormalities improved in all patients from 2 to 17 weeks after the initiation of immunosuppressive treatment. The NVC scores were inversely correlated with anti-MDA5 antibody titres at baseline. NVC scores and forced vital capacity were positively correlated. Baseline values of M-CSF and stem cell factor were correlated with anti-MDA-5 titres., Conclusion: Our study suggested that NVC scores and disease activity were inversely correlated before treatment. Vascular-related growth factors, such as M-CSF and stem cell factor, may be associated with the disease mechanism in patients with anti-MDA5 antibody-positive DM., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To evaluate survival and associated comorbidities in inclusion body myositis (IBM) in a population-based, case-control study., Methods: We utilized the expanded Rochester Epidemiology Project medical records-linkage system, including 27 counties in Minnesota and Wisconsin, to identify patients with IBM, other inflammatory myopathies (IIM), and age/sex-matched population-controls. We compared the frequency of various comorbidities and survival among groups., Results: We identified 50 IBM patients, 65 IIM controls and 294 population controls. Dysphagia was most common in IBM (64%) patients. The frequency of neurodegenerative disorders (dementia/parkinsonism) and solid cancers was not different between groups. Rheumatoid arthritis was the most common rheumatic disease in all groups. A total of 36% of IBM patients had a peripheral neuropathy, 6% had Sjögren's syndrome and 10% had a haematologic malignancy. T-cell large granular lymphocytic leukaemia was only observed in the IBM group. None of the IBM patients had hepatitis B or C, or HIV. IBM patients were 2.7 times more likely to have peripheral neuropathy, 6.2 times more likely to have Sjögren's syndrome and 3.9 times more likely to have a haematologic malignancy than population controls. IBM was associated with increased mortality, with a 10-year survival of 36% from index, compared with 67% in IIM and 59% in population controls. Respiratory failure or pneumonia (44%) was the most common cause of death., Conclusions: IBM is associated with lower survival, and higher frequency of peripheral neuropathy, Sjögren's syndrome and haematologic malignancies than the general population. Close monitoring of IBM-related complications is warranted., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Background and Objectives: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up., Methods: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model., Results: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI., Conclusions: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern., (Copyright © 2021 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Myositis-specific autoantibodies are relevant factors that define the disease phenotype of dermatomyositis (DM). Anti-Mi-2 antibody-positive DM patients may present with the typical skin lesions and prominent myositis. On the other hand, adult DM patients with anti-TIF-γ antibody seem to be associated with internal malignancy. Here, we report a rare case of juvenile dermatomyositis (JDM) exhibiting anti-Mi-2 and anti-transcriptional intermediary factor-1 gamma (TIF1-γ) antibodies, with no internal malignancy. A 16-year-old female Japanese patient under treatment with a 2-year history of chronic eczematous lesions was admitted to our department with elevated levels of muscle enzymes. Characteristic skin changes, such as Gottron's papules of the hand, heliotrope rash of the eyelids, and poikiloderma-like legions and diffuse pigmentation on the back, were observed. Histologically, the patient's skin was characterized by the presence of lymphocytic vascular inflammation and endothelial swelling, which are consistent with DM. Severe symmetric proximal muscle weakness, elevated serum muscle enzymes and the presence of anti-TIF1-γ and Mi-2 antibodies were noted. The diagnosis of JDM was made according to the European League Against Rheumatism (EULAR) diagnostic criteria. A high dose of corticosteroids and following intravenous cyclophosphamide treatment (750 mg three times) resulted in an improvement in clinical manifestations and functional outcomes, and recurrence did not occur. Estimation of autoantibodies may serve as an ancillary tool in delineating and defining distinct clinical phenotypes in JDM.

Objectives: Recent studies have confirmed that heart failure is one of the most important causes of death in patients with idiopathic inflammatory myopathy (IIM). Left ventricle diastolic dysfunction (LVDD) is closely associated with heart failure. Our aim is to determine if the prevalence of LVDD is increased in IIM patients., Methods: We performed a time- and language-restricted literature search to identify studies conducted to compare the echocardiographic parameters in IIM patients and controls. Mean differences were used to calculate the effect sizes of the echocardiographic parameters., Results: A total of 13 studies met the inclusion criteria and comprised a total of 227 juvenile dermatomyositis (JDM) patients, 391 adult IIM patients, and 550 controls. The adult IIM patients had lower mitral annular early diastolic velocity (e') and peak of early diastolic flow velocity/peak of late diastolic flow velocity (E/A) ratio compared to controls. The mean left atrial dimension and E/e' ratio was higher in adult IIM patients as compared to controls. Similarly, in JDM patients, the decreased e' was also observed., Conclusion: Patients with IIM were more likely to have echocardiographic parameters indicative of diastolic dysfunction. The early heart assessments should be performed in IIM patients., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: Peripheral muscle involvement in SSc may comprise myositis or a non-inflammatory myopathy. There is little understanding of the nature of SSc myopathy. This pilot study aimed to evaluate the presence of diffuse fibrosis in the peripheral muscle of patients with SSc by determining extracellular volume (ECV) MRI measurement., Methods: SSc patients, with either suspected myopathy or no muscle involvement, and healthy controls (HCs) had native T1 and ECV MRI quantification of the thigh and creatine-kinase (CK) measured. Suspected myopathy was defined as current / history of minimally raised CK (>320; <600 IU/l) ± presence of clinical signs/symptoms (including proximal lower-limb muscle weakness and/or myalgia) ± a Manual Muscle Testing (MMT) 8 score of <5 in the thighs., Results: Twelve SSc patients and 10 HCs were recruited. Of the 12 patients, 9 had limited cutaneous SSc, 4 had interstitial lung disease, and 7 had suspected myopathy. The higher skeletal muscle ECV was recorded for SSc patients compared with HCs [mean (s.d.) 23 (11)%, vs 11 (4)%, P = 0.04]. Peripheral muscle ECV was associated with CK (rho = 0.554, P = 0.061) and was higher in SSc patients with myopathy than in those with no myopathy [mean (s.d.) 28 (10) vs 15 (5), P = 0.023]. It was determined that an ECV of 22% best identified myopathy (with a sensitivity of 71% and a specificity of 80%)., Conclusion: This hypothesis-generating study showed higher ECV in SSc patients compared with HCs, as well as association of ECV with suspected myopathy, suggesting the presence of diffuse fibrosis in the peripheral muscle of SSc patients. Further studies are needed to understand the nature of SSc myopathy., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Overlap syndromes account for about 25% of autoimmune diseases. They are many possible combinations of known autoimmune diseases increasingly diagnosed with the identification of of a large number of autoantibodies. In this case report, we present a patient with rare overlapping rheumatoid arthritis-antisynthetase syndrome with associated secondary Sjögren's syndrome atypically presenting without interstitial lung disease., Case Presentation: A 52-year-old Sinhalese female, a known patient with type 2 diabetes mellitus, presented with a history of symmetrical inflammatory-type polyarthritis with significant morning stiffness, proximal muscle weakness, pain, and roughening of the fingertips with associated sicca symptoms of 5 months duration. Examination revealed features of active joint inflammation, mechanic's hand, xerostomia, and left-sided breast lump. Investigations confirmed the presence of rheumatoid arthritis with strongly positive rheumatoid factor (202 U/ml) and anti-cyclic citrullinated peptide antibody (717 U/ml). Antisynthetase syndrome was also diagnosed with borderline-positive anti-aminoacyl-tRNA antibodies but without interstitial lung disease. Sjögren's syndrome was confirmed by the clinical history and histology and considered a secondary disorder. As her breast lump proved to be benign, no further interventions were done. She was started on sulfasalazine and methotrexate with steroid bridging therapy and achieved remission and had good control of the disease without any joint deformity or flare-up on 6-month clinic follow-up., Discussion: Overlapping rheumatoid arthritis-antisynthetase syndrome is a very rare disease with disabling complications. Early identification of the atypical presentations of the overlap syndromes, by thorough investigations, helps physicians to prescribe proper disease-modifying antirheumatoid drugs and biological drugs. It also helps predict the prognosis of the patients before they develop complications., (© 2022. The Author(s).)

A woman in her late 30s recently diagnosed with viral myopericarditis presented with 1 month of worsening fatigue, diffuse myalgias and chest pain radiating to her back. While undergoing work-up for chest wall myositis, she rapidly decompensated, developing heart failure and acute hypoxaemic respiratory failure. Her clinical course was complicated by cardiac arrest and severe cardiogenic shock requiring intra-aortic balloon pump support., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Interstitial lung disease (ILD) occurs with Idiopathic Inflammatory Myopathy (IIM) as a life-threating complication and is considered the most important prognostic determinant in this disease group. The antibody anti-melanoma differentiation-associated gene 5 (anti-MDA5) is associated to rapidly progressive ILD and poor overall survival. Rituximab (RTX) is becoming a drug of choice in management of refractory IIM-ILDs and rapidly progressive ILDs, despite its low level of evidence. We report the case of a 49-year-old man with new-onset clinically amyopathic dermatomyositis (CADM) with severe respiratory symptoms and mixed radiologic pattern of non-specific interstitial and organizing pneumonia, refractory to high dose corticosteroids and intravenous immunoglobulin and oxygen dependent. He was started on RTX 375mg/m2/week of which he completed 4 perfusions, with significant clinical improvement, and has been on maintenance to date with the rheumatology RTX standard protocol with no need for oxygen supplementation. RTX may represent a rescue therapy for patients with severe anti-MDA5-related CADM-ILD refractory to conventional immunotherapies. We identified reports of a total of 12 patients treated with RTX. Infection was the only reported adverse event (25%). Respiratory improvement (defined by symptoms, imaging or PFTs) was observed in 75% of patients, with 2 (17%) having achieved clinical remission. A total of three deaths occurred (25%), all resulting from ILD progression despite treatment. No therapeutic protocol with RTX seems to be more efficient nor associated with more adverse events than the others. Comparative studies are necessary.

We report the case of a 79-year-old woman with hepatocellular carcinoma (HCC) who presented with creatine kinase (CK)-MM elevation. On admission, her serum CK-MM level exceeded 4000 IU/L (normal, 44-206 IU/L), and computed tomography revealed two HCCs in hepatic segment VIII (23 mm, 86 mm). The patient denied experiencing muscular symptoms such as weakness or pain. Hypothyroidism, ischemic heart disease, muscular dystrophy, autoimmune myopathy, drug-induced rhabdomyolysis, and paraneoplastic inflammatory myositis syndrome (PIMS) were included in the differential diagnosis for high CK-MM, but none were suspected. Although the cause of elevated CK-MM was not elucidated, an HCC-related mechanism was considered and the tumor was resected. The CK-MM levels declined gradually to 300 IU/L postoperatively without any special perioperative management. Nineteen cases of HCC-associated CK-MM elevation have been reported in English thus far, in all of which, inflammatory myositis was concluded as the cause of CK-MM elevation. However, in this case, the elevation of CK-MM was associated with HCC-related mechanisms distinct from PIMS, suggesting HCC-related mechanisms should not be excluded as a cause of high CK-MM, even though PIMS is negative., (© 2022. Japanese Society of Gastroenterology.)

A female in her late 40s presented to the emergency department during the COVID-19 pandemic with shortness of breath, fever and productive cough following a recent diagnosis of bilateral non-massive pulmonary emboli. She had elevated inflammatory markers and her chest X-ray revealed bilateral infiltrates. Her SARS-CoV-2 PCR was negative, and she was treated for community-acquired pneumonia. However, despite treatment she rapidly deteriorated and developed severe respiratory failure, requiring mechanical ventilation.On further investigation, she tested positive for anti-Jo-1 antibodies and a diagnosis of antisynthetase syndrome sine myositis was made. This led to successful treatment with high dose corticosteroids and intravenous immunoglobulin.This case highlights an uncommon presentation of a rare condition, as well as the benefits of working in a multidisciplinary team on the intensive care unit., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)