Prevalence of hearing loss, tinnitus, vertigo and sudden deafness among patients with polymyositis and dermatomyositis.

Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to explore differences in the prevalence of inner ear symptoms among patients with and without PM/DM using a nationwide population-based dataset. Data for this study were retrieved from the Taiwan National Health Insurance Research Database. The study sample included 1622 patients diagnosed with PM/DM and 8109 propensity-score matched comparison patients without PM/DM. We performed multivariate logistic regressions to calculate odds ratios (ORs) and 95% confidence interval (CI) for tinnitus, hearing loss, sudden deafness, and vertigo among patients with PM/DM versus comparison patients. Chi-square tests showed statistically significant differences between patients with PM/DM and comparison patients in the prevalence of tinnitus (16.1% vs. 12.7%, p < 0.001), non-conductive hearing loss (9.2% vs. 6.8%, p < 0.001), and vertigo (14.4% vs. 11.1%, p < 0.001). The adjusted ORs for tinnitus, non-conductive hearing loss, and vertigo, respectively, were 1.332 (95% CI = 1.147-1.547), 1.399 (95% CI = 1.154-1.696), and 1.374 (95% CI = 1.173-1.611) for patients with PM/DM when compared to comparison patients. Our study finds that patients with PM/DM have higher prevalence rates of tinnitus, non-conductive hearing loss, and vertigo than comparison patients.

A bibliometric analysis of the research status and trends in studies on polymyositis and dermatomyositis with interstitial lung disease from 2000 to 2022 using Web of Science.

BACKGROUND: The main subtypes of idiopathic inflammatory myopathies (IIMs)-polymyositis (PM) and dermatomyositis (DM)-are often presented as interstitial lung disease (ILD) in clinical practice; therefore, many researchers have combined the three studies into PM/DM with ILD. METHODS: Using bibliometrics, the research status, progress, and hotspots of PM/DM with ILD between 2000 and 2022 were studied. Literature data on PM/DM with ILD were retrieved from the Web of Science (WoS) database for the research period. Visualization software, including VOSviewer, Pajek, CiteSpace, and Scimago Graphica were used for bibliometric analysis. RESULTS: A total of 1555 relevant articles were obtained, and the overall research in this field showed an increasing trend. Regarding contributing countries and venues, Japan published the most articles while Rheumatology was the most prolific journal. Regarding authors, the most published article was by Wang Guochun from Changchun University of Technology in China. Keyword analysis and cocited literature cluster analysis showed that diagnosis, classification, autoantibodies, antibodies, prognosis, complications, and treatment of PM/DM with ILD have been hot topics in this field recently. Moreover, our study shows that anti-mda5 antibody, mortality, gene 5 antibody, IIMs, double-blind, and prognostic factors, among others, may be new hot topics. CONCLUSION: This study found that research on PM/DM with ILD has increased over time, and scholars are paying more attention to this field. The development of new drugs for the management, treatment, and prevention of PM/DM with ILD is the primary task of researchers and a direction for future research in this field.

Low utilization of statins in patients with dermatomyositis/polymyositis and hyperlipidemia: a multicenter USA-based study (2013-2023).

OBJECTIVE: While the cardioprotective benefits of statins for rheumatoid arthritis (RA) patients are well-established, there might be a hesitation in recommending them for dermatomyositis/polymyositis (DM/PM) patients with hyperlipidemia (HLD), particularly with myopathy. We sought to contrast statin prescription patterns between DM/PM-HLD and RA-HLD patients and delve into the mortality variations among DM/PM-HLD statin users and non-users. METHODS: We examined a decade's worth of anonymized US health data from the TriNetX database. Inclusion criteria were a subsequent HLD diagnosis after an initial DM/PM or RA diagnosis. We compared statin initiation rates and mortality outcomes, adjusting for demographics and cardiovascular risks through propensity score matching. RESULTS: The analysis comprised 33,000 RA-HLD and 1079 DM/PM-HLD patients. RA-HLD patients exhibited higher statin initiation (27.4%) than DM/PM-HLD patients (17.91%, p < 0.0001). Notably, DM/PM-HLD statin users (n = 311) presented a reduced mortality rate (75 deaths/1000/year) compared to non-users (n = 661) with 147 deaths/1000/year (p = 0.0273, HR = 0.515, CI 0.28-0.93). CONCLUSION: There is a marked disparity in statin initiation between DM/PM-HLD and RA-HLD patients, accompanied by elevated mortality in DM/PM-HLD non-users. It is imperative for further research to elucidate this discrepancy and formulate patient-centric cardiovascular guidelines for DM/PM-HLD patients. Key Points • Statin initiation among patients with DM/PM-HLD is significantly lower than that with RA-HLD. • Mortality rates within the statin initiator DM/PM-HLD were significantly lower compared to non-statin DM/PM-HLD initiators, spanning multiple time intervals.

Inflammatory biomarkers in polymyositis/dermatomyositis patients with interstitial lung disease: a retrospective study.

BACKGROUND: Dermatomyositis (DM)/polymyositis (PM) is a systemic autoimmune disease characterized by proximal limb muscle with high morbidity and mortality and poor prognosis mediated by immune dysfunction; its etiology is unknown. DM/PM patients are at excessive risk of interstitial lung disease (ILD) and a higher risk of death. However, the role of circulating lymphocyte subsets, which play a pivotal role in occurrence and progression of DM/PM and ILD, respectively, remains unclear in DM/PM patients with ILD. METHODS: Demographic characteristics, general data, and peripheral lymphocyte levels measured by flow cytometry were collected and analyzed in 47 DM/PM patients with ILD, 65 patients without ILD, and 105 healthy controls (HCs). RESULTS: The most important first symptom of DM/PM patients is rash. Compared with non-ILD patients, the levels of neutrophil/lymphocyte ratio (NLR), systemic inflammatory response index (SIRI) were significantly higher and the levels of C reactive protein (CRP) were significantly lower in patients with ILD. Compared with HCs, DM/PM patients, with or without ILD, had decreased absolute counts of T, CD4 + T, CD8 + T, natural killer (NK), helper T (Th) 1, Th2, Th17, and regulatory T (Treg)cells. The fewest Th1 and Treg cells and the the lowest CD8 + T and Th1 cells percentages were seen in peripheral blood of patients with ILD. Longer duration, decreased lymphocyte/monocyte ratio (LMR)levels and CD8 + T and Th1 cells proportions, and fewer circulating Treg cells were independent risk factors for DM/PM with ILD. CONCLUSIONS: The identification of peripheral blood T lymphocyte subsets, especially Treg cells, and blood count in DM/PM appears to be useful in the comprehensive assessment of clinical lung involvement.

Polymyositis following varicella and mumps infection in adults: report of two cases.

Idiopathic immune myopathies (IIMs) are autoimmune diseases caused by immune-mediated muscle damage. The etiology remains unclear. Epidemiological and experimental studies, both in animals and humans, hint at viruses as major environmental factors able to trigger aberrant immune responses through many different mechanisms. However, only a few cases of either dermatomyositis or polymyositis following a specific viral infection have been reported in the literature. The objective of this study is to describe the clinical features and the treatment strategy of 2 cases of polymyositis developing shortly after chickenpox and mumps, respectively, and to review the existing literature on the topic. The clinical records of the 2 patients suspected to have developed inflammatory myositis following a viral infection were reviewed. Their clinical history, main laboratory findings, and treatment outcome are presented here. Moreover, a literature search was performed in the PubMed and MEDLINE databases to identify reports describing the association between viral infections and IIMs in patients aged ≥18. The 2 patients reported here developed polymyositis shortly after chickenpox and mumps, respectively, suggesting a causal role for viruses in triggering autoimmunity. Only a few reports published between 1990 and 2020 were found in the literature, possibly linking infections to myositis development. Intravenous immunoglobulin and rituximab were effective for the treatment of viral-triggered polymyositis.

Serum uric acid is independently associated with hypertension in patients with polymyositis and dermatomyositis.

Serum uric acid (UA), as an antioxidant, has been associated with hypertension in the general population. Hypertension is highly prevalent in patients with polymyositis and dermatomyositis (PM/DM). Owning elevated levels of reactive oxygen species, patients with PM/DM have lower concentrations of UA in comparison with healthy people. We explored a potential association between UA levels and hypertension in PM/DM and evaluated whether this association is independent of hypertension risk factors, PM/DM characteristics and relevant drugs. A total of 472 PM/DM patients were assessed. UA and related laboratory data were measured. Demographic, hypertension-related factors, PM/DM characteristics and drug use were assessed as potential covariates. Results were analyzed using logistic models to test the independence of the association between UA and hypertension. UA levels were higher in hypertension subjects compared to non-hypertensive PM/DM patients [284.70 (239.93-357.38) vs 264.00(222.50-322.75), p = .017]. When adjusted for hypertension risk factors, PM/DM characteristics and drugs, the odds of being a hypertensive PM/DM patient per 1 µmol/L UA increase were significantly increased: odds ratio = 1.473 (95% confidence interval:1.063-2.042, p = .020). This cross-sectional study suggests that UA levels are independently associated with hypertension in PM/DM patients.

Characteristics and risk of interstitial lung disease in dermatomyositis and polymyositis: a retrospective cohort study in Japan.

Dermatomyositis and polymyositis are rare, idiopathic inflammatory myopathies. Interstitial lung disease is one of the most common and potentially severe extra-muscular manifestations of dermatomyositis and polymyositis and is strongly linked to poor prognosis and early mortality. We aimed to characterise the demographic and clinical characteristics, incidence, and treatment of interstitial lung disease in patients with dermatomyositis or polymyositis. We conducted a retrospective cohort study using the Japan Medical Data Center healthcare claims database. Patients in the database with dermatomyositis (International Classification of Disease version 10 M33.0, M33.1, M33.9) or polymyositis (M33.2) from 01-Jan-2011 until 31-Dec-2019 were identified and followed-up for interstitial lung disease (J84.x) until death, dis-enrolment, or study end (31 December 2020). Cumulative risk curves compared interstitial lung disease risk in dermatomyositis versus polymyositis. Risk factors were evaluated by Cox proportional hazard models. There were 886 patients with dermatomyositis and 745 patients with polymyositis included in the cohort analysis. Mean (standard deviation) age at dermatomyositis/polymyositis diagnosis was 46.0 (16.0)/49.7 (13.3) years and 300 (34%)/104 (14%) developed interstitial lung disease during follow-up. The incidence rate of interstitial lung disease per 100 person-years was 18.42 (95% CI 16.42-20.59) for dermatomyositis and 5.39 (95% CI 4.43-6.50) for polymyositis. In the analysis adjusted for sex, age, and comorbidity score, the risk of interstitial lung disease was significantly higher in patients with dermatomyositis than with polymyositis (hazard ratio 2.72, 95% CI 2.18-3.41). The rate diverged markedly between the groups in the first year after diagnosis. Risk factors for interstitial lung disease were older age in dermatomyositis, female sex and rheumatoid arthritis in polymyositis. Glucocorticoids with/without tacrolimus were the most common newly prescribed drugs after the interstitial lung disease diagnosis. In conclusion, the risk of developing interstitial lung disease was significantly higher in patients with dermatomyositis than with polymyositis, and risk factors were different in the 2 patient groups.

The Prevalence of Dementia among Dermatomyositis and Polymyositis Patients: A Retrospective Cohort Study.

BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are inflammatory mediated myopathies characterized by progressive symmetric proximal muscle weakness and associated with extra-muscular involvement. Central nervous system complications are rarely reported with these diseases. OBJECTIVES: To investigate the association between dementia and PM/DM. METHODS: A retrospective cohort study was conducted using a database from Clalit Health Care, the largest health maintenance organization in Israel. Patients with a first recorded diagnosis of PM/DM were included and were compared with age- and sex-matched controls by a ratio of 1:5. The prevalence of dementia among PM/DM patients compared to controls was assessed using a univariate and a multivariable model. Binary logistic regression analysis was conducted to assess the association of different factors with dementia within the PM/DM cohort. RESULTS: The study included 2085 PM/DM cases (17.0%) and 10,193 age- and sex-matched controls (83.0%). During the follow-up time, 36 PM/DM patients were diagnosed with dementia compared to 160 controls, with a univariate hazard ratio (HR) of 1.10 (95% confidence interval [95%CI] 0.77-1.58). Within the PM/DM cohort, significant predictors for the development of dementia included increased age at diagnosis (5 years increment; OR 1.86, 95%CI 1.57-2.21, P < 0.001) and treatment with glucocorticoids (OR 5.40, 95%CI 1.67-17.67, P = 0.005). CONCLUSIONS: In our cohort, inflammatory myopathies were not associated with dementia. Age and treatment with glucocorticoids were associated with dementia. If dementia is diagnosed in patients with inflammatory myopathies, other systemic causes should be investigated.

[Risk factors analysis and prediction model establishment of malignant tumor in patients with polymyositis/dermatomyositis].

Objective: To analyze the risk factors of polymyositis/dermatomyositis (PM/DM) complicated with malignant tumor and to construct clinical prediction model. Methods: A total of 427 PM/DM patients, who were admitted to Rheumatism Immunity Branch, the Second Affiliated Hospital, Air Force Medical University from January 1, 2015 to January 1, 2021, were enrolled in the study, including 129 males and 298 females. The mean age was (51.4±12.2) years. The patients were divided into control group (without malignant tumor, n=379) and case group (with malignant tumor, n=48) according to whether they were complicated with malignant tumors. In the two groups, 70% of the patients' clinical data were randomly selected as the training set data, and the remaining 30% were used as the validation set data. The clinical parameters were retrospectively collected, and risk factors of PM/DM complicated with malignant tumor were analyzed by binary logistic regression. R software was used to construct a clinical prediction model for malignant tumors in PM/DM patients using training set data. Validation set data were used to assess the feasibility of the model. The area under the receiver operating characteristic (ROC) curve (AUC), calibration curve and decision curve analysis (DCA) were used to evaluate the predictive ability, accuracy and clinical applicability of the nomogram model. Results: The age of the control group was (50.4±11.8) years, and males accounted for 26.9%(102/379); the age of the case group was (59.1±12.7) years, and the proportion of males was 56.3% (27/48). The proportion of male, age, the positive rate of anti-transcription mediator 1-γ (TIF1-γ) antibody, glucocorticoid therapy resistance, and levels of creatine kinase (CK), carbohydrate antigen 125(CA125) and carbohydrate antigen 199 (CA199) were all higher in the case group than those in control group, while incidence of interstitial lung disease (ILD), arthralgia, Raynaud's phenomenon, serum albumin (ALB) level and lymphocyte (LYM) count were all lower than those in control group (all P<0.05). Binary logistic regression analysis showed that male (OR=2.931, 95%CI: 1.356-6.335), glucocorticoid therapy resistance (OR=5.261, 95%CI: 2.212-12.513), older age (OR=1.056, 95%CI: 1.022-1.091), elevated CA125 (OR=8.327, 95%CI: 2.448-28.319) and positive anti-TIF1-γ antibody (OR=7.529, 95%CI: 2.436-23.270) were risk factors of malignancy in PM/DM patients (all P<0.05); and complicated with ILD (OR=0.261, 95%CI: 0.099-0.689), complicated with arthralgia (OR=0.238,95%CI:0.073-0.779), elevated LYM count (OR=0.267, 95%CI: 0.103-0.691) were protective factors of malignancy in PM/DM patients (all P<0.05). The AUC of ROC curve predicting malignancy in PM/DM patients with the training concentrated prediction model was 0.887 (95%CI: 0.852-0.922), with a sensitivity of 77.9% and a specificity of 86.3%; it was 0.925 (95%CI: 0.890-0.960), 86.5% and 88.0% in the validated centralized prediction model, respectively. The correction curves of the training set and the validation set indicated that the predictive model had good calibration ability. Both the DCA curves of the training set and the validation set showed that the proposed predictive model had good clinical applicability. Conclusions: Older age, male, glucocorticoid therapy resistance, not complicated with ILD and arthralgia, elevated CA125, positive anti-TIF1-γ antibody, decreased LYM count are risk factors for malignancy in PM/DM patients, and the established nomogram model shows good predictive ability.

Polyarteritis nodosa initially presenting as concomitant peripheral neuropathy and myositis in unilateral limb: A case report.

RATIONALE: We report the case of a patient who initially presented with peripheral neuropathy and myositis without typical organ involvement, such as the kidneys, skin, or gastrointestinal system, but was ultimately diagnosed with polyarteritis nodosa (PAN). PATIENT CONCERNS: A 62-year-old man presented with radicular pain in his right lower extremity. One week later, he complained of right ankle motor weakness and pain in the right posterior thigh, which led to admission. After 6 weeks of hospitalization, he newly experienced pain in his right testicle and anterior thigh. DIAGNOSIS: The patient was initially diagnosed with polymyositis combined with sciatic neuropathy using magnetic resonance imaging, electrodiagnostic tests, and muscle biopsy. However, with the emergence of other systemic symptoms such as testicular pain, vasculitis was suspected, and the patient was reclassified as PAN using the 2007 European Medicines Agency algorithm and the American College of Rheumatology criteria. INTERVENTIONS: The patient was treated with glucocorticoids for more than 6 months, and antiviral medication was prescribed to prevent hepatitis B virus reactivation. OUTCOMES: The patient's radicular pain and pain in the right anterior and posterior thighs and testicle improved, and there were no signs of recurrence. LESSONS: In patients presenting with radicular and focal muscle pain, it is crucial to consider the potential for PAN, as observed in this case report.

Pulmonary hypertension, nephrotic syndrome, and polymyositis due to hepatitis C virus infection: A case report.

BACKGROUND: Hepatitis C infection not only damages the liver but also often accompanies many extrahepatic manifestations. Incidences of pulmonary hypertension (PH) caused by hepatitis C are rare, and incidences of concurrent nephrotic syndrome and polymyositis are even rarer. CASE SUMMARY: Herein we describe the case of a 57-year-old woman who was admitted to our department for intermittent chest tightness upon exertion for 5 years, aggravated with dyspnea for 10 d. After relevant examinations she was diagnosed with PH, nephrotic syndrome, and polymyositis due to chronic hepatitis C infection. A multi-disciplinary recommendation was that the patient should be treated with sildenafil and macitentan in combination and methylprednisolone. During treatment autoimmune symptoms, liver function, hepatitis C RNA levels, and cardiac parameters of right heart catheterization were monitored closely. The patient showed significant improvement in 6-min walking distance from 100 to 300 m at 3-mo follow-up and pulmonary artery pressure drops to 50 mmHg. Long-term follow-up is needed to confirm further efficacy and safety. CONCLUSION: Increasing evidence supports a relationship between hepatitis C infection and diverse extrahepatic manifestations, but it is very rare to have PH, nephrotic syndrome, and polymyositis in a single patient. We conducted a literature review on the management of several specific extrahepatic manifestations of hepatitis C.

The prevalence and effects of treatments of rapidly progressive interstitial lung disease of dermatomyositis/polymyositis adults: A systematic review and meta-analysis.

Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of numerical prevalence and therapy comparison in this field. Therefore, the purpose of this study was to determine the prevalence of RP-ILD in DM/PM patients and compare prognoses, including remission rate and survival data, between treatments. Studies with reports of RP-ILD in DM/PM patients and studies with definite remission and/or survival data of DM/PM-RP-ILD were included in the study. Data sources were Pubmed, Embase, and Cochrane Library without language restrictions. Two authors (WHL and WWQ) extracted independently the data. Estimates of the pooled effects were calculated using the Mantel-Haenszel technique (random effects). The prevalence meta-analysis included 18 papers with 6058 DM/PM patients, and 31 papers were analyzed for treatment effects, including remission rate, 6-month survival rate, 1-year survival rate, and 5-year survival rate. Database search yielded 1816 articles. In the DM/PM population, the combined prevalence of RP-ILD was 8.9% (95% CI, 5.8% to 12.1%). Patients with RP-ILD have a remission rate of 58.4% (95% CI, 47.3% to 69.4%), with biologic treatment with the highest remission rate, followed by triple therapy (defined as adding a third intravenous medication, including cyclophosphamide and immunoglobulin). Biologics therapy had the highest overall survival rate at six months (95% CI, 49.8% to 73.9%), followed by cDMARDs, plasma exchange, and triple therapy. The 1-year survival rate was 77.4% (95% CI, 66.7% to 88.1%), and triple therapy and cDMARDs had the best survival rates. The 5-year survival rate was 40.0% (95% CI, 10.0% to 69.9%). The prevalence of RP-ILD in DM/PM was approximately 8.9%, with a poor long-term prognosis. The use of biological agents appears to provide the best therapeutic outcomes, providing RP-ILD management with a novel evidence-based therapy. The use of strong immunosuppressive treatments may result in life-threatening side effects, thus clinicians must closely monitor the condition.

Pregnancy course and outcomes of patients with polymyositis and dermatomyositis (PM/DM) managed in a single center.

We aimed to determine the association between disease activity during pregnancy and pregnancy outcomes of women with polymyositis and dermatomyositis (PM/DM). Patients with PM/DM who were managed from pregnancy to delivery at Kagawa University Hospital from March 2006 to May 2021 were enrolled. Clinical data were retrospectively analyzed to evaluate the association between disease activity during pregnancy and pregnancy outcomes. Eight pregnancies in 5 women with PM/DM were analyzed. The mean age at conception was 28.3 ± 3.8 years, and mean disease duration was 6.3 ± 3.2 years. Four patients required an increased glucocorticoid dosage because of worsening disease activity (sustained elevation of creatine phosphokinase [CPK] concentration). Two patients who continuously received immunosuppressive drugs from conception to delivery showed no increase in disease activity and did not need increased glucocorticoid dosages. The pregnancy outcomes were 1 spontaneous abortion and 7 live births. The mean gestation length was 35.3 ± 5.2 weeks, and mean birthweight was 2297.7 ± 1041.4 g. Five adverse pregnancy outcomes (APOs) occurred (2 preterm births and 4 low birthweights); most of these cases had sustained elevation of CPK concentration and increased glucocorticoid dosages. No APOs occurred in the 2 patients who received continuous immunosuppressive medication. Continued use of pregnancy-compatible medications and control of disease activity with lower glucocorticoid dosages in pregnancies with PM/DM may be important to achieve good pregnancy outcomes.

Clinical Characteristics and Risk Factors of Polymyositis and Dermatomyositis Combined with Interstitial Lung Disease in Patients Residing in the Northeast Sichuan Province of China.

BACKGROUND: Polymyositis (PM) and dermatomyositis (DM) are non-suppurative and autoimmune inflammatory diseases of striated muscle. Interstitial lung disease (ILD) is a group of heterogeneous diseases that mainly involve the pulmonary interstitium, alveoli, and/or bronchioles, also known as diffuse parenchymal lung disease (DPLD). A significant cause of death in persons with polymyositis (PM) and dermatomyositis (DM) is concurrent interstitial lung disease (ILD). However, research on the clinical characteristics and associated influencing factors of PM/DM combined with ILD (PM/DM-ILD) is currently scarce in China. OBJECTIVE: The study aimed to probe the clinical features and risk factors of PM/DM-ILD. METHODS: The data of 130 patients with PM/DM were gathered. General medical status, clinical symptoms, laboratory parameters, high-resolution CT, therapeutic outcomes, and prognoses were retrospectively reviewed in patients with PM/DM with (ILD group) and without (NILD) ILD. RESULTS: The age of the ILD group (n=65) was more than the NILD group (n=65), and the difference was statistically significant; there were no significant between-group variations in the PM/DM ratio, sex, or duration of the disease. The initial symptoms were arthritis and respiratory symptoms in the ILD group, and myasthenia symptoms in the NILD group. Incidences of Raynaud's phenomenon, dry cough, expectoration, dyspnea on exertion, arthritis, fever, total globulin (GLOB), erythrocyte sedimentation rate (ESR), and anti-Jo-1 antibody rate were higher for ILD; however, albumin (ALB), creatine kinase aspartate aminotransferase activity ratio (CK/AST) and CK levels were significantly lower in the ILD group. Bivariate logistic regression analysis showed age, dry cough, arthritis, dyspnea on exertion, anti-Jo-1 antibody, and elevated GLOB to be independent risk factors for ILD among patients with PM/DM. CONCLUSION: Advanced age, dry cough, arthritis, dyspnea on exertion, anti-Jo-1 antibody positivity, and elevated GLOB level are risk factors for PM/DM-ILD. This information could be utilized to carefully monitor changing lung function in these patients.

Dermatomyositis associated with thymoma: A case report and literature review.

Abstract: Thymoma can present with paraneoplastic-autoimmune neuro-muscular disorders, including polymyositis, dermatomyositis, and granulomatous myositis. Rarely, concomitant subclinical myasthenia gravis (MG) can be a diagnostic dilemma and cause deleterious outcomes regarding missed or delayed diagnosis. We report a Turkish patient presented with thymoma associated dermatomyositis and positive acetylcholine receptor antibody without evident MG clinic.

Prognosis of spontaneous pneumomediastinum occurring in dermatomyositis or polymyositis patients with interstitial lung disease according to antimelanoma differentiation-associated gene 5 antibody status: a retrospective cohort study.

OBJECTIVES: Spontaneous pneumomediastinum (SPNM) historically has been considered a poor prognostic factor in dermatomyositis/polymyositis patients complicated with interstitial lung disease (ILD). However, there is a lack of actual data regarding the association between SPNM occurrence and mortality in dermatomyositis/polymyositis patients. This study aimed to assess the association between SPNM occurrence and mortality in myositis patients with ILD according to antimelanoma differentiation-associated gene 5 (MDA5) antibody status. METHODS: Dermatomyositis/polymyositis patients with ILD who were hospitalised at five Japanese hospitals from 2016 to 2020 were included in this retrospective observational study. We collected data about baseline characteristics including myositis-specific autoantibodies, treatments, SPNM and death within 1 year from therapy initiation or strengthening. Baseline characteristics and outcomes were compared between patients with and without SPNM (the SPNM group and the non-SPNM group, respectively). RESULTS: A total of 119 patients were analysed. SPNM occurred in 23 patients, and 15 patients died. Fifteen patients with SPNM were anti-MDA5 antibody positive. The mortality rate was significantly higher in the SPNM group (34.8%) than in the non-SPNM group (7.3%) (p=0.001). All deaths in the SPNM group occurred in anti-MDA5 antibody-positive patients (8/15), whereas none of the anti-MDA5 antibody-negative patients in the SPNM group died (0/8). In anti-MDA5 antibody-positive patients, the mortality rate was significantly higher in patients with SPNM occurrence (53.3%) than in those without SPNM occurrence (4.0%) (p=0.001). CONCLUSION: SPNM occurred more frequently in anti-MDA5 antibody-positive than in anti-MDA5 antibody-negative myositis patients. SPNM occurrence was associated with higher mortality risk, especially in anti-MDA5 antibody-positive patients.

Recurrent Exacerbations and Evolution into Polymyositis in a Patient with Interstitial Pneumonia with Autoimmune Features: A Case Report and Literature Review.

Interstitial pneumonia with autoimmune features (IPAF) is a new disease entity proposed in 2015. Numerous questions regarding IPAF require clarification, including diagnostic criteria, standard managements for stable disease and exacerbation, and prognosis. We report a case of a 67-year-old Asian woman who presented with progressive dyspnea. Chest computed tomography (CT) scans revealed nonspecific interstitial pneumonia. Serologic testing indicated positive anti-Jo-1 without presence of extrathoracic manifestations. An IPAF diagnosis was made after a multidisciplinary discussion. The patient experienced a severe exacerbation requiring mechanical ventilation, and she was successfully salvaged with methylprednisolone pulse therapy and single-dose cyclophosphamide. During the one-year follow-up, she reported bilateral leg muscle weakness with noticeably elevated serum creatine kinase, suggesting polymyositis. The development of malignancy was also noted 15 months after the initial presentation, and the patient eventually died. This report demonstrated successful salvage treatment with glucocorticoid pulse therapy for IPAF with acute exacerbation. However, the maintenance therapy failed to control disease progression. The treatment strategies for exacerbation and stable disease in IPAF remain unknown and need further studies. Given the high risk of evolution into a defined connective tissue disease (CTD), regular evaluation of the clinical features and biomarkers of CTDs is essential for patients with IPAF.

Pneumomediastinum, pneumatosis intestinalis and pneumoperitoneum in a patient with polymyositis: case-based review.

Pneumomediastinum (PnM), pneumatosis intestinalis (PI), and pneumoperitoneum (PP) are rare complications of inflammatory myositis. We present a 59-year-old polymyositis (PM) patient who experienced all three complications simultaneously. The patient who presented with proximal muscle weakness, dysphagia, and weight loss was diagnosed with PM due to elevated muscle enzymes and consistent electromyography and muscle biopsy with inflammatory myopathy. On the 45th day of her immunosuppressive treatment, PnM, PI, and PP were detected incidentally in 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) scan performed for severe weight loss and treatment-resistant severe disease. Since the patient had no symptoms or signs of PnM and PP, no additional intervention was applied to the current treatment, and spontaneous regression was observed in the follow-up. In addition to this case, we reviewed patients with PM who developed PBM, PP, and PI in the literature.