ABSTRACT
OBJECTIVES: This research aims to investigate the prevalence, epidemiological characteristics, mortality rates, survival rates and the rate of malignancy in patients diagnosed with inflammatory myopathies (IIM) in Oman. METHODS: This is a longitudinal study, that covered a span of 16 years at eight rheumatology centres in Oman. The study included all adults and paediatric patients diagnosed with different types of idiopathic inflammatory myopathies (IIM) and who fulfil either the Bohan classification criteria or the 2017 EULAR/ACR classification criteria. RESULTS: The study included a total of 116 patient with an average age of 38.78 (±17.61 SD) years. The most prevalent form of myositis was found to be dermatomyositis (DM) 48 (41.38%), followed by polymyositis (PM) 36 (31.03%) and juvenile myositis (JDM) 18(15.52%). However, inclusion body myositis and necrotising myopathy were relatively rare conditions. The prevalence rates for DM, PM and JDM were determined as 2.2, 2.2, and 1.14 per 100,000 population respectively. Cardiac complications were observed in 14.66% of cases. Among the individuals studied, a history of malignancy was present in around 1.72% of cases. ANA antibodies were present in 71.55% of the cases, anti-Jo 1 and anti-RNP/SM antibodies were detected in 8.62%, and Anti-Ro antibodies in 24.14%. The overall mortality rate was found to be 6.90% with a rate of 11.1% among JDM cases. The five-year survival rates for PM, DM and JDM were found to be 94.4%, 91.7% and 89.0% respectively. These rates decline over a 10-year period to 67%, 69% and 83.3% respectively. CONCLUSIONS: The study highlights the prevalence, mortality, and survival rates of IIM in Oman. Patients with JDM had a higher mortality rate. This underscores the significance of using novel healthcare strategies to improve clinical outcomes and meet special requirements for this group of patients.
Subject(s)
Myositis , Humans , Oman/epidemiology , Prevalence , Male , Female , Adult , Middle Aged , Myositis/mortality , Myositis/epidemiology , Myositis/diagnosis , Longitudinal Studies , Young Adult , Adolescent , Neoplasms/mortality , Neoplasms/epidemiology , Child , Aged , Survival Rate , Risk Factors , Time Factors , Prognosis , Polymyositis/epidemiology , Polymyositis/mortality , Polymyositis/diagnosis , Dermatomyositis/mortality , Dermatomyositis/epidemiology , Dermatomyositis/diagnosisABSTRACT
OBJECTIVES: Risk prediction for patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD) is challenging due to heterogeneity in the disease course. We aimed to develop a mortality risk prediction model for PM/DM-ILD. METHODS: This prognostic study analysed patients with PM/DM-ILD admitted to Nanjing Drum Hospital from 2016 to 2021. The primary outcome was mortality within 1 year. We used a least absolute shrinkage and selection operator (LASSO) logistic regression model to identify predictive laboratory indicators. These indicators were used to create a laboratory risk score, and we developed a mortality risk prediction model by incorporating clinical factors. The evaluation of model performance encompassed discrimination, calibration, clinical utility and practical application for risk prediction and prognosis. RESULTS: Overall, 418 patients with PM/DM-ILD were enrolled and randomly divided into development (n=282) and validation (n=136) cohorts. LASSO logistic regression identified four optimal features in the development cohort, forming a laboratory risk score: C reactive protein, lactate dehydrogenase, CD3+CD4+ T cell counts and PO2/FiO2. The final prediction model integrated age, arthralgia, anti-melanoma differentiation-associated gene 5 antibody status, high-resolution CT pattern and the laboratory risk score. The prediction model exhibited robust discrimination (area under the receiver operating characteristic: 0.869, 95% CI 0.811 to 0.910), excellent calibration and valuable clinical utility. Patients were categorised into three risk groups with distinct mortality rates. The internal validation, sensitivity analyses and comparative assessments against previous models further confirmed the robustness of the prediction model. CONCLUSIONS: We developed and validated an evidence-based mortality risk prediction model with simple, readily accessible clinical variables in patients with PM/DM-ILD, which may inform clinical decision-making.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Humans , Lung Diseases, Interstitial/mortality , Lung Diseases, Interstitial/etiology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/complications , Male , Female , Middle Aged , Dermatomyositis/complications , Dermatomyositis/mortality , Dermatomyositis/diagnosis , Risk Assessment , Prognosis , Aged , Adult , Risk Factors , Logistic Models , Polymyositis/complications , Polymyositis/mortality , Polymyositis/diagnosis , ROC CurveABSTRACT
OBJECTIVES: To investigate the prevalence and characteristics of typical polymyositis (PM) in Chinese patients with idiopathic inflammatory myopathy (IIM). METHODS: Patients diagnosed with IIM according to the 2017 EULAR/ACR criteria were included. Serological aspects including myositis-specific antibodies (MSA) and pathological data were re-evaluated. The diagnosis of typical PM was strictly done using the pathological criteria, while excluding other IIM subtypes such as dermatomyositis (DM), immune-mediated necrotising myopathies (IMNM), anti-synthetase syndrome (ASS), and sporadic inclusion body myositis (sIBM), based on their respective diagnostic criteria. RESULTS: A total of 544 IIM patients with muscle biopsy were involved, and 129 of them were diagnosed with initial PM according to the 2017 EULAR/ACR criteria. Only 6 (1.1%, 6/544) patients met the strict definition of typical PM after re-evaluation. Patients with typical PM were MSA-negative (100% vs. 35.7%, p=0.003) and had CD8+ T cells surrounding or invading non-necrotic muscle fibres in muscle biopsies (100% vs. 7.8%, p<0.001) compared to the initially diagnosed PM patients. All typical PM patients achieved clinical remission at the second-year follow-up. Typical PM patients had a favourable prognosis compared to MSA-negative IMNM and unspecific myositis patients. CONCLUSIONS: Strictly defined typical PM is a rare clinical subtype in Chinese IIM patients. Typical PM patients with classical pathology were MSA-negative and responded well to treatment and had a favourable prognosis. It is crucial for clinicians to combine clinical, serological, and pathological features to properly distinguish PM from other IIM subtypes.
Subject(s)
Autoimmune Diseases , Myositis, Inclusion Body , Myositis , Polymyositis , Humans , Myositis/diagnosis , Myositis/epidemiology , Myositis/therapy , Polymyositis/diagnosis , Polymyositis/epidemiology , Antibodies , China/epidemiology , AutoantibodiesABSTRACT
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.
Subject(s)
Deafness , Dermatomyositis , Gastropoda , Hearing Loss, Sudden , Polymyositis , Tinnitus , Humans , Animals , Dermatomyositis/complications , Dermatomyositis/epidemiology , Dermatomyositis/diagnosis , Hearing Loss, Sudden/complications , Hearing Loss, Sudden/epidemiology , Tinnitus/complications , Tinnitus/epidemiology , Prevalence , Polymyositis/complications , Polymyositis/epidemiology , Polymyositis/diagnosis , Deafness/complications , Deafness/epidemiology , Vertigo/complications , Vertigo/epidemiologyABSTRACT
OBJECTIVE: To compare cancer incidence, type, and survival between patients with idiopathic inflammatory myopathies (IIMs) in Western Australia (WA) and the general population. METHODS: Administrative health data for hospitalized patients with incident IIM (n = 803, 56.5% female, median age 62.0 yrs), classified by a validated algorithm as polymyositis (PM; 36.2%), dermatomyositis (DM; 27.4%), inclusion body myositis (IBM; 17.1%), overlap myositis (OM; 10.7%), and other IIM (8.6%), were linked to WA cancer and death registries for the period of 1980 to 2014. Cancer incidence rates (CIRs) before and after IIM diagnosis as well as cancer mortality were compared with age-, sex-, and calendar year-matched controls (n = 3225, 54.9% female, median age 64 yrs) by rate ratios (RRs) and Kaplan-Meier survival estimates. RESULTS: The prediagnosis CIR was similar for patients with IIM and controls (6.57 vs 5.95; RR 1.11, 95% CI 0.88-1.39) and for patients evolving to DM (n = 220) or other IIM subtypes (6.59 vs 6.56; RR 1.01, 95% CI 0.38-3.69). During follow-up, CIR was higher for all DM (4.05, 95% CI 3.04-5.29), with increased CIR for lung cancer vs controls (1.05 vs 0.33; RR 3.18, 95% CI 1.71-5.47). Cancer post diagnosis shortened life span by 59 months for patients with IIM (103 vs 162 months, P < 0.01), but reduced survival rates were observed only in patients with DM and IBM. CONCLUSION: Cancer risk was not increased prior to IIM, but CIR for lung cancer was increased following DM diagnosis. As cancer reduced survival only in patients with DM and IBM, these data support a strategy of limited cancer screening in IIM.
Subject(s)
Dermatomyositis , Lung Neoplasms , Myositis , Polymyositis , Humans , Female , Middle Aged , Male , Dermatomyositis/diagnosis , Dermatomyositis/epidemiology , Western Australia/epidemiology , Myositis/epidemiology , Myositis/diagnosis , Polymyositis/diagnosis , Polymyositis/epidemiologyABSTRACT
A diagnosis of polymyositis can readily be made when there is a typical history of proximal muscle weakness together with clinical findings, and there is corroboratory evidence in the form of elevated creatine kinase lactate dehydrogenase, aldolase, and serum glutamic-oxaloacetic transaminase (aspartate aminotransferase). A muscle biopsy usually helps in making the confirmatory diagnosis. A female in her 50s presented with non-healing multiple deep necrotic ulcers with muscle weakness. The initial possibility of vasculitis ulcers remained. Later, this proved to be a case of polymyositis with mildly elevated creatine kinase (which is usually not the case), atypical skin manifestations (usually there is no skin involvement), and negative extended myositis specific antibody panel with the growth of Burkholderia cepacia (perhaps the triggering factor). Hence, polymyositis can present with a myriad of atypical findings. Thus, thorough clinical examination and an integrated approach are necessary for early identification and treatment of the disease.
Subject(s)
Burkholderia , Polymyositis , Humans , Female , Ulcer , Polymyositis/diagnosis , Polymyositis/drug therapy , Muscle Weakness , Creatine KinaseABSTRACT
Late-onset Pompe disease manifests predominantly in the proximal lower limbs and may be mistaken for an inflammatory myopathy. A 46-year-old man with acromegaly had an 8-year history of progressive weakness. His myopathy was initially attributed to the acromegaly, but severe progression prompted a muscle biopsy, which suggested an inflammatory myopathy. However, his weakness progressed despite treatment for polymyositis. His muscle ultrasound scan pattern was more suggestive of Pompe disease than polymyositis, and Pompe disease was confirmed by genetic and enzymatic testing. Patients with apparent polymyositis, which persists despite treatment, require reconsideration of the diagnosis, with particular attention to treatable genetic causes.
Subject(s)
Acromegaly , Glycogen Storage Disease Type II , Myositis , Polymyositis , Male , Humans , Middle Aged , Glycogen Storage Disease Type II/diagnosis , Polymyositis/diagnosis , Polymyositis/pathology , Diagnostic ErrorsABSTRACT
YKL-40 is implicated in inflammation and tissue repair, but no reports have investigated its involvement in myositis in polymyositis (PM) and dermatomyositis (DM). Therefore, we aimed to investigate the relationship between YKL-40 and PM/DM. We retrospectively enrolled 35 patients diagnosed with PM/DM along with 26 healthy controls (HCs). Both PM and DM were diagnosed according to Bohan and Peter's criteria. Serum YKL-40 levels were measured, age-corrected to YKL-40 percentile values, and compared to HCs. Patients with myositis without interstitial lung disease were also enrolled and compared to HCs. Immunofluorescence staining was performed to identify YKL-40-positive inflammatory cells in muscle biopsy samples from two patients each with PM and DM. Age-corrected serum YKL-40 levels were significantly higher in patients with PM/DM compared to HCs with and without lung disease; however, these levels decreased significantly after treatment. Immunohistochemical analysis showed infiltration of YKL-40-positive inflammatory cells into the intramuscular sheath and perimuscular membrane. Immunofluorescence staining showed CD68 expression in YKL-40-positive inflammatory cells, suggesting that these cells were macrophages. To the best of our knowledge, this is the first study to demonstrate that YKL-40-positive macrophages are present in PM and DM, indicating that YKL-40 may be involved in PM/DM.
Subject(s)
Dermatomyositis , Myositis , Polymyositis , Humans , Retrospective Studies , Chitinase-3-Like Protein 1 , Polymyositis/diagnosis , Polymyositis/pathology , Myositis/etiology , MacrophagesABSTRACT
To determine long term overall and subgroup specific incidence rates and associated mortality for idiopathic inflammatory myopathies (IIM) in a population wide study. We included patients hospitalised between 1980 and 2015 with incident IIM as defined by relevant diagnostic codes for dermatomyositis (DM) polymyositis (PM), inclusion body myositis (IBM), other IIM and overlap myositis (OM) in the Western Australia Health Hospital Morbidity Data Collection (n = 846). Trends over time for annual incidence rate per million population (AIR) were analysed by least square regression and Kaplan-Meier survival and mortality rates (MR)/100 person years compared with a matched control group (n = 3681). The averaged AIR for all IIM was 19 (CI 10.4-27.5) and stable over time with point prevalence reaching 205.3 (CI 185.6-226.6) per million in 2015. Over time, the AIR for DM 5.0 (CI 0.6-9.4) and IBM 3.3 (CI 0.7-9.6) was stable, while AIR decreased for PM (p < 0.01) and increased for other IIM (p < 0.01) and OM (p < 0.01). IBM patients were eldest at diagnosis (68 years, CI 59-77) with male preponderance in IBM (53.4%) and other IIM (55.8%) groups. Crude mortality (54.5 vs 41.3%), MR ratio (6.65 vs 5.91) and 5 (65.8% vs 71.6%) and 10-year (52.5% vs 58.7%) survival were all worse for IIM patients (all p < 0.05). IBM patients had highest MR (10.1; CI 8.38-12.14) and lowest 10-year survival (39.2%). While cardiovascular disease and cancer were predominant causes of death, they were proportionally lower in IIM patients, where respiratory and rheumatic disease were more frequent causes of death. While the overall incidence of IIM in WA was stable over 35 years, the spectrum of IIM has changed significantly with increases especially in other IIM and OM. The overall prognosis with IIM remains guarded with 10-year survival just over 50%.
Subject(s)
Myositis, Inclusion Body , Myositis , Polymyositis , Humans , Male , Western Australia/epidemiology , Myositis/diagnosis , Polymyositis/epidemiology , Polymyositis/diagnosis , PrognosisABSTRACT
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.
Subject(s)
Dermatomyositis , Lung Diseases, Interstitial , Polymyositis , Humans , Female , Dermatomyositis/complications , Dermatomyositis/epidemiology , Dermatomyositis/diagnosis , Retrospective Studies , Japan/epidemiology , Polymyositis/complications , Polymyositis/epidemiology , Polymyositis/diagnosis , Lung Diseases, Interstitial/complications , Lung Diseases, Interstitial/epidemiology , Lung Diseases, Interstitial/diagnosis , Cohort Studies , PrognosisABSTRACT
Introducción: La polimiositis, la frase conocida como miopatía idiopática inflamatoria, es una enfermedad poco frecuente, considerada rara y heterogénea, que se caracteriza por la debilidad muscular, por lo que puede dificultar la movilidad cotidiana Objetivo: Analizar los tratamientos farmacológicos y no farmacológicos en pacientes diagnosticados con polimiositis. Métodos: Se realizó una búsqueda bibliográfica donde se siguió la recomendación PRISMA. Las fuentes de información consultadas fueron: SciELO, LILACS, PubMed, Elsevier, EBSCO, Medline, Google Académico, en el período de 2018 a 2022. Resultados: Se consultaron un total de 14 268 artículos correspondientes a la búsqueda bibliográfica, de ellos 42 artículos cumplieron con los criterios de selección. Se utilizó el método PRISMA según su recomendación, quedaron un total de cuatro artículos científicos originales de las cuales tres describen tratamientos farmacológicos, que mencionan a los corticoides y a los inmunosupresores; sin embargo, en aquellos pacientes que no responden al tratamiento se le recomienda la intervención clínica con inmunoglobulina G (IgG), que proporciona anticuerpos como moléculas monoméricas policlonales, que son bien tolerada. Por otro lado, dos artículos describen como tratamiento no farmacológico a la rehabilitación física con el objetivo de evitar el deterioro muscular. Conclusiones: El tratamiento en los pacientes diagnosticados con polimiositis debe ser individualizado, a partir de la gravedad de dicho padecimiento. A Una mayor afectación del cuerpo del paciente a nivel muscular, menor será la respuesta al tratamiento. Es importante la rehabilitación física y el uso de fármacos para controlar y aliviar la polimiositis(AU)
Introduction: Polymyositis known as idiopathic inflammatory myopathy is a rare disease. It is heterogeneous disease, characterized by symmetrical muscle weakness, which can make daily mobility difficult. Objective: To analyze pharmacological and non-pharmacological treatments in patients diagnosed with polymyositis. Methods: A bibliographic search was carried out following PRISMA recommendation. The information sources consulted were SciELO, LILACS, PubMed, Elsevier, EBSCO, Medline, Google Scholar from 2018 to 2022. Results: 14,268 articles corresponding to the bibliographic search were consulted, only 42 met the selection criteria. PRISMA method was used according to its recommendation. Four original scientific articles remained, three of them describe pharmacological treatments mentioning corticosteroids and immunosuppressants. However, in those patients who do not respond to treatment, clinical intervention with immunoglobulin G (IgG) is recommended, which provides antibodies as polyclonal monomeric molecules, which are well tolerated. On the other hand, two articles describe physical rehabilitation as a non-pharmacological treatment with the aim of avoiding muscle deterioration. Conclusions: Treatment in patients diagnosed with polymyositis should be individualized, based on the severity of the condition. A greater involvement of the patient's body at the muscular level, the lower the response to treatment. Physical rehabilitation and the use of drugs is important to control and relieve polymyositis(AU)
Subject(s)
Humans , Male , Female , Polymyositis/diagnosis , Polymyositis/drug therapy , Polymyositis/therapyABSTRACT
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.
Subject(s)
Dementia , Dermatomyositis , Polymyositis , Humans , Child, Preschool , Dermatomyositis/complications , Dermatomyositis/epidemiology , Dermatomyositis/diagnosis , Retrospective Studies , Glucocorticoids , Prevalence , Polymyositis/complications , Polymyositis/epidemiology , Polymyositis/diagnosis , Dementia/epidemiology , Dementia/etiologyABSTRACT
Polymyositis (PM) and dermatomyositis (DM) are the two subtypes of idiopathic inflammatory myositis and are characterized as symmetrical progressive muscle weakness in the proximal extremities. PM/DM affect multiple organs and systems, including the cardiovascular, respiratory and digestive tract systems. An in-depth understanding of PM/DM biomarkers will facilitate development of simple and accurate strategies for diagnosis, treatment, and prognosis prediction. This review summarized the classic biomarkers of PM/DM, including anti-aminoacyl tRNA synthetases (ARS) antibody, anti-Mi-2 antibody, anti-melanoma differentiation-associated gene 5 (MDA5) antibody, anti-transcription intermediary factor 1-γ (TIF1-γ) antibody, anti-nuclear matrix protein 2 (NXP2) antibody, among others. Among them, anti-aminoacyl tRNA synthetases antibody is the most classic. In addition, many potential novel biomarkers were also discussed in this review, including anti-HSC70 antibody, YKL-40, interferons, myxovirus resistance protein 2, regenerating islet-derived protein 3-α, interleukin (IL)-17, IL-35, microRNA (miR)-1 and so on. Among the biomarkers of PM/DM described in this review, classic biomarkers have become the mainstream biomarkers to assist clinicians in diagnosis due to their early discovery, in-depth research, and widespread application. The novel biomarkers also have potential and broad research prospects, which will make immeasurable contributions to exploring biomarker-based classification standards and expanding their application value.
Subject(s)
Dermatomyositis , Polymyositis , Humans , Dermatomyositis/diagnosis , Polymyositis/diagnosis , Biomarkers , Autoantibodies , Ligases , RNA, Transfer , Retrospective StudiesABSTRACT
Troponins are an excellent sensitive marker for myocardial ischaemic damage. However, there are several non-ischaemic cardiac and non-cardiac reasons for troponin elevation. Many cases of troponin T elevation and some troponin I cases have been reported in the literature due to inflammatory muscle disease. Here, we report a woman in her 50s who initially presents with fatigue and weakness, and is found to have elevated troponin T. The patient was appropriately worked up for cardiac causes with ECG and echocardiogram. She had positive antinuclear antibodies, antineutrophil cytoplasmic antibody and myositis panel. The elevation of troponins was attributed to polymyositis and treated with methotrexate and prednisone with recovery of patient's symptoms. This article emphasises the struggle of diagnosis in a patient with no reported medical history, having low to moderate risk of silent myocardial infarction.
Subject(s)
Myocardial Infarction , Polymyositis , Female , Humans , Troponin T , Biomarkers , Myocardial Infarction/diagnosis , Troponin I , Polymyositis/diagnosis , Polymyositis/drug therapyABSTRACT
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.
Subject(s)
Hepatitis C , Hypertension, Pulmonary , Nephrotic Syndrome , Polymyositis , Female , Humans , Middle Aged , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/etiology , Hepacivirus , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/drug therapy , Nephrotic Syndrome/etiology , Hepatitis C/complications , Hepatitis C/diagnosis , Hepatitis C/drug therapy , Polymyositis/complications , Polymyositis/diagnosis , Polymyositis/drug therapyABSTRACT
OBJECTIVE: To explore the frequency of circulating CD4+ T cells expressing PD-1+, TIM-3+ in polymyositis (PM) and dermatomyositis (DM) patients and its correlation with inflammatory factors, CD244+ and FOXP3+ T cell subtypes and prognosis. STUDY DESIGN: Observational study. Place and Duration of the Study: Ganzhou people's Hospital, Ganzhou, Jiangxi, China, from July 2019 to June 2021. METHODOLOGY: PM and DM patients were treated according to the institution's guidelines and followed up for 2 years. Fifty healthy volunteers were enrolled as controls. Serum interleukin (IL)-6, C-reactive protein (CRP), IL-17, and tumour necrosis factor α (TNF-α) levels were detected by enzyme-linked immunosorbent assay (ELISA). TIM-3+, PD-1+, CD244+, and FOXP3+ expressions were measured using flow cytometry. Inability to live normally, recurrence or death was defined as poor prognosis. RESULTS: The ESR, ALT, AST, LDH and ferritin concentration in PM/DM patients were remarkably elevated than that in healthy volunteers. The frequencies of PD-1+, TIM-3+, CD244+, and FOXP3+ were all remarkably enhanced in PM/DM patients compared with the healthy volunteers. The frequencies of PD-1+, TIM-3+, FOXP3+, and TIM-3+/PD-1+ T cells were significantly elevated in the poor prognosis group compared with the good prognosis group. The frequency of CD4+TIM-3+PD-1+ had satisfactory diagnostic value for PM/DM patients with bad prognoses. IL-17, TIM-3+, PD-1+and TIM-3+ PD-1+ were the risk factors for PM/DM patients with bad outcomes. CONCLUSION: The frequency of circulating CD4+ T cells expressing TIM-3+PD-1+ could be used to predict the prognosis of PM/DM patients. KEY WORDS: Tim-3, PD-1, Dermatomyositis, Polymyositis, Inflammatory.
Subject(s)
Dermatomyositis , Polymyositis , Humans , Dermatomyositis/diagnosis , Dermatomyositis/metabolism , Interleukin-17 , Programmed Cell Death 1 Receptor , T-Lymphocytes/metabolism , Hepatitis A Virus Cellular Receptor 2 , Polymyositis/diagnosis , Polymyositis/metabolism , Interleukin-6 , Prognosis , Forkhead Transcription Factors , Signaling Lymphocytic Activation Molecule FamilyABSTRACT
Idiopathic inflammatory myopathy (IIM) are heterogeneous autoimmune diseases that primarily affect the proximal muscles. IIM subtypes include dermatomyositis (DM), polymyositis (PM), and anti-synthetase syndrome (ASS). Metabolic disturbances may cause irreversible structural damage to muscle fibers in patients with IIM. However, the metabolite profile of patients with different IIM subtypes remains elusive. To investigate metabolic alterations and identify patients with different IIM subtypes, we comprehensively profiled plasma metabolomics of 46 DM, 13 PM, 12 ASS patients, and 30 healthy controls (HCs) using UHPLC-Q Exactive HF mass spectrometer. Multiple statistical analyses and random forest were used to discover differential metabolites and potential biomarkers. We found that tryptophan metabolism, phenylalanine and tyrosine metabolism, fatty acid biosynthesis, beta-oxidation of very long chain fatty acids, alpha-linolenic acid and linoleic acid metabolism, steroidogenesis, bile acid biosynthesis, purine metabolism, and caffeine metabolism are all enriched in the DM, PM, and ASS groups. We also found that different subtypes of IIM have their unique metabolic pathways. We constructed three models (five metabolites) to identify DM, PM, ASS from HC in the discovery and validation sets. Five to seven metabolites can distinguish DM from PM, DM from ASS, and PM from ASS. A panel of seven metabolites can identify anti-melanoma differentiation-associated gene 5 positive (MDA5 +) DM with high accuracy in the discovery and validation sets. Our results provide potential biomarkers for diagnosing different subtypes of IIM and a better understanding of the underlying mechanisms of IIM.
Subject(s)
Autoimmune Diseases , Dermatomyositis , Myositis , Polymyositis , Humans , Myositis/diagnosis , Polymyositis/diagnosis , BiomarkersABSTRACT
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.