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Contrast Media & Molecular Imaging 2022This paper aims to investigate the clinical and laboratory test characteristics of patients with anti-MDA5 antibody-positive PM/DM by analyzing the clinical...
This paper aims to investigate the clinical and laboratory test characteristics of patients with anti-MDA5 antibody-positive PM/DM by analyzing the clinical characteristics, laboratory test results, and 1-year survival rate of patients with anti-MDA5 antibody-positive PM/DM in polymyositis (PM) and dermatomyositis (DM). To further investigate the impact of positive anti-MDA5 antibodies on the prognosis of PM/DM patients. According to the anti-MDA5 antibody test results, 18 cases with positive anti-MDA5 antibodies were in the positive group and 46 cases with negative anti-MDA5 antibodies were in the negative group. The clinical manifestations, laboratory tests, treatment protocols, and prognostic risk factors were collected for both groups. The chi-square test, Mann-Whitney method, Fisher test, -test, Kaplan-Meier method, and Log-rank test were used for statistical analysis. Anti-MDA5 antibody positivity was more common in patients with DM/CADM. With no statistically significant differences in age and sex ratio between the two groups, The differences in erythrocyte sedimentation rate (ESR), ferritin (Fer), and creatine kinase (CK) levels in the positive group were statistically significant compared with the negative group. Clinically, the positive group was more prone to arthralgia, skin rash, and interstitial pneumonia.
Topics: Autoantibodies; Dermatomyositis; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Polymyositis
PubMed: 35992549
DOI: 10.1155/2022/7102480 -
Seminars in Arthritis and Rheumatism Feb 2020Juvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on...
OBJECTIVE
Juvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on long-term outcomes is limited. In this study we investigate correlations between clinical and demographic features with long-term outcomes in a referral population of adult patients with JIIM.
METHODS
Forty-nine adults with JIIM were assessed at two referral centers between 1994 and 2016. Features of active disease and damage at a cross-sectional assessment were obtained. Regression modeling was used to examine factors associated with long-term outcomes, defined by the presence of calcinosis or a higher adjusted Myositis Damage Index (MDI) score. A multivariable model of MDI was constructed using factors that were statistically significant in bivariate models.
RESULTS
At a median of 11.5 [IQR 4.5-18.9] years following diagnosis, median American College of Rheumatology (ACR) functional class was 2 [1.5-3.0], Health Assessment Questionnaire (HAQ) score was 0.4 out of 3.0 [0.0-1.0], and manual muscle testing (MMT) score was 229 out of 260 [212.6-256.8]. Median MDI score was 6.0 [3.5-8.9], with the most commonly damaged organ systems being cutaneous and musculoskeletal. Factors associated with an elevated MDI score were the presence of erythroderma and other cutaneous manifestations, disease duration, and ACR functional class. Calcinosis was present in 55% of patients. The strongest predictors of calcinosis were disease duration, periungual capillary changes, and younger age at diagnosis.
CONCLUSION
In a tertiary referral population, long-term functional outcomes of JIIM are generally favorable, with HAQ scores indicative of mild disability. Although most patients had mild disease activity and virtually all had significant disease damage, severe or systemic damage was rare. Certain clinical features are associated with long-term damage and calcinosis.
Topics: Adolescent; Adult; Calcinosis; Dermatomyositis; Female; Humans; Male; Middle Aged; Severity of Illness Index; Skin; Treatment Outcome; Young Adult
PubMed: 31303436
DOI: 10.1016/j.semarthrit.2019.06.014 -
BMC Infectious Diseases Mar 2022Epstein-Barr virus (EBV) infects more than 90% of the population worldwide. However, chronic active EBV infection (CAEBV) is one of the EBV-positive T- or...
BACKGROUND
Epstein-Barr virus (EBV) infects more than 90% of the population worldwide. However, chronic active EBV infection (CAEBV) is one of the EBV-positive T- or NK-lymphoproliferative diseases with high morbidity and mortality. Here, we report a case of a 9-year girl with CAEBV, successively presenting with polymyositis and coronary artery dilation (CAD).
CASE PRESENTATION
The girl complained of fatigue for more than 1 month. Muscle strength examinations had no abnormal findings. Blood chemistries showed elevated alanine aminotransferase (ALT), aspartate aminotransferase (AST), and creatine kinase (CK). Magnetic resonance imaging (MRI) showed spotty high-intensity signals in thigh muscles, and electromyogram suggested myogenic damage. The significant findings were positive EBV antibodies (EBVEA-IgG, EBVCA-IgG, and EBVNA-IgG), increased EBV DNA copies in B, T, and NK cells, and positive EBV-encoded small RNA in biopsy muscle specimen. The girl received ganciclovir, intravenous immunoglobulin, and methylprednisolone, and her symptoms improved. On the 45th day of hospitalization, echocardiograph revealed CAD. She received additional anticoagulants and Tocilizumab. Her condition improved and continued to be followed up at the clinic preparing for hematopoietic stem cell transplantation.
CONCLUSIONS
This is the first reported case of CAEBV successively with polymyositis and CAD. This case makes the diagnoses of autoimmune diseases in children more complicated. Careful investigation of hidden CAEBV should be recommended in children with atypical polymyositis or CAD.
Topics: Chronic Disease; Coronary Vessels; Dilatation; Epstein-Barr Virus Infections; Female; Herpesvirus 4, Human; Humans; Polymyositis
PubMed: 35255852
DOI: 10.1186/s12879-022-07221-9 -
Respiration; International Review of... 2015Lung involvement in rheumatologic diseases has a broad spectrum of clinical and radiological presentations, from acute and inflammatory predominance to chronic and... (Review)
Review
Lung involvement in rheumatologic diseases has a broad spectrum of clinical and radiological presentations, from acute and inflammatory predominance to chronic and fibrotic lung disorders. Therapy has to be guided by the type of rheumatologic disease, the kind of lung involvement and the risk associated with the clinical state of the patient and prognostic factors. Although several therapeutic approaches have been used, the best treatment is still not certain; clinical trials are mandatory, which would require a better knowledge of the pulmonary pathogenesis of immunologic diseases. The following review is focused on the therapeutic options for those rheumatologic disorders that could present as a predominant interstitial lung disease which may confer a bad or life-threatening prognosis.
Topics: Adrenal Cortex Hormones; Antirheumatic Agents; Arthritis, Rheumatoid; Azathioprine; Cyclophosphamide; Humans; Immunosuppressive Agents; Lung Diseases; Lupus Erythematosus, Systemic; Mycophenolic Acid; Polymyositis; Rituximab; Scleroderma, Systemic; Sjogren's Syndrome
PubMed: 26461464
DOI: 10.1159/000441238 -
International Journal of Molecular... May 2017Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the... (Review)
Review
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM). The diagnosis of IIMs, including manual muscle testing, laboratory studies, and non-invasive imaging have become important in classifying IIM subtypes and for identifying disease severity. Treatment has evolved from an era where glucocorticoid therapy was the only option to a time now that includes traditional steroid-sparing agents along with immunoglobulin therapy and biologics, such as rituximab.
Topics: Animals; Autoantibodies; Autoimmune Diseases; Dermatomyositis; Humans; Myositis; Polymyositis
PubMed: 28524083
DOI: 10.3390/ijms18051084 -
Indian Journal of Pathology &... May 2022Idiopathic inflammatory myopathy (IIM) is a broad term that includes dermatomyositis, polymyositis, overlap myositis, sporadic inclusion body myositis, and... (Review)
Review
Idiopathic inflammatory myopathy (IIM) is a broad term that includes dermatomyositis, polymyositis, overlap myositis, sporadic inclusion body myositis, and immune-mediated necrotizing myopathy. The understanding of the pathogenesis of IIM is ever-evolving with regular updates in the classification schema. With the recognition of autoantibodies and their detection, the diagnostic algorithms are changing in favor of non-invasive diagnoses. However, muscle biopsy has immensely contributed to our understanding of the pathogenesis of inflammatory myopathies, and the pathologic features of different subtypes are well established. The biopsy also aids in distinguishing myopathies with overlapping clinical features, particularly dystrophies, which can show inflammation on biopsy in some cases. In this article, the various classification schemes of the IIM are reviewed. Also, the pathogenesis and pathology of each type of IIM have been highlighted. This article emphasizes the role of muscle biopsy in the diagnosis of inflammatory myopathies.
Topics: Autoantibodies; Biopsy; Dermatomyositis; Humans; Muscles; Myositis; Polymyositis
PubMed: 35562156
DOI: 10.4103/ijpm.ijpm_1033_21 -
Clinical and Experimental Rheumatology Feb 2022To identify the risk factors in Chinese patients with adult polymyositis and dermatomyositis for their comorbidities and explore a subclassification system.
OBJECTIVES
To identify the risk factors in Chinese patients with adult polymyositis and dermatomyositis for their comorbidities and explore a subclassification system.
METHODS
Clinical records of 397 patients with idiopathic inflammatory myopathies were retrospectively reviewed. Logistic regression was used to identify potential risk factors for interstitial lung disease (ILD), other rheumatic diseases, and malignancy after bivariate analysis. Hierarchical clustering and decisional tree were utilised to identify subgroups and explore a subclassification system.
RESULTS
A total of 119 polymyositis and 191 dermatomyositis patients were included. Anti-PM/Scl, anti-Ro52, anti-aminoacyl-tRNA synthetase and anti-MDA5 (adjusted odds ratios (AOR)=4.779, 1.917, 5.092 and 7.714 respectively) antibodies were risks (p<0.05), whereas overlapping malignancy was protective (AOR=0.107; p=0.002) for ILD across polymyositis, dermatomyositis and the total group. In subgroup models, Raynaud's phenomenon, arthralgia and semi-quantitative anti-nuclear antibody (AOR=51.233, 4.261, 3.047 respectively) were risks for other overlapping rheumatic diseases (p<0.05). For overlapping malignancy, male and anti-TIF1γ antibodies (AOR=2.533, 16.949) were risks (p<0.05), whereas disease duration and combination of ILD (AOR=0.954, 0.106) were protective in the total group (p<0.05); while anti-NXP2 antibodies were identified as risk factors (AOR=73.152; p=0.038) in polymyositis. Hierarchical clustering suggested a subclassification with 6 subgroups: malignancy overlapping dermatomyositis, classical dermatomyositis, polymyositis with severe muscle involvement, dermatomyositis with ILD, polymyositis with ILD, and overlapping of myositis with other rheumatic diseases.
CONCLUSIONS
Accompanying ILD, other rheumatic diseases and malignancy are strongly associated with clinical manifestation and myositis-specific or myositis-associated autoantibodies among Chinese polymyositis and dermatomyositis patients. The subclassification system proposed a more precise phenotype defining toward stratified treatments.
Topics: Autoantibodies; China; Dermatomyositis; Humans; Machine Learning; Male; Polymyositis; Retrospective Studies
PubMed: 34251311
DOI: 10.55563/clinexprheumatol/i2xeao -
Journal of Managed Care & Specialty... Nov 2020Flare activity or worsening symptoms are not well defined for myositis. To (a) characterize dermatomyositis (DM) and polymyositis (PM) flares from the patient...
Flare activity or worsening symptoms are not well defined for myositis. To (a) characterize dermatomyositis (DM) and polymyositis (PM) flares from the patient perspective and (b) report the corresponding disability and rate of unplanned medical encounters. Online survey data were collected from volunteer patients from The Myositis Association and Johns Hopkins Myositis Center. Flare frequency; Health Assessment Questionnaire Disability Index (HAQ-DI), HAQ-Pain Index, Work Productivity and Activity Impairment (WPAI) scales; emergency department/urgent care (ED/UC) visits; and hospital admissions during the past year were examined. 564 individuals with selfreported diagnoses of DM/PM were surveyed between December 2017 and May 2018. Recall of symptom flares was reported by 524 respondents (78.1% were female, mean age of 55 years). Among the respondents, 378 (72.1%) reported ≥ 1 flare in the past year. The pattern of flare frequency was similar for DM and PM respondents. The most common symptoms were muscle weakness (83%), extreme fatigue (78%), and muscle pain/discomfort (64%). Increasing flare frequency was associated with significantly ( < 0.01) greater mean HAQ-DI and HAQ-Pain scores, myositis-related ED/UC visits, hospital admissions, WPAI work productivity loss (among those employed), and WPAI nonwork activity impairment. DM/PM-related flares are common with exacerbations of muscle weakness and fatigue being the most common flare symptoms. Flare frequency was associated with greater disability, pain, work productivity loss, nonwork activity impairment, and increased ED/UC utilization. Higher frequency of patient-reported flares may serve as a marker of worsening physical functioning and intensifying health care needs and, therefore, suggests their importance in the clinical assessment of patients with DM/PM. This study was supported by Mallinckrodt Pharmaceuticals (Bedminster, NJ) via grants to Vedanta Research and The Myositis Association. Christopher-Stine has received compensation from previous Mallinckrodt Advisory Board meetings, unrelated to this subject matter. Wan is an employee of Mallinckrodt Pharmaceuticals and is a stockholder of the company. Reed and Bostic received grant support from Mallinckrodt Pharmaceuticals for data collection and analysis. McGowan is an employee of The Myositis Foundation, which received grant funding to support study data collection. Kelly has no conflicts to disclose. This study was presented, in part or full, at the 2019 Annual American College of Rheumatology and Association of Rheumatology Professional Meeting (November 8-13, 2018; Atlanta, GA) and at the Third Global Conference on Myositis (March 27, 2019; Berlin, Germany).
Topics: Absenteeism; Adult; Aged; Cost of Illness; Dermatomyositis; Disability Evaluation; Efficiency; Female; Health Resources; Humans; Male; Middle Aged; Pain Measurement; Polymyositis; Self Report; Sick Leave; Symptom Flare Up
PubMed: 33119444
DOI: 10.18553/jmcp.2020.26.11.1424 -
Internal Medicine (Tokyo, Japan) 2023Generalized subcutaneous edema is rare in dermatomyositis. We herein report an 82-year-old woman with dermatomyositis who presented with generalized subcutaneous edema....
Generalized subcutaneous edema is rare in dermatomyositis. We herein report an 82-year-old woman with dermatomyositis who presented with generalized subcutaneous edema. Three weeks before admission, she first noticed facial edema, and the symptoms exacerbated, with limb edema occurring. On admission, muscle weakness in the limbs and skin rash were noted. Her muscle enzyme levels were elevated, and serum anti-transcriptional intermediary factor 1-γ antibody was detected. She was diagnosed with dermatomyositis and treated with corticosteroids and tacrolimus, which improved her muscle weakness, muscle enzymes, and edema. Thus, generalized subcutaneous edema can occur during dermatomyositis, with facial edema as the initial symptom.
Topics: Female; Humans; Aged, 80 and over; Dermatomyositis; Edema; Muscle Weakness; Exanthema; Angioedema
PubMed: 37532515
DOI: 10.2169/internalmedicine.0687-22 -
Medicine Apr 2023We aimed to determine the association between disease activity during pregnancy and pregnancy outcomes of women with polymyositis and dermatomyositis (PM/DM). Patients...
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.
Topics: Pregnancy; Infant, Newborn; Humans; Female; Dermatomyositis; Polymyositis; Glucocorticoids; Retrospective Studies; Pregnancy Outcome
PubMed: 37026900
DOI: 10.1097/MD.0000000000033462