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Reumatologia Clinica Apr 2024The use of specific antibodies in inflammatory myopathies has improved the characterization of this disease, identifying different clinical phenotypes. Patients with...
The use of specific antibodies in inflammatory myopathies has improved the characterization of this disease, identifying different clinical phenotypes. Patients with dermatomyositis (DM) and anti-MDA5 antibodies display typical skin symptoms, lesser muscular involvement, and a prevalence of interstitial lung disease (ILD) of up to 91%. Beyond ILD, spontaneous pneumomediastinum (SN) has been identified as a rare but potentially fatal pulmonary manifestation. Two cases of this complication in patients with anti-MDA5 DM are reported.
Topics: Female; Humans; Male; Middle Aged; Autoantibodies; Dermatomyositis; Interferon-Induced Helicase, IFIH1; Mediastinal Emphysema
PubMed: 38614887
DOI: 10.1016/j.reumae.2024.04.002 -
Frontiers in Medicine 2024Idiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles,...
Myositis-associated antibodies predict the severity of lung involvement in adult patients with inflammatory myositis - a cohort study of 70 adult patients with myositis in a single center.
INTRODUCTION
Idiopathic inflammatory myopathies (IIMs) encompass a diverse group of diseases characterized by considerable variability in clinical manifestations, antibody profiles, and responsiveness to immunosuppressive therapies. This study aimed to investigate the association between organ involvement and distinct myositis autoantibodies in individuals with IIM in a single-center cohort.
METHODS
Patients with ICD diagnoses M33.1, M33.2, M33.9, or M609 who (1) had been tested with Euroline blot assay for myositis autoantibodies and (2) met the classification criteria of definite/probable polymyositis (PM) or dermatomyositis (DM), anti-synthetase syndrome (ASS), or inclusion body myositis (IBM) were included. Medical journals were retrospectively examined with respect to clinical disease features.
RESULTS
Seventy patients (median age 58 years; 66% females) were included and represented the following diagnosis: PM ( = 23), DM ( = 21), ASS ( = 23), and IBM ( = 3). Most of the patients (87%) presented a muscle biopsy indicative of myositis. The presence of autoantibodies was as follows: myositis-specific antibodies, MSA ( = 53), myositis-associated antibodies, MAA ( = 33), both MSA + MAA ( = 24), MSA only ( = 29), MAA only ( = 9), no MSA, or MAA ( = 8). Anti-Jo-1 was the most common MSA (19%), whereas the most common MAA was anti-Ro/SSA52 (31%). We observed a significant association between antibody patterns and lung disease. In our cohort, 47% of the patients in the whole study group, 86% of patients with anti-SSA52, and 100% with anti-Jo-1 had pulmonary involvement. Patients with both MSA and MAA had a higher incidence of lung disease and decreased CO-diffusion capacity. This was especially prominent in anti-Ro/SSA52-positive patients. Interestingly, none of the patients suffered from lung disease if only antibodies against Mi-2α, Mi-2β, NXP2, HMGCR, and TIF1γ were present or no MSA/MAA were detected.
DISCUSSION
The simultaneous presence of both MAA and MSA indicates an increased risk of lung involvement in patients with inflammatory myopathies. The presence of any MAA, and especially anti-Ro/SSA52, is associated with more severe pulmonary disease. Our data suggest that MAA antibodies might be relevant markers for early detection and treatment of lung involvement in IIM.
PubMed: 38606156
DOI: 10.3389/fmed.2024.1340310 -
JAMA Dermatology May 2024
Topics: Humans; Dermatomyositis; Lung Diseases, Interstitial; Interferon-Induced Helicase, IFIH1; Autoantibodies; Female; Middle Aged; Male; Adult; Aged; Age of Onset
PubMed: 38598206
DOI: 10.1001/jamadermatol.2024.0507 -
Journal of Clinical Medicine Feb 2024Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune disorders often complicated by interstitial lung disease (ILD). The clinical characteristics and...
BACKGROUND
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune disorders often complicated by interstitial lung disease (ILD). The clinical characteristics and outcomes of IIM-associated ILD have been reported variably, but the literature on rural populations is scarce.
METHODS
A retrospective cross-sectional study was conducted at a rural tertiary academic medical center. Twenty-nine patients met the final inclusion criteria. The primary outcome was to assess the disease state and immunological and radiographic features of IIM-associated ILD. Secondary outcomes included disease progression, ILD exacerbation, mortality rate, and factors associated with poor outcome.
RESULTS
Dermatomyositis (n = 15, 51.72%) followed by polymyositis (n = 8, 27.58%) were predominant myopathies. The most common autoantibodies were anti-Jo1 antibodies (n = 11, 37.93%). Indeterminate usual interstitial pneumonitis (41.30%, n = 12) was the most common radiographic pattern followed by non-specific interstitial pneumonia (n = 5, 17.24%). ILD exacerbation (n = 14, 66.66%) and mortality rate (n = 6, 20.69%) were high. Albumin levels were significantly lower in patients who died.
CONCLUSIONS
The clinical characteristics of patients with IIM-associated ILD in rural Appalachia exhibit notable distinctions, and outcomes are worse compared to other populations. Larger studies are needed to investigate other prognostics factors and longitudinal trends of clinical characteristics and outcomes of IIM-associated ILD in rural populations.
PubMed: 38592101
DOI: 10.3390/jcm13051294 -
Acta Radiologica (Stockholm, Sweden :... Jun 2024The precise and objective assessment of thigh muscle edema is pivotal in diagnosing and monitoring the treatment of dermatomyositis (DM) and polymyositis (PM).
BACKGROUND
The precise and objective assessment of thigh muscle edema is pivotal in diagnosing and monitoring the treatment of dermatomyositis (DM) and polymyositis (PM).
PURPOSE
Radiomic features are extracted from fat-suppressed (FS) T2-weighted (T2W) magnetic resonance imaging (MRI) of thigh muscles to enable automatic grading of muscle edema in cases of polymyositis and dermatomyositis.
MATERIAL AND METHODS
A total of 241 MR images were analyzed and classified into five levels using the Stramare criteria. The correlation between muscle edema grading and T2-mapping values was assessed using Spearman's correlation. The dataset was divided into a 7:3 ratio of training (168 samples) and testing (73 samples). Thigh muscle boundaries in FS T2W images were manually delineated with 3D-Slicer. Radiomics features were extracted using Python 3.7, applying Z-score normalization, Pearson correlation analysis, and recursive feature elimination for reduction. A Naive Bayes classifier was trained, and diagnostic performance was evaluated using receiver operating characteristic (ROC) curves and comparing sensitivity and specificity with senior doctors.
RESULTS
A total of 1198 radiomics parameters were extracted and reduced to 18 features for Naive Bayes modeling. In the testing set, the model achieved an area under the ROC curve of 0.97, sensitivity of 0.85, specificity of 0.98, and accuracy of 0.91. The Naive Bayes classifier demonstrated grading performance comparable to senior doctors. A significant correlation (r = 0.82, <0.05) was observed between Stramare edema grading and T2-mapping values.
CONCLUSION
The Naive Bayes model, utilizing radiomics features extracted from thigh FS T2W images, accurately assesses the severity of muscle edema in cases of PM/DM.
Topics: Humans; Magnetic Resonance Imaging; Edema; Dermatomyositis; Male; Female; Polymyositis; Middle Aged; Adult; Thigh; Muscle, Skeletal; Sensitivity and Specificity; Aged; Retrospective Studies; Image Interpretation, Computer-Assisted; Radiomics
PubMed: 38591947
DOI: 10.1177/02841851241244507 -
BMC Pulmonary Medicine Apr 2024Autoimmune pulmonary alveolar proteinosis (APAP) is a diffuse lung disease that causes abnormal accumulation of lipoproteins in the alveoli; however, its pathogenesis...
A case of autoimmune pulmonary alveolar proteinosis during the course of treatment of rapidly progressive interstitial pneumonia associated with anti-MDA5 antibody-positive dermatomyositis.
BACKGROUND
Autoimmune pulmonary alveolar proteinosis (APAP) is a diffuse lung disease that causes abnormal accumulation of lipoproteins in the alveoli; however, its pathogenesis remains unclear. Recently, APAP cases have been reported during the course of dermatomyositis. The combination of these two diseases may be coincidental; however, it may have been overlooked because differentiating APAP from a flare-up of interstitial pneumonia associated with dermatomyositis is challenging. This didactic case demonstrates the need for early APAP scrutiny.
CASE PRESENTATION
A 50-year-old woman was diagnosed with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatitis and interstitial pneumonia in April 2021. The patient was treated with corticosteroids, tacrolimus, and cyclophosphamide pulse therapy for interstitial pneumonia complicated by MDA5 antibody-positive dermatitis, which improved the symptoms and interstitial pneumonia. Eight months after the start of treatment, a new interstitial shadow appeared that worsened. Therefore, three additional courses of cyclophosphamide pulse therapy were administered; however, the respiratory symptoms and interstitial shadows did not improve. Respiratory failure progressed, and 14 months after treatment initiation, bronchoscopy revealed turbid alveolar lavage fluid, numerous foamy macrophages, and numerous periodic acid-Schiff-positive unstructured materials. Blood test results revealed high anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody levels, leading to a diagnosis of APAP. The patient underwent whole-lung lavage, and the respiratory disturbance promptly improved. Anti-GM-CSF antibodies were measured from the cryopreserved serum samples collected at the time of diagnosis of anti-MDA5 antibody-positive dermatitis, and 10 months later, both values were significantly higher than normal.
CONCLUSIONS
This is the first report of anti-MDA5 antibody-positive dermatomyositis complicated by interstitial pneumonia with APAP, which may develop during immunosuppressive therapy and be misdiagnosed as a re-exacerbation of interstitial pneumonia. In anti-MDA5 antibody-positive dermatomyositis, APAP comorbidity may have been overlooked, and early evaluation with bronchoalveolar lavage fluid and anti-GM-CSF antibody measurements should be considered, keeping the development of APAP in mind.
Topics: Female; Humans; Middle Aged; Pulmonary Alveolar Proteinosis; Dermatomyositis; Autoantibodies; Lung Diseases, Interstitial; Cyclophosphamide; Dermatitis; Interferon-Induced Helicase, IFIH1; Autoimmune Diseases
PubMed: 38589870
DOI: 10.1186/s12890-024-02989-9 -
Proceedings of the National Academy of... Apr 2024Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) is characterized by amyopathic DM with interstitial lung disease (ILD)....
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) is characterized by amyopathic DM with interstitial lung disease (ILD). Patients with anti-MDA5 antibody-associated ILD frequently develop rapidly progression and present high mortality rate in the acute phase. Here, we established a murine model of ILD mediated by autoimmunity against MDA5. Mice immunized with recombinant murine MDA5 whole protein, accompanied with complete Freund's adjuvant once a week for four times, developed MDA5-reactive T cells and anti-MDA5 antibodies. After acute lung injury induced by intranasal administration of polyinosinic-polycytidylic acid [poly (I:C)] mimicking viral infection, the MDA5-immunized mice developed fibrotic ILD representing prolonged respiratory inflammation accompanied by fibrotic changes 2 wk after poly (I:C)-administration, while the control mice had quickly and completely recovered from the respiratory inflammation. Treatment with anti-CD4 depleting antibody, but not anti-CD8 depleting antibody, suppressed the severity of MDA5-induced fibrotic ILD. Upregulation of interleukin (IL)-6 mRNA, which was temporarily observed in poly (I:C)-treated mice, was prolonged in MDA5-immunized mice. Treatment with anti-IL-6 receptor antibody ameliorated the MDA5-induced fibrotic ILD. These results suggested that autoimmunity against MDA5 exacerbates toll-like receptor 3-mediated acute lung injury, and prolongs inflammation resulting in the development of fibrotic ILD. IL-6 may play a key role initiating ILD in this model.
Topics: Humans; Animals; Mice; Dermatomyositis; Prognosis; Disease Progression; Autoimmunity; Melanoma; Interferon-Induced Helicase, IFIH1; Autoantibodies; Lung Diseases, Interstitial; Interleukin-6; Inflammation; Acute Lung Injury; Retrospective Studies
PubMed: 38588434
DOI: 10.1073/pnas.2313070121 -
RMD Open Apr 2024We aimed to explore a new and readily available practical marker for rapidly progressive interstitial lung disease (RP-ILD) and poor short-term outcomes in patients with...
AIM
We aimed to explore a new and readily available practical marker for rapidly progressive interstitial lung disease (RP-ILD) and poor short-term outcomes in patients with idiopathic inflammatory myopathies (IIM).
METHODS
A total of 1822 consecutive patients with IIM between 2009 and 2021 were evaluated retrospectively. All proven cases of naïve ILD with complete medical records were included. Red cell distribution width (RDW) values at the initial stage, 3 months and last follow-up were collected. The clinical characteristics and outcomes of the patients were recorded.
RESULTS
We identified 532 patients with IIM with an average follow-up of 4 years. ILD prevalence was higher in patients of elevated RDW (p<0.001). The patients with ILD and elevated RDW had lower levels of PaO/FiO, FVC% and DLco% and a higher prevalence of RP-ILD than those with normal RDW (p<0.001). Prognostic analysis revealed that RDW was an independent risk factor for prognosis in patients with IIM-ILD (HR=2.9, p=0.03). Patients with dermatomyositis (DM) with RP-ILD with a change in RDW within 3 months (∆RDW-3) greater than 0 were more likely to die within 3 months. Moreover, the prevalence of ∆RDW-3>0 was higher in patients with RP-ILD and positive for anti-melanoma differentiation-associated gene 5 antibody who died within 3 months (87.5%) compared with those alive at 3 months (24.6%) (p<0.001).
CONCLUSION
These findings suggest that repeated RDW assays could assist physicians in identifying patients with DM-ILD who were at a high risk of RP-ILD and death.
Topics: Humans; Dermatomyositis; Retrospective Studies; Erythrocyte Indices; Lung Diseases, Interstitial; Myositis
PubMed: 38580342
DOI: 10.1136/rmdopen-2023-003931 -
Swiss Medical Weekly Mar 2024We report the first case of anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis as a systemic immune-related adverse event in a 64-year-old...
We report the first case of anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis as a systemic immune-related adverse event in a 64-year-old man receiving pembrolizumab to treat advanced lung cancer. The patient experienced hypothyroidism, myalgia, skin involvement, dyspnoea and diarrhoea. Laboratory tests revealed raised inflammatory markers, hypercreatinekinasemia and anti-MDA5 autoantibodies. Electroneuromyography and pathognomonic signs on physical examination confirmed the diagnosis of pauci-myopathic dermatomyositis. Pembrolizumab was discontinued and immunosuppressive therapy led to rapid and progressive improvement, with complete remission of dermatomyositis. This case report widens the spectrum of systemic immune-related adverse events associated with pembrolizumab.
Topics: Humans; Male; Middle Aged; Antibodies, Monoclonal, Humanized; Autoantibodies; Dermatomyositis; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Lung Neoplasms
PubMed: 38579289
DOI: 10.57187/s.3513 -
Rheumatology (Oxford, England) Apr 2024This study aimed to assess the incidence and risk factors surrounding mental illnesses in patients diagnosed with systemic autoimmune rheumatic diseases (SARDs).
OBJECTIVE
This study aimed to assess the incidence and risk factors surrounding mental illnesses in patients diagnosed with systemic autoimmune rheumatic diseases (SARDs).
METHODS
This retrospective cohort study used nationwide, population-based claim data taken from Taiwan's National Health Insurance Research Database (NHIRD) to identify patients certified as having a catastrophic illness for Systemic lupus erythematosus (SLE), Rheumatoid arthritis (RA), Systemic sclerosis (SSc), Dermatomyositis (DM), Polymyositis (PM) or Sjogren's syndrome (SS) from the years 2002-2020. We furthermore calculated the incidence of mental illness in patients diagnosed with SARDs while exploring factors associated with the development of mental illness using multivariable Cox regression analysis shown as adjusted hazard ratios (HRs) with 95% confidence intervals (CIs).
RESULTS
Among the 28 588 participants, the average age was 47.4 (SD 14.9) years, with most participants being female (76.4%). When compared with patients with rheumatoid arthritis, patients with SLE (HR: 1.20, 95% CI: 1.10-1.32), SS (HR: 1.29, 95% CI: 1.19-1.39), and DM (HR: 1.28, 95% CI: 1.04-1.32) showed a significantly increased risk of developing mental illness. Additionally, when compared with patients with rheumatoid arthritis, patients with SLE (HR: 1.32, 95% CI: 1.21-1.44), SSc (HR: 1.20, 95% CI: 1.02-1.41), SS (HR: 1.17, 95% CI: 1.08-1.26), DM (HR: 1.73, 95% CI: 1.44-2.07), and PM (HR: 1.64, 95% CI: 1.32-2.03) showed a significantly increased risk of antidepressant use.
CONCLUSIONS
This population-based cohort study revealed that patients diagnosed with SLE, SS and DM had significantly higher risks of developing mental illness when compared with patients with RA.
PubMed: 38579187
DOI: 10.1093/rheumatology/keae203