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RMD Open Apr 2024Risk prediction for patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD) is challenging due to heterogeneity in the disease...
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.
Topics: Humans; Lung Diseases, Interstitial; Male; Female; Middle Aged; Dermatomyositis; Risk Assessment; Prognosis; Aged; Adult; Risk Factors; Logistic Models; Polymyositis; ROC Curve
PubMed: 38663883
DOI: 10.1136/rmdopen-2023-003850 -
International Journal of Rheumatic... Apr 2024To determine the direct health service costs and resource utilization associated with diagnosing and characterizing idiopathic inflammatory myopathies (IIMs), and to...
AIM
To determine the direct health service costs and resource utilization associated with diagnosing and characterizing idiopathic inflammatory myopathies (IIMs), and to assess for limitations and diagnostic delay in current practice.
METHODS
A retrospective, single-center cohort analysis of all patients diagnosed with IIMs between January 2012 and December 2021 in a large tertiary public hospital was conducted. Demographics, resource utilization and costs associated with diagnosing IIM and characterizing disease manifestations were identified using the hospital's electronic medical record and Health Intelligence Unit, and the Medicare Benefits Schedule.
RESULTS
Thirty-eight IIM patients were identified. IIM subtypes included dermatomyositis (34.2%), inclusion body myositis (18.4%), immune-mediated necrotizing myopathy (18.4%), polymyositis (15.8%), and anti-synthetase syndrome (13.2%). The median time from symptom onset to diagnosis was 212 days (IQR: 118-722), while the median time from hospital presentation to diagnosis was 30 days (8-120). Seventy-six percent of patients required emergent hospitalization during their diagnosis, with a median length of stay of 8 days (4-15). The average total cost of diagnosing IIM was $15 618 AUD (STD: 11331) per patient. Fifty percent of patients underwent both MRI and EMG to identify affected muscles, 10% underwent both pan-CT and PET-CT for malignancy detection, and 5% underwent both open surgical and percutaneous muscle biopsies. Autoimmune serology was unnecessarily repeated in 37% of patients.
CONCLUSION
The diagnosis of IIMs requires substantial and costly resource use; however, our study has identified potential limitations in current practice and highlighted the need for streamlined diagnostic algorithms to improve patient outcomes and reduce healthcare-related economic burden.
Topics: Humans; Retrospective Studies; Myositis; Male; Female; Middle Aged; Tertiary Care Centers; Hospitals, Public; Hospital Costs; Aged; Adult; Health Resources; Health Care Costs; Delayed Diagnosis; Predictive Value of Tests; Time Factors; Australia
PubMed: 38661316
DOI: 10.1111/1756-185X.15153 -
Frontiers in Immunology 2024Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy affecting children, being marked by chronic inflammation which mostly impacts on both skin and... (Review)
Review
Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy affecting children, being marked by chronic inflammation which mostly impacts on both skin and skeletal muscles; diagnostic criteria of JDM include an unforeseeable mixture of clinical features, while treatment modalities commonly require corticosteroids or immunosuppressant agents. Although the pathogenesis of JDM is not completely understood, several infectious triggers have been linked to its priming via anecdotal reports related to children. Pediatric cases of recent-onset JDM have been temporally associated to an infectious disease by the power of increased titers of circulating antibodies to a putative infectious agent, including parasites, and/or detectable viral RNA or bacterial DNA. With this narrative review we offer an update about JDM association with a host of infections, namely parvovirus B19, Epstein-Barr virus, Coxsackie virus, human immune deficiency virus, severe acute respiratory syndrome coronavirus 2, and , as resulting from the medical literature. Few are the evidence-proved results addressing JDM as an unambiguous post-infectious disorder and available data specifically related to children are poor, highlighting the need of further research into the exploration between environmental cut-out factors and JDM.
Topics: Humans; Dermatomyositis; Child; COVID-19; SARS-CoV-2
PubMed: 38660309
DOI: 10.3389/fimmu.2024.1377952 -
BMJ (Clinical Research Ed.) Apr 2024
PubMed: 38658038
DOI: 10.1136/bmj.q941 -
Therapeutische Umschau. Revue... Feb 2024Interstitial Lung Disease associated with Connective Tissue Diseases Abstract: Interstitial lung diseases (ILD) are in up to one-third of cases associated with... (Review)
Review
Interstitial Lung Disease associated with Connective Tissue Diseases Abstract: Interstitial lung diseases (ILD) are in up to one-third of cases associated with connective tissue diseases (CTD). In systemic sclerosis, rheumatoid arthritis, polymyositis/dermatomyositis, Sjögren's syndrome, and mixed connective tissue disease, an associated ILD significantly increases morbidity and mortality. The diagnostic workup for suspected CTD-ILD includes a range of functional, radiological, laboratory, and, if necessary, invasive tests. A thorough medical history and physical examination with targeted rheumatological diagnosis is particularly important. Also, patients with unclassified ILDs should be evaluated thoroughly for any underlying CTD. Pharmacological treatment options for CTD-ILD differ significantly from those for other ILDs. In addition to short-term glucocorticoids, antimetabolites and biological agents are often used. Antifibrotic drugs have also been successfully used in CTD-ILDs. The decision on whether and which immunosuppressive and/or antifibrotic therapy is indicated depends on the underlying disease, disease activity, extrapulmonary manifestations, severity of organ involvement, ILD progression, comorbidities, and patient preferences. Complex treatment decisions are ideally made in multidisciplinary expert teams.
Topics: Lung Diseases, Interstitial; Humans; Connective Tissue Diseases; Immunosuppressive Agents; Interdisciplinary Communication
PubMed: 38655827
DOI: No ID Found -
Orphanet Journal of Rare Diseases Apr 2024Rapid progressive interstitial lung disease (RP-ILD) is the leading cause of anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis...
Elevated serum B-cell activator factor levels predict rapid progressive interstitial lung disease in anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis.
BACKGROUND
Rapid progressive interstitial lung disease (RP-ILD) is the leading cause of anti-melanoma differentiation associated protein 5 antibody positive dermatomyositis (anti-MDA5DM) related death. Elevated serum B-cell activating factor (BAFF) levels have been implicated in connective tissue diseases associated ILD. Here, we evaluate whether BAFF could be a prognostic biomarker for predicting RP-ILD in anti-MDA5DM patients.
METHODS
Serums were collected from 39 patients with anti-MDA5DM (20 with RP-ILD and 19 with non-RP-ILD), 20 antisynthase syndrome (ASS) patients and 20 healthy controls (HC). BAFF concentration was measured by an enzyme-linked immunosorbent assay.
RESULTS
Serum BAFF level was higher in anti-MDA5DM patients than those in ASS patients and HC (3882.32 ± 1880.09 vs. 2540.89 ± 1403.04 and 2486.28 ± 767.97 pg/mL, p = 0.0056 and 0.0038, respectively). Within anti-MDA5DM groups, RP-ILD patients exhibited higher BAFF concentration than non-RP-ILD group (4549.78 ± 1839.97 vs. 3297.28 ± 1794.69 pg/mL, p = 0.04). The BAFF concentration was positively correlated with levels of C-reactive protein (CRP), dehydrogenase (LDH) and cytokeratin (CK) in anti-MDA5DM patients (r = 0.350, p = 0.035; r = 0.393, p = 0.016; r = 0.518, p = 0.001; respectively). The best cut-off value of BAFF concentration was 2971.5 pg/mL by ROC curve (AUC area = 0.690, p = 0.045) and BAFF > 2971.5 pg/mL was an independent risk factor for RP-ILD using multivariate analysis (OR = 9.389, 95% CI = 1.609-54.769; p = 0.013).
CONCLUSIONS
Serum BAFF could be a useful prognostic biomarker for early detecting RP-ILD risk in anti-MDA5DM patients.
Topics: Humans; Dermatomyositis; Interferon-Induced Helicase, IFIH1; Autoantibodies; Lung Diseases, Interstitial; Biomarkers; Prognosis; Retrospective Studies; Disease Progression
PubMed: 38637830
DOI: 10.1186/s13023-024-03153-6 -
JAMA Dermatology Jun 2024
Topics: Humans; Dermatomyositis; Interferon-Induced Helicase, IFIH1; Hospitalization; Female; Continuity of Patient Care; Male; Middle Aged; Adult; Autoantibodies
PubMed: 38630455
DOI: 10.1001/jamadermatol.2024.0403 -
Basic and Clinical Neuroscience 2023Muscle biopsy is commonly used to diagnose inflammatory myopathies. We evaluated the ability of muscle ultrasound, a non-invasive and simple tool, to distinguish between...
INTRODUCTION
Muscle biopsy is commonly used to diagnose inflammatory myopathies. We evaluated the ability of muscle ultrasound, a non-invasive and simple tool, to distinguish between healthy subjects and patients with inflammatory myopathy.
METHODS
This study was conducted on 17 patients recently diagnosed with biopsy inflammatory myopathies (12 dermatomyositis, 5 polymyositis) compared with 17 age- and gender-matched healthy control adults. All patients underwent clinical assessments, including manual muscle testing, hand-held dynamometry, and muscle ultrasound evaluations, including thickness and echo intensity in predefined muscle groups.
RESULTS
The disease duration was seven months (interquartile range: 3 to 11 months). Except for the biceps and gastrocnemius, patients' muscles had significantly higher echo intensity and lower thickness than the control group. The echo intensity sum-score manifested the highest area under the curve compared to the sum-scores of other variables (echo intensity vs manual muscle testing: Area under curves-difference=0.18, P<0.01; echo intensity vs dynamometry: Area under curves-difference=0.14, P=0.02; echo intensity vs thickness: Area under curves-differences-difference=0.25, P<0.01).
CONCLUSION
The echo intensity of muscles differed significantly between healthy individuals and patients with inflammatory myopathies and may serve as a useful diagnostic biomarker.
PubMed: 38628832
DOI: 10.32598/bcn.2021.3567.1 -
International Journal of Surgery Case... May 2024Intestinal duplication is an uncommon congenital malformation affecting the alimentary tract. This article presents a case of enteric duplication cyst (EDC) in an adult,...
INTRODUCTION AND IMPORTANCE
Intestinal duplication is an uncommon congenital malformation affecting the alimentary tract. This article presents a case of enteric duplication cyst (EDC) in an adult, accompanied by a review of the available literature.
CASE PRESENTATION
A 34-year-old woman with polymyositis underwent a routine CT scan as part of her medical assessment revealing an 8 cm mass near the caecum and terminal ileum. Diagnostic procedures confirmed a cystic spherical mass. The patient underwent ileo-cecal resection, with primary anastomosis and an uneventful recovery.
CLINICAL DISCUSSION
Studies indicate that the frequency of polymyositis coexisting with a neoplasm range from 6 % to 40 %. Therefore, a body CT scan is recommended for patients with myopathy as in our patient. Intestinal duplications are predominantly found in children but can also occur in adults, often discovered incidentally or due to complications. Diagnostic imaging techniques, such as ultrasonography and CT scan, are crucial in identifying duplication location and characteristics. In this case, colonoscopy indicated ileocecal valve compression, and histological examination confirmed an enteric duplication cyst with ectopic gastric mucosa.
CONCLUSION
Enteric duplication cysts are rare, and the existing literature on the topic somewhat limited. Early diagnosis and surgical intervention are essential to stave off potential complications and reduce morbidity. Clinician awareness of enteric duplication cysts enables timely management, enhancing patient outcomes. Further research is needed to improve understanding and optimize patient care.
PubMed: 38615468
DOI: 10.1016/j.ijscr.2024.109606 -
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