scholarly journals Morphological Characteristics of Idiopathic Inflammatory Myopathies in Juvenile Patients

Cells ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 109
Author(s):  
Anne Schänzer ◽  
Leonie Rager ◽  
Iris Dahlhaus ◽  
Carsten Dittmayer ◽  
Corinna Preusse ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Laboratory Data ◽  
Treatment Strategies ◽  
Surrogate Marker ◽  
Morphological Characteristics ◽  
Inflammatory Cells ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Morphological Findings ◽  
Characteristic Features

Background: In juvenile idiopathic inflammatory myopathies (IIMs), morphological characteristic features of distinct subgroups are not well defined. New treatment strategies require a precise diagnosis of the subgroups in IIM, and, therefore, knowledge about the pathomorphology of juvenile IIMs is warranted. Methods: Muscle biopsies from 15 patients (median age 8 (range 3–17) years, 73% female) with IIM and seven controls were analyzed by standard methods, immunohistochemistry, and transmission electron microscopy (TEM). Detailed clinical and laboratory data were accessed retrospectively. Results: Proximal muscle weakness and skin symptoms were the main clinical symptoms. Dermatomyositis (DM) was diagnosed in 9/15, antisynthetase syndrome (ASyS) in 4/15, and overlap myositis (OM) in 2/15. Analysis of skeletal muscle tissues showed inflammatory cells and diffuse upregulation of MHC class I in all subtypes. Morphological key findings were COX-deficient fibers as a striking pathology in DM and perimysial alkaline phosphatase positivity in anti-Jo-1-ASyS. Vascular staining of the type 1 IFN-surrogate marker, MxA, correlated with endothelial tubuloreticular inclusions in both groups. None of these specific morphological findings were present in anti-PL7-ASyS or OM patients. Conclusions: Morphological characteristics discriminate IIM subtypes in juvenile patients, emphasizing differences in aetiopathogenesis and supporting the notion of individual and targeted therapeutic strategies.

scholarly journals Myositis autoantibodies in a racially diverse population of children with idiopathic inflammatory myopathies

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Megan Mariko Perron ◽  
Natalia Vasquez-Canizares ◽  
Gabriel Tarshish ◽  
Dawn M. Wahezi
Keyword(s):  
Clinical Phenotype ◽  
Laboratory Data ◽  
Small Sample ◽  
Organ Involvement ◽  
Idiopathic Inflammatory Myopathies ◽  
Diverse Population ◽  
Inflammatory Myopathies ◽  
Racially Diverse ◽  
Parametric Testing ◽  
Overlap Myositis

Abstract Background Juvenile idiopathic inflammatory myopathies (JIIMs) is a group of autoimmune disorders, including juvenile dermatomyositis (JDM), juvenile polymyositis (JPM) and overlap myositis, that are characterized by proximal muscle weakness, elevated levels of serum muscle enzymes, and pathognomonic skin findings. While the exact etiology of JIIMs is unclear, the presence of myositis specific autoantibodies (MSAs) have been associated with certain clinical phenotypes, organ involvement and disease prognosis. To date, there have been few studies of the associations between MSA presence and patient ethnicity. It is important to understand the extent to which ethnicity impacts disease manifestations, organ involvement and clinical outcomes. The goal of our study is to determine MSA and myositis associated autoantibody (MAA) presence, clinical phenotype, and disease course in a racially diverse population of pediatric patients with JIIMs. Methods Patients age 2–21 years with a prior diagnosis of JDM, JPM or overlap myositis, who had been tested for MSA/MAA, were eligible for study inclusion. Clinical and laboratory data were collected retrospectively via manual chart review in this single-center study. Descriptive statistics were performed to summarize each variable. Given the small sample size, non-parametric testing was performed using Fischer’s exact test, Wilcoxon rank sum test and Kruskal-Wallis test. Results Thirty one patients were included in the analysis. Race and ethnicity were self-reported as Hispanic (48.4%), white (25.8%), and Black (25.8%). The most prevalent MSAs were anti-MDA5 (25.8%), anti-p155/140 (22.6%) and anti-MJ (19.4%). Presence of autoantibodies (p = 0.04) and pulmonary disease (p = 0.03) were significantly higher in patients of Black or Hispanic descent compared with white descent. Anti-MDA5 antibodies, cutaneous ulceration, cardiopulmonary involvement, hospitalizations and one death were only reported in patients with Black or Hispanic descent. Patients with anti-MDA5 antibodies were more likely to be male (p = 0.04) and to have cutaneous ulceration (p = 0.02). Conclusions This study describes the prevalence of MSA/MAA in a racially diverse group of patients with JIIM and further delineates clinical phenotype and disease complications in these groups. We found a relatively high proportion of children with anti-MDA5 antibodies and described potentially worse clinical courses in children of Black or Hispanic descent. Further investigation is warranted to examine these findings.

Serum levels of galectin-3 in idiopathic inflammatory myopathies: a potential biomarker of disease activity

Rheumatology ◽  
2020 ◽  
Vol 60 (1) ◽  
pp. 322-332
Author(s):  
Eri Watanabe ◽  
Kazunori Kato ◽  
Takahisa Gono ◽  
Emiko Chiba ◽  
Chihiro Terai ◽  
...  
Keyword(s):  
Disease Activity ◽  
Interstitial Pneumonia ◽  
Activity Index ◽  
Biological Activities ◽  
Inflammatory Cells ◽  
Serum Levels ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Potential Biomarker ◽  
Galectin 3

Abstract Objectives Galectin-3 is involved in various biological activities, including immune activations and fibrosis. Idiopathic inflammatory myopathies (IIMs) are autoimmune diseases of unknown aetiology, often complicated by interstitial lung disease (ILD). The aim of this study was to evaluate the expression of galectin-3 in sera and tissues of patients with IIM and assess the associations of galectin-3 with patient characteristics and disease activity. Results Serum galectin-3 levels were significantly higher in IIM patients than in healthy controls. The serum galectin-3 levels positively correlated with serum levels of inflammatory markers and proinflammatory cytokines/chemokines and the Myositis Intention-to-Treat Activity Index. Stratification analysis revealed that patients with IIM-associated ILD (IIM-ILD) had significantly higher levels of serum galectin-3 than those without IIM-ILD. In addition, patients with acute/subacute interstitial pneumonia had significantly higher levels of serum galectin-3 than those with chronic interstitial pneumonia. Furthermore, serum galectin-3 levels in IIM-ILD patients correlated with the radiological assessments of parenchymal lung involvement and treatment response. Immunohistochemical analysis revealed that galectin-3 was expressed in inflammatory cells of myositis and dermatitis sections, whereas in ILD sections, galectin-3 was expressed in interstitial fibrosis and inflammatory cells. Conclusion Galectin-3 may be involved in the pathogenesis of inflammatory and fibrotic conditions in IIM and can serve as a potential biomarker of disease activity, especially in patients with IIM-ILD.

scholarly journals NK Cell Patterns in Idiopathic Inflammatory Myopathies with Pulmonary Affection

Cells ◽  
2021 ◽  
Vol 10 (10) ◽  
pp. 2551
Author(s):  
Marc Pawlitzki ◽  
Christopher Nelke ◽  
Leoni Rolfes ◽  
Rebecca Hasseli ◽  
Stylianos Tomaras ◽  
...  
Keyword(s):  
Cell Surface ◽  
Nk Cells ◽  
Nk Cell ◽  
Surrogate Marker ◽  
Clinical Severity ◽  
Cell Surface Expression ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Cell Repertoire ◽  
Color Flow

Background: Pulmonary affection (PA) is associated with a substantial increase in morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). However, the underlying immune mechanisms of PA remain enigmatic and prompt deeper immunological analyses. Importantly, the Janus-faced role of natural killer (NK) cells, capable of pro-inflammatory as well as regulatory effects, might be of interest for the pathophysiologic understanding of PA in IIM. Methods: To extend our understanding of immunological alterations in IIM patients with PA, we compared the signatures of NK cells in peripheral blood using multi-color flow cytometry in IIM patients with (n = 12, of which anti-synthetase syndrome = 8 and dermatomyositis = 4) or without PA (n = 12). Results: We did not observe any significant differences for B cells, CD4, and CD8 T cells, while total NK cell numbers in IIM patients with PA were reduced compared to non-PA patients. NK cell alterations were driven by a particular decrease of CD56dim NK cells, while CD56bright NK cells remained unchanged. Comparisons of the cell surface expression of a large panel of NK receptors revealed an increased mean fluorescence intensity of NKG2D+ on NK cells from patients with PA compared with non-PA patients, especially on the CD56dim subset. NKG2D+ and NKp46+ cell surface levels were associated with reduced vital capacity, serving as a surrogate marker for clinical severity of PA. Conclusion: Our data illustrate that PA in IIM is associated with alterations of the NK cell repertoire, suggesting a relevant contribution of NK cells in certain IIMs, which might pave the way for NK cell-targeted therapeutic approaches.

Pediatric Idiopathic Inflammatory Myopathies: An Update on Diagnostic and Treatment Strategies

2013 ◽  
Vol 44 (06) ◽  
pp. 314-323 ◽  
Author(s):  
Julia Wanschitz ◽  
Katharina Vill ◽  
Matthias Baumann ◽  
Wolfgang Muller-Felber
Keyword(s):  
Treatment Strategies ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

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