Inflammatory milieu of muscle biopsies in juvenile dermatomyositis
- PDF / 1,843,550 Bytes
- 9 Pages / 595.276 x 790.866 pts Page_size
- 74 Downloads / 206 Views
Rheumatology International https://doi.org/10.1007/s00296-020-04735-w
INTERNATIONAL
OBSERVATIONAL RESEARCH
Inflammatory milieu of muscle biopsies in juvenile dermatomyositis Erdal Sag1 · Gulsev Kale2 · Goknur Haliloglu3 · Yelda Bilginer1 · Zuhal Akcoren2 · Diclehan Orhan2 · Safak Gucer2 · Haluk Topaloglu3 · Seza Ozen1 · Beril Talim2 Received: 17 September 2020 / Accepted: 13 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Juvenile dermatomyositis (JDM) is an inflammatory myopathy which causes severe morbidity and high mortality if untreated. In this study, we aimed to define the T-helper cell profile in the muscle biopsies of JDM patients. Muscle biopsies of twentysix patients (50% female) were included in the study. Immunohistochemical expression of CD3, CD20, CD138, CD68, IL-17, Foxp3, IFN-ɣ, IFN-alpha and IL-4 was studied and muscle biopsies were scored using the JDM muscle biopsy scoring tool. Inflammatory cells were in small clusters in perimysium and perivascular area or scattered throughout the endomysium in most biopsies; however in 2 biopsies, lymphoid follicle-like big clusters were observed, and in one, there was a very dense and diffuse inflammatory infiltration nearly destroying all the muscle architecture. Seventy-three per cent of the biopsies had T cells, 88% had B cells, 57% had plasma cells, and all had macrophages. As for T-helper cell subtypes, 80% of the biopsies were Th1 positive, 92% Th17 positive and 30% Treg positive. No IL-4 positive inflammatory cell was detected, and only 2 biopsies showed IFN-alpha positivity. The mean JDM biopsy score was 17.6, meaning moderate to severe muscular involvement. Visual analogue score of the pathologist was strongly correlated with histopathological features. B cells, macrophages, plasma cells and T cells constitute the inflammatory milieu of the JDM muscle biopsies. As for T cells, JDM is a disease mainly related with Th1 and Th17 T-helper cell subtypes and to some extend Treg. Th2 cells are not involved in the pathogenesis. Keywords JDM · Muscle biopsy · Inflammatory cells · T cells · B cells
Introduction Juvenile dermatomyositis (JDM) is a rare but severe autoimmune disease and is the most common of the idiopathic inflammatory myopathies in childhood, with an incidence of 2–3 million children/year below age of 16 [1]. It was first described in 1887 by 4 different researchers, among them Unverricht defined the cutaneous and muscle features and Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00296-020-04735-w) contains supplementary material, which is available to authorized users. * Erdal Sag [email protected] 1
Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey
2
Pediatric Pathology Unit, Department of Pediatrics, Hacettepe University, Ankara, Turkey
3
Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey
called the disease as dermatom
Data Loading...
