| Title: |
Occurrence of focal myositis during Behçet's disease: The identification of a specific vasculitis‐associated focal myopathy |
| Authors: |
Gallay, Laure; Hot, Arnaud; Allenbach, Yves; Maucort‐Boulch, Delphine; Comarmond, Cloe; Marques, Cindy; Perard, Laurent; Simon, Anne; Mariampillai, Kuberaka; Cacoub, Patrice; Mery‐Bossard, Laure; Cathebras, Pascal; Feasson, Leonard; Berezne, Alice; Morati, Chafika; Lessard, Lola; Faruch, Marie; Streichenberger, Nathalie; Saadoun, David |
| Source: |
Neuropathology and Applied Neurobiology ; volume 49, issue 2 ; ISSN 0305-1846 1365-2990 |
| Publisher Information: |
Wiley |
| Publication Year: |
2023 |
| Collection: |
Wiley Online Library (Open Access Articles via Crossref) |
| Description: |
Aims This study aimed to report the association of focal myositis (FM) and Behçet's disease (BD) and to analyse the main characteristics of such an association. Methods This is a retrospective multicentre study of patients with BD and FM (BD + FM+ group) and those without FM (BD − FM+ group). Clinical, laboratory, radiological, pathological, treatment and outcome data were analysed. Results The BD + FM+ group included 10 patients; the median [interquartile range] age at BD diagnosis was 25 [16–35] years, and at FM diagnosis, it was 30 [26–42] years. The diagnosis of BD preceded FM in the majority of cases ( n = 8/10). FM occurrence was associated with BD flare‐ups in three cases. The creatine kinase levels remained normal or slightly increased. Histological analyses identified relatively preserved muscle tissue, associated with vasculitis ( n = 5/6). All patients required treatment; most patients relapsed ( n = 9/10). The BD − FM+ group included 35 patients. A comparison of the groups identified a trend towards a younger median age at diagnosis of FM among those with BD ( p = 0.063) and more frequent focal muscle swelling in the BD + FM+ group ( p = 0.029). The pathological analysis identified significantly less frequent muscle alterations in the BD + FM+ group (muscle fibre size heterogeneity, p = 0.021; necrosis, p = 0.007; and fibrosis, p = 0.027). BD + FM+ patients had a higher frequency of relapse ( p = 0.003) and systematic treatment ( p = 0.042). Conclusions FM occurring during BD appears to be part of the systemic vasculitis process and presents as a vasculitis‐associated focal myopathy with a specific clinico‐histological pattern. Patients with this association require long‐term follow‐up and adapted management. This case series also highlights the need for research on BD diagnostic criteria in cases of FM. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1111/nan.12900 |
| Availability: |
https://doi.org/10.1111/nan.12900; https://onlinelibrary.wiley.com/doi/pdf/10.1111/nan.12900 |
| Rights: |
http://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.C61F8EDF |
| Database: |
BASE |