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Abstract
Objective To better define in a cohort study the clinical and pathologic features of focal myositis (FM).
Methods With the use of the usual clinicopathologic definition, each confirmed case of FM in the Lyon University Hospital's myopathologic database between 2000 and 2016 was retrieved. Clinical, pathologic, imaging, serologic, and therapeutic data were collected. When data were missing but feasible, appropriate pathologic analyses were performed.
Results Of the 924 patients included in the database, 37 (4%) had confirmed FM (14 female, 23 male patients). The main symptoms were pain (n = 30, 81%), muscular mass (n = 16, 43%), erythema at the site of FM (n = 12, 32%), and fever (n = 9, 24%). Serum creatine kinase was normal in most patients (81%); serum immune abnormalities were frequent (inflammatory syndrome in sera [39%], dysglobulinemia [52%], and anti-nuclear antibody positivity [29%]). In addition to confirming previously reported findings, pathologic analyses found significant rates of vasculitis (68%) and fasciitis (73%). Here, FM appeared frequently to be associated with other diseases such as immune-mediated inflammatory disease (IMID; 32%), neoplasia (24%), and radiculopathy (11%). Regarding outcomes, 64% of the cases had received immunosuppressive drugs, and the relapse rate was 41%.
Conclusion The present study suggests that FM is not as innocuous as previously believed, particularly considering the associated disorders. Notably, patients with FM should receive careful IMID and neoplasia screening.
Glossary
- CREST=
- calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia;
- FM=
- focal myositis;
- FMSS=
- FM simple score;
- HPS=
- hematoxylin-phloxine-saffron;
- IMID=
- immune-mediated inflammatory disease;
- MHC-I=
- major histocompatibility complex class I;
- NADI=
- no associated disorders identified
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
CME Course: NPub.org/cmelist
- Received October 31, 2016.
- Accepted in final form December 20, 2017.
- © 2018 American Academy of Neurology
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