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Abstract
Objective. The current classification criteria for idiopathic inflammatory myopathy (IIM) retain PM as a major disease subgroup. However, evolution in the understanding of IIM has suggested that many of these patients could be better described as having an alternative diagnosis. In the present study, we apply the latest understanding of IIM subtyping to retrospectively review PM diagnoses in a large cohort of IIM patients. Methods. Within a previously reported cohort of 255 patients from a UK tertiary myositis clinic, 37 patients classified as PM according to both the EULAR/ACR IIM criteria and expert opinion were identified. Clinical data and complementary tests were reviewed, and consensus decisions regarding final classification were reached in each case. Results. Nine (9/37, 24.3%) patients remained classified as PM, 3.5% (9/255) of the original cohort; these PM patients were seronegative for myositis antibodies, responsive to immunosuppression, and in 4/7 (57.1%) patients where muscle biopsy was performed had HLA-1 upregulation and endomysial inflammatory infiltrates. Immunemediated necrotizing myopathy (5/37, 13.5%) and connective tissue disease overlap myositis (7/37, 19%) were the main alternative diagnoses. The remaining patients were diagnosed as: unspecified myopathy (6/37, 16%), dermatomyositis (2/37, 5%), cancer-associated myopathy (3/37, 8.1%), and non-inflammatory myopathy (1/37, 3%, myofibrillar myopathy). Four patients (4/37, 10%) had insufficient data available to confidently reclassify. Conclusion. Our study confirms that PM can now be considered a rare IIM subgroup. A thorough examination, complete myositis autoantibody panel, and careful interpretation of the biopsy results is recommended to confirm the correct IIM sub-type.
Original language | English |
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Pages (from-to) | 3398-3403 |
Number of pages | 6 |
Journal | Rheumatology |
Volume | 60 |
Issue number | 7 |
Early online date | 24 Dec 2020 |
DOIs | |
Publication status | Published - 1 Jul 2021 |
Keywords
- CTD overlap myositis
- PM
- idiopathic inflammatory myopathy
- immune-mediated necrotizing myopathy
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Dive into the research topics of 'Polymyositis: is there anything left? A retrospective diagnostic review from a tertiary myositis centre'. Together they form a unique fingerprint.Projects
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MMRG: Manchester Myositis Research Group
Chinoy, H. (PI), Lamb, J. (PI), Ollier, W. (PI), Rothwell, S. (CoI), Lilleker, J. (CoI), Oldroyd, A. (PGR student), Snedden, A. (PGR student), Platt, H. (Support team) & New, P. (Support team)
1/01/10 → …
Project: Research
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Press/Media
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Retrospectively reviewing polymyositis diagnoses in a large single-centre cohort
15/02/21
1 Media contribution
Press/Media: Blogs and social media