Tan, Cheng Yin and Ambang, Tomica and Raja Mohamed, Raja Jasmin Begum and Shahrizaila, Nortina and Sockalingam, Sargunan and Suzuki, Shigeaki and Nishino, Ichizo and Wong, Kum Thong and Goh, Khean Jin (2021) Immune-mediated necrotizing myopathy in a multi-ethnic Malaysian cohort. Neurology Asia, 26 (2). pp. 291-299. ISSN 1823-6138,
Full text not available from this repository.Abstract
Objective: To describe the clinical features and treatment outcomes of immune-mediated necrotizing myopathy (IMNM) in Malaysian patients. Methods: We describe a cohort of IMNM patients from a tertiary medical centre in Kuala Lumpur, Malaysia, in terms of their demography, clinical features, investigations, treatments and outcome. Comparisons were made between the anti-signal recognition particle (SRP) positive, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) positive and seronegative subgroups. Results: IMNM was seen in 23.7% of inflammatory myopathy cases in the University of Malaya muscle biopsy databank. Of these, 35 patients who underwent serological testing for IMNM myositis-specific antibodies were included in the study. 45.7% were anti-SRP positive, 20% anti-HMGCR positive, and 28.6% seronegative. Two (5.7%) patients had dual positivity. Mean age of onset was 37.1 +/- 17.0 years (range of 8 to 76 years) with female predominance (743%). Twenty three (65.7%) patients presented subacutely (<= 6 months) with the majority having symmetrical severe weakness (MRC grade <= 3), with proximal and lower limb predominance. Four (11.4%) had respiratory involvement but none had cardiac symptoms. Extramuscular manifestations included skin lesions (20%), arthralgia (5.7%), associated connective tissue disease (11.4%) and none with cancer. Anti-SRP positivity was significantly associated with muscle wasting and anti-HMGCR group with cutaneous involvement. Anti-HMGCR positivity was not significantly associated with statin use. Most patients required a combination of at least two immunotherapies and at follow up, 50% showed good recovery with minimal weakness while a third had at least moderately severe disability. Conclusion: IMNM is a common inflammatory myopathy seen in all ethnic groups in Malaysia. Its clinical characteristics are consistent with other populations. Extramuscular involvement is uncommon. Most patients require combined immunosuppressive therapy with variable outcome; about half having a good outcome with minimal disability and a third with more severe disease with significant persistent disability.
| Item Type: | Article |
|---|---|
| Funders: | UNSPECIFIED |
| Uncontrolled Keywords: | Immune-mediated nccrotizing myopathy; Necrotizing autoimmunc myopathy; Anti-SRP myopathy; Anti-HMGCR myopathy; Inflammatory myopathy; Necrotizing myopathy |
| Subjects: | R Medicine > RC Internal medicine > RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry |
| Divisions: | Faculty of Medicine > Medicine Department Faculty of Medicine > Pathology Department |
| Depositing User: | Ms Zaharah Ramly |
| Date Deposited: | 13 Jun 2022 01:12 |
| Last Modified: | 13 Jun 2022 01:12 |
| URI: | http://eprints.um.edu.my/id/eprint/34284 |
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