Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy

Watanabe, Yoshifumi; Uruha, Akinori; Suzuki, Shigeaki; Nakahara, Jin; Hamanaka, Kohei; Takayama, K.; Suzuki, Norihiro; Nishino, Ichizo

doi:10.1136/jnnp-2016-313166

Cited by 257 publications

(304 citation statements)

References 26 publications

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“…Respiratory muscle weakness was not routinely investigated, nor was cardiac dysfunction, although both have been reported in the literature, particularly in association with anti-SRP (1,5,17).…”

Section: Discussionmentioning

confidence: 99%

Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

Ashton

Junckerstorff

Bundell

et al. 2016

Neuromuscular Disorders

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Section: Discussionmentioning

confidence: 99%

Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

Ashton

Junckerstorff

Bundell

et al. 2016

Neuromuscular Disorders

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“…Specifically, autoantibody-negative IMNM has been associated with a markedly increased risk of malignancy (95%SCR CI: 8.35–24.41) [30••]. In contrast, anti-HMGCR myopathy may have a relatively weak association with cancer (95%SCR CI: from 0.4–2.4 to 1.02–6.07) [30••], although this has not been demonstrated in all cohorts [21••, 31•, 32•, 33••]. Finally, anti-SRP myopathy is not associated with cancer [13••, 16••, 30••, 33••, 34•].…”

Section: Cancer Associationmentioning

confidence: 99%

“…As in other forms of myositis, anti-HMGCR and anti-SRP myopathy cases usually have multifocal upregulation of class I major histocompatibility complex (~ 50%) and the deposition of membrane attack complex (MAC) on the sarcolemma of non-necrotic muscle fibers (~ 20–50%) [1, 2, 8, 13••, 32•, 33••, 34•, 43•]. The presence of perifascicular atrophy or non-necrotic fibers surrounded and invaded by lymphocytes (primary inflammation) is uncommon, if ever present [1, 2, 8, 13••, 32•, 33••, 34•, 43•]. In anti-HMGCR myopathy, it has been shown that the inflammatory infiltrate is mainly composed of macrophages, but that scant CD4+ or CD8+ (both ~ 50%) lymphocytes as well as CD123+ plasmacytoid dendritic cells (~ 70%) can also be present (Fig.…”

Section: Diagnosis and Managementmentioning

confidence: 99%

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Immune-Mediated Necrotizing Myopathy

2018

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Purpose of Review Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. Recent Findings Anti-signal recognition particle (SRP) and anti-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies are closely associated with IMNM and define unique subtypes of patients. Importantly, the new European Neuromuscular Centre criteria recognize anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM as three distinct subtypes of IMNM. Anti-SRP myopathy patients have more severe muscle involvement, have more common extra-muscular features, and may respond best to immunosuppressive regimens that include rituximab. In contrast, anti-HMGCR myopathy is often associated with statin exposure and intravenous immunoglobulin treatment may be an effective treatment, even as monotherapy. Both anti-SRP and anti-HMGCR myopathy tend to be most severe in younger patients. Furthermore, children with these forms of IMNM may present with dystrophy-like features which are potentially reversible with immunosuppressant treatment. IMNM patients with either autoantibody may experience fatty replacement of muscle soon after disease onset, suggesting that intense and early immunosuppressant therapy may provide the best chance to avoid long-term disability. Summary IMNM is composed of anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM. Both anti-SRP and anti-HMGCR myopathy can cause severe weakness, especially in younger patients. Anti-SRP myopathy patients tend to have the most severe weakness and most prevalent extra-muscular features. Autoantibody-negative IMNM remains poorly described.

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“…Watanabe et al 1 report on prospectively studied patients with necrotising autoimmune myopathy (NAM), diagnosed among 460 individuals with inflammatory myopathies (IM), over a 4-year period. This is the largest ever NAM cohort diagnosed with histological criteria and exclusion of genetically-defined muscular dystrophies.…”

mentioning

confidence: 99%

Necrotising autoimmune myopathy (NAM): antibodies seem to be specific markers in aiding diagnosis

Dalakas

2016

J Neurol Neurosurg Psychiatry

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Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy

Abstract: Anti-SRP antibodies are associated with severe neurological symptoms, more so than are anti-HMGCR antibodies. Although these autoantibodies are independent serological markers associated with IMNM, patients bearing either share common characteristics.

Cited by 257 publications

References 26 publications

Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

Immune-Mediated Necrotizing Myopathy

Necrotising autoimmune myopathy (NAM): antibodies seem to be specific markers in aiding diagnosis

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