Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy

Hou, Ying; Shao, Kai; Yan, Yaping; Dai, Tao; Li, Wei; Zhao, Yuying; Li, Duoling; Lu, Jian‐Qiang; Norman, Gary L.; Yan, Chuanzhu

doi:10.1007/s00415-021-10621-7

Cited by 15 publications

(21 citation statements)

References 41 publications

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“…Muscle biopsy in anti-HMGCR antibody-positive IMNM demonstrates necrosis with a pauci-inflammatory infiltrate, whereas biopsy specimens of DM rarely show necrosis, instead displaying a perivascular and perifascicular CD4 + T-lymphocytic infiltrate and perifascicular atrophy. 6 , 9 Both have a higher incidence of cancer, with DM showing a stronger association compared with anti-HMGCR antibody-positive IMNM (6-fold vs 2-fold increase). 2 Lastly, DM and anti-HMGCR antibody-positive IMNM differ in their treatments with the latter more often necessitating intravenous immunoglobulin, rituximab, and systemic corticosteroids, as required by our patient.…”

Section: Discussionmentioning

confidence: 98%

“… 5 One case series found that 9 of 21 anti-HMGCR antibody-positive patients had DM-like rashes, including heliotrope rash, Gottron sign, and erythema of the cheeks, limbs, chest, abdomen, and back; these patients were more likely to be younger and to experience juvenile-onset disease compared with anti-HMGCR antibody-positive patients without DM-like rashes. 6 …”

Section: Discussionmentioning

confidence: 99%

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Statin-naïve anti–3-hydroxy-3-methylglutaryl coenzyme A reductase antibody-positive necrotizing myopathy with heliotropic pseudoangioedema

et al. 2022

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Statin-naïve anti–3-hydroxy-3-methylglutaryl coenzyme A reductase antibody-positive necrotizing myopathy with heliotropic pseudoangioedema

et al. 2022

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“…Two patients had accompanying perifascicular atrophy, which is a known histopathological feature of DM. Dermatomyositis cases with anti-HMGCR autoantibodies have been anecdotally reported previously; one study even suggested presence of distinctive skin lesions ( 13 ). Here, we present a group of patients with both clinical and histopathological features of dermatomyositis associated with anti-HMGCR autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

Screening for Anti-HMGCR Antibodies in a Large Single Myositis Center Reveals Infrequent Exposure to Statins and Diversiform Presentation of the Disease

et al. 2022

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BackgroundThe objective of this study is to assess the frequency of autoantibodies against 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) in a single center myositis cohort and to analyze associations with statin exposure, clinical features, and outcome of disease course.MethodsA total of 312 patients with idiopathic inflammatory myopathies (IIMs) followed at the rheumatology clinic, Karolinska University Hospital, were identified in the Euromyositis registry between 1988 and 2014 and were classified according to the 2017 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) criteria. Available serum samples were analyzed for anti-HMGCR autoantibodies by ELISA. Positive sera were confirmed by immunoprecipitation. Clinical data were extracted from Euromyositis registry and medical records. Muscle samples were examined by two pathologists blinded to the subjects’ autoantibody status.ResultsOf 312 patients, 13 (4.3%) were positive for anti-HMGCR. Two of the 13 (15%) anti-HMGCR–positive patients had histories of statin use versus 12 (4.2%) in the anti-HMGCR–negative group. In the anti-HMGCR–positive group, five (38%) had a clinical phenotype compatible with dermatomyositis. Muscle biopsies of patients with HMGCR autoantibodies showed findings consistent with immune-mediated necrotizing myopathy in all cases except for one. Five (38%) patients required treatment with intravenous immunoglobulin compared to seven (2.3%) without this antibody. At the last visit, seven patients had chronic, active disease course, and five of 13 patients were in remission, including three without treatment.ConclusionsPatients with IIM related to anti-HMGCR autoantibodies may present with a wide range of symptoms, more than previously anticipated. When a broad approach to screening for these antibodies is applied, only a minority of patients was found to have previous statin exposure. The results of this study justify the addition of anti-HMGCR autoantibodies to routine diagnostic procedures in patients with myositis.

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“…For HMGCR-IMNM patients, the frequency of extramuscular manifestations was low. However, several studies have reported skin conditions in these patients, including Raynaud phenomenon, a nonspecific skin rash and dermatomyositis-like skin eruptions [11,15,26 ▪ –29 ▪ ,50]. HMGCR-IMNM patients with skin conditions usually develop pruritic erythematous papules with photosensitivity (Fig.…”

Section: Clinical Symptoms: Focus On the Difference Between Hmgcr-imn...mentioning

confidence: 99%

“…Interestingly, limb-girdle muscular dystrophy (LGMD) presentation [25] and isolated hyperCKemia [16] were also included in the spectrum of HMGCR-IMNM. In addition, recent studies revealed the small percentage of HMGCR-IMNM patients have dermatological manifestations [26 ▪ –29 ▪ ]. Here, we review manifestations of HMGCR-IMNM, and highlight recent clinical advances in HMGCR-IMNM…”

Section: Introductionmentioning

confidence: 96%

Anti-HMGCR myopathy: clinical and histopathological features, and prognosis

Kurashige

2021

Current Opinion in Rheumatology

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Purpose of reviewThis review aims to describe clinical and pathological features, prognosis and treatment in patients with anti-HMGCR antibody positive immune-mediated necrotizing myopathy (HMGCR-IMNM) based on recent findings.Recent findingsUsing advances in diagnostic modalities that can confirm the presence of anti-HMGCR antibody, the clinical and pathological manifestations of HMGCR-IMNM were found to be broader than previously reported. Although only a small percentage of HMGCR-IMNM patients present with atypical manifestations, some of these patients show slow disease progression and clinical symptoms, which are similar to those of limb-girdle muscular dystrophies. Other atypical HMGCR-IMNM patients have skin conditions similar to dermatomyositis-like skin rush or dermatological presentations of Jessner-Kanoff disease or cutaneous lymphoma, whose pathological changes including CD8-positive and bcl-2-positive lymphocytic accumulations, similar to Jessner-Kanoff lymphocytic infiltration of skin or low-grade cutaneous lymphoma, which are observed in muscle and skin.SummaryAnti-HMGCR autoantibodies define unique populations of IMNM patients. Recent studies have revealed that clinicopathological manifestations of HMGCR-IMNM, especially extramuscular symptoms and pathological manifestations, are more common than previously recognized.

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Anti-HMGCR myopathy overlaps with dermatomyositis-like rash: a distinct subtype of idiopathic inflammatory myopathy

Cited by 15 publications

References 41 publications

Statin-naïve anti–3-hydroxy-3-methylglutaryl coenzyme A reductase antibody-positive necrotizing myopathy with heliotropic pseudoangioedema

Statin-naïve anti–3-hydroxy-3-methylglutaryl coenzyme A reductase antibody-positive necrotizing myopathy with heliotropic pseudoangioedema

Screening for Anti-HMGCR Antibodies in a Large Single Myositis Center Reveals Infrequent Exposure to Statins and Diversiform Presentation of the Disease

Anti-HMGCR myopathy: clinical and histopathological features, and prognosis

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