Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study

Yongchairat, Khemmapop; Tanboon, Jantima; Waisayarat, Jariya; Narongroeknawin, Pongthorn; Chevaisrakul, Parawee; Dejthevaporn, Charungthai; Katchamart, Wanruchada

doi:10.1007/s10067-019-04756-2

Cited by 6 publications

(6 citation statements)

References 26 publications

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“…A previous study reported that CK levels in IMNM are always up to 10-15 times the upper normal level, although in LGMD 2B, it can increase more than 20 times. The study also shows that signi cant CK elevation indicates a higher probability of muscular dystrophy than IMNM [22]. The level of LDH in LSM patients can reach 808 (341, 1248) U/L, with the highest value of up to 2433 U/L, which is higher than that in the other groups.…”

Section: Clinical and Pathological Characteristics Of Imnm In The Iim Groupmentioning

confidence: 71%

Clinical and Pathological Features of Immune-Mediated Necrotising Myopathies in a Single-Centre Muscle Biopsy Cohort

Yang

Tian

Zhang

et al. 2022

Preprint

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Objective Immune-mediated necrotising myopathy (IMNM) is a recently entitled novel subset of idiopathic inflammatory myopathies (IIM) characterized by significant elevated creatine kinase (CK) level, muscle weakness and predominant muscle fibre necrosis in muscle biopsy. This study aimed to investigate the clinical and pathological characteristics of patients with IMNM in our single-centre muscle biopsy cohort. Methods A total of 860 patients who had muscle biopsy reports in our centre from May 2008 to December 2017 were enrolled in this study. IMNM was diagnosed in according with 2018 European Neuromuscular Centre (ENMC) clinicopathological diagnostic criteria for IMNM. Results The muscle biopsy cohort consisted of 531 patients with IIM (61.7%), 253 patients with non-IIM (29.4%), and 76 undiagnosed patients (8.8%). Among IIM patients, polymyositis (PM), dermatomyositis(DM), amyopathic dermatomyositis, juvenile DM, and inclusion body myositis were 182(21.2%), 236(27.4%), 83(9.7%), 18(2.1%) and 3(0.3%), respectively. In PM subgroup, 59 patients met serological and pathological characteristics of IMNM according to 2018 ENMC criteria including 29 anti-SRP-positive patients,10 anti-HMGCR-positive patients and 20 MSA-negative patients. Limb girdle muscular dystrophy (LGMD) 2B and lipid storage myopathy (LSM) were 29 and 16 respectively, which present similar manifestations of IMNM with elevated CK levels and muscle weakness among non-IIM group. IMNM patients had older age of onset (mean: 42.25 vs 21.66 and 24.56, p<0.0001), shorter duration of diseases (mean: 22.56 vs 66.69 and 48.94, p<0.0001) and more frequent of dysphagia (33.9% vs 3.4% and 6.3%, p<0.0001) compare to patients with LGMD 2B and LSM. Muscle biopsy from IMNM patients showed frequent muscle fibre necrosis (96.6% vs 72.4% and 56.3%, p<0.0001), overexpression of MHC-I on sarcolemma (81.4% vs 37.9% and 12.9%, p<0.0001) and CD4+ T cell endomysial infiltration (89.9% vs 53.6% and 50%, p<0.0001) compared with LGMD 2B and LSM patients. Conclusions It is easy to distinguish IMNM from other subtype of IIM according to clinical symptoms and MSAs profiles. However, distinguishing IMNM from disorders clinically similar non-IIM need to combine with clinical, serological and pathological features.

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Section: Clinical and Pathological Characteristics Of Imnm In The Iim Groupmentioning

confidence: 71%

Clinical and Pathological Features of Immune-Mediated Necrotising Myopathies in a Single-Centre Muscle Biopsy Cohort

Yang

Tian

Zhang

et al. 2022

Preprint

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“…On the contrary, in statin‐associated MG the time interval from the initiation of statins to the onset of MG ranges from 6 months to 6 years 13 . The most likely hypothesis to account for the coexistence of both NAM and MG in our patient is based on the immunomodulatory effects of statins, 5,18 which may trigger an increased immunogenicity to neuromuscular peptides in subjects with distinctive HLA haplotypes. These drugs are recognized to induce other autoimmune disorders such as a lupus‐like syndrome, myositis, and immune hepatitis 19 .…”

Section: Discussionmentioning

confidence: 94%

“…Serum myositis‐specific autoantibodies associated with NAM include anti‐3‐hydroxy‐3‐methylglutarylcoenzyme‐A reductase (HMGCR) and antisignal recognition particle (SRP) 4 . Anti‐HMGCR myopathy is characterized by severe, rapidly progressive proximal muscle weakness along with dysphagia in 16%‐30% of the patients, and elevated serum CK levels 5,6 . Muscle biopsy shows necrosis with scarce or no inflammation.…”

Section: Introductionmentioning

confidence: 99%

“…4 Anti-HMGCR myopathy is characterized by severe, rapidly progressive proximal muscle weakness along with dysphagia in 16%-30% of the patients, and elevated serum CK levels. 5,6 Muscle biopsy shows necrosis with scarce or no inflammation. In most patients with a history of statin exposure, symptoms do not resolve after withdrawing the drug, and only a prompt immunosuppressive treatment leads to clinical improvement.…”

Section: Introductionmentioning

confidence: 99%

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Statin‐associated necrotizing autoimmune myopathy with concurrent myasthenia gravis

Frasson¹,

Simonetto²,

Bertolasi

et al. 2021

Clinical Case Reports

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“…11,14 Skin manifestations occur in less than 10% of patients with IMNM, 2 including heliotrope rash, Gottron papules, V-sign, Shawl sign, Mechanic's hands, and skin hyperpigmentation. [15][16][17] Erythema nodosum (EN) is a common form of acute nodular septal panniculitis without signs of vasculitis. [18][19][20][21][22] It is clinically characterized by bilaterally symmetrical red and painful subcutaneous nodules, mainly localized to the lower extremities, pretibial areas especially.…”

Section: Introductionmentioning

confidence: 99%

<p>Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum</p>

Ying¹,

Li²,

Tang³

et al. 2020

JIR

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Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by severe diffuse proximal myofiber necrosis in the context of inflammatory myopathy. Autoantibodies of anti-signal recognition particle and anti-hydroxy-3-methylglutaryl-CoA reductase are two antibodies specific to IMNM. Erythema nodosum (EN) is often accompanied by various systemic diseases, such as autoimmune diseases. Herein, we report a female patient with signal recognition particle-associated IMNM, with EN as the first presentation. She showed significant clinical improvement after the initiation of glucocorticoids, intravenous immunoglobulin, rituximab, and mycophenolate mofetil. This case indicates that IMNM can initially present as EN. IMNM and EN might have overlapping pathogeneses.

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Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study

Cited by 6 publications

References 26 publications

Clinical and Pathological Features of Immune-Mediated Necrotising Myopathies in a Single-Centre Muscle Biopsy Cohort

Clinical and Pathological Features of Immune-Mediated Necrotising Myopathies in a Single-Centre Muscle Biopsy Cohort

Statin‐associated necrotizing autoimmune myopathy with concurrent myasthenia gravis

<p>Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum</p>

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