Immune-mediated necrotizing myopathies are serologically heterogeneous and autoantibodies may predict their clinical phenotype: two cases associated with anti-Pl7 antibodies

Meyer, Abbie L.; Messer, Laurent; Goetz, Joëlle; Lannes, Béatrice; Jc, Weber; Geny, Benard; Je, Gottenberg; Sibilia, Jean

doi:10.3109/03009742.2013.864421

Cited by 6 publications

(6 citation statements)

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“…The most common histopathologic categories on muscle biopsy were non-specific myositis (38.5%) and a necrotizing myopathy (21.4%). Of note, the presence of a necrotizing myopathy is common in patients with immune-mediated myopathies associated with anti-SRP and anti-HMGCR autoantibodies and has also been reported in the antisynthetase syndrome (30,31). Our findings highlight that a necrotizing muscle biopsy is relatively non-specific and may be found in scleroderma as well as other autoimmune myopathies.…”

Section: Discussionmentioning

confidence: 94%

Spectrum of Muscle Histopathologic Findings in Forty‐Two Scleroderma Patients With Weakness

Paik

Wigley

Lloyd

et al. 2015

Arthritis Care & Research

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Objective To determine if distinct muscle pathological features exist in scleroderma subjects with weakness. Methods This retrospective study included weak scleroderma subjects with muscle biopsies available for review. Biopsies were systematically assessed for individual pathologic features including inflammation, necrosis, fibrosis, and acute neurogenic atrophy. Based on the aggregate individual features, biopsies were assigned a histopathologic category of polymyositis, dermatomyositis, necrotizing myopathy, non-specific myositis, “acute denervation”, “fibrosis only”, or “other”. Clinical data analyzed included autoantibody profiles, scleroderma subtype and disease duration, Medsger muscle severity scores, creatine kinase (CK), electromyography (EMG), and muscle magnetic resonance imaging (MRI). Results 42 subjects (79% female and 64% diffuse scleroderma) were included in this study. Necrosis (67%), inflammation (48%), acute neurogenic atrophy (48%), and fibrosis (33%) were the most prevalent pathologic features. The presence of fibrosis was strongly associated with anti-PM-Scl antibodies. Histopathologic categories included non-specific myositis (36%), necrotizing myopathy (21%), dermatomyositis (7%), “acute denervation” (7%), “fibrosis only” (7%), and polymyositis (5%). Disease duration of scleroderma at the time of muscle biopsy was shorter in polymyositis than other histopathologic categories. Patients with anti-PM-Scl and Scl-70 antibodies also had a shorter disease duration than those with other auto-antibody profiles. Conclusion Non-specific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histological evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of myopathy in scleroderma.

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Section: Discussionmentioning

confidence: 94%

Spectrum of Muscle Histopathologic Findings in Forty‐Two Scleroderma Patients With Weakness

Paik

Wigley

Lloyd

et al. 2015

Arthritis Care & Research

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“…For example, in a series of 38 patients with a necrotizing myopathy on muscle biopsy, there were 16 (42%) anti-HMGCR, six (16%) anti-SRP, two (5%) anti-PL-12, one (3%) anti-PL-7, and one (3%) anti-Jo-1-positive patients [13]. Subsequent studies have confirmed antisynthetase syndrome patients may present with necrotizing muscle biopsies [19,20]. Interestingly, Bhansing et al [21 && ] recently reported that among 24 patients with scleroderma myopathy, four (17%) had muscle fiber necrosis without inflammation.…”

Section: The Plot Thickens mentioning

confidence: 87%

Necrotizing myopathies

Mammen

2014

Current Opinion in Rheumatology

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A significant number of patients with autoimmune myopathy have a predominantly necrotizing muscle biopsy with minimal lymphocytic infiltration. This biopsy finding occurs in various forms myositis, including the antisynthetase syndrome, scleroderma-associated myopathy, antisignal recognition particle-associated myopathy, statin-associated anti-HMG-CoA reductase-positive autoimmune myopathy, and statin-naïve anti-HMG-CoA reductase-positive myopathy. Future progress in elucidating pathogenic mechanisms and defining optimal treatment strategies may depend upon recognizing these distinct forms of myositis and analyzing them as separate entities.

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“…Most patients had inflammatory macrophagic infiltrates, which were predominantly located in the endomysium area, and they had a significant presence of fiber muscle necrosis, proposing a necrotising myopathy pattern. Despite the fact that this pattern is classically described in patients with immune-mediated necrotising myopathies (12), some studies have provided indirect evidence that ASSD may have a necrotising pattern in muscle biopsy (6)(7)(8)(9)22). Classically, signs and symptoms may help to differentiate between both dis- Although both diseases (IMNM and ASSD) may present a predominantly necrotising pattern in muscle biopsy, the presence of inflammation per se seems to be more prominent in ASSD than IMNM (10).…”

Section: Discussionmentioning

confidence: 99%

“…Until now, a well-established definition and validated criteria of a histological pattern for ASSD have not existed. Scarce case reports have shown a diffuse necrosis in muscle biopsies among patients with anti-ARS positive autoantibodies (6)(7)(8)(9). However, these studies have not given details as to which criteria were used to classify these patients as having ASSD, clinical and laboratory manifestations, and/or defined exclusion criteria.…”

Section: Introductionmentioning

confidence: 99%

High prevalence of necrotising myopathy pattern in muscle biopsies of patients with anti-Jo-1 antisynthetase syndrome

Silva

Borges

Shinjo

2022

Clinical and Experimental Rheumatology

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ObjetiveUntil now, researchers have not provided a well-defined muscle histological pattern for antisynthetase syndrome (ASSD).Therefore, we aimed to analyse the muscle biopsies of patients with anti-Jo-1 ASSD. Methods This study included 26 patients with anti-Jo-1 ASSD admitted for investigation of the disease and obligatorily with muscleimpairment, from 2010 to 2021, whose serial frozen muscle sections were analysed. ResultsPatients' mean age at disease diagnosis was 42.8±11.6 years, and the female gender was most predominant. Concerning muscle biopsies, cell infiltrates were present in 76.9% of the samples, and they were mainly located at the endomysium area (70%), with a predominance of macrophages (92.9%). Fiber muscle necrosis was present in 92.3% and was diffused in 54.2%. Expression of MHC-I was seen in all samples. Samples were mostly marked by the presence of CD68+ and discreet/ low CD4 + and CD8 + staining, which is consistent with a higher predominance of observed necrosis and macrophage cell infiltrates. In general, 38.5% of patients had a necrotising myopathy pattern in muscle biopsies, whereas 34.6% and 26.9% had a general inflammatory myopathy pattern and nonspecific myopathy, respectively. This necrotising myopathy pattern was not associated with the demographic, clinical, or laboratory data. ConclusionOur data show that almost 40% of patients with well-defined anti-Jo-1 ASSD with objective muscle impairment have a necrotising myopathy pattern in their muscle biopsies. Although this pattern is more classically related to immune-mediated necrotising myopathies, in association with clinical manifestations and the presence of anti-Jo-1 autoantibodies, this characteristic may lead to ASSD diagnosis.

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Immune-mediated necrotizing myopathies are serologically heterogeneous and autoantibodies may predict their clinical phenotype: two cases associated with anti-Pl7 antibodies

Cited by 6 publications

References 9 publications

Spectrum of Muscle Histopathologic Findings in Forty‐Two Scleroderma Patients With Weakness

Spectrum of Muscle Histopathologic Findings in Forty‐Two Scleroderma Patients With Weakness

Necrotizing myopathies

High prevalence of necrotising myopathy pattern in muscle biopsies of patients with anti-Jo-1 antisynthetase syndrome

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