PDF(Pubmed)
Abstract：
UNASSIGNED: Idiopathic inflammatory myopathies (IIM), also called autoimmune myositis, are heterogeneous. These include dermatomyositis (DM), inclusion body myositis, immune mediated necrotizing myopathy (IMNM), anti-synthetase syndrome (ASS), and overlap polymyositis. Classification of IIM has evolved from clinical to clinico-pathologic to the recent clinico-sero-pathologic with the discovery of myositis-specific antibodies (MSA) and myositis-associated antibodies. The various antibodies have shown association with specific phenotypes.
UNASSIGNED: To analyze muscle biopsy features with respect to each MSA and MAA to understand the frequency of findings in each entity.
UNASSIGNED: Biopsy-proven cases of IIM where myositis profile was available were included in the study after obtaining Institutional Ethics Committee (IEC) approval. In addition to the stains and enzyme histochemistry, immunohistochemistry with MHC class I and II and MxA was performed. Features like perifascicular atrophy, perifascicular necrosis, scattered necrosis, inflammation, etc. were analyzed. Myositis profile was performed by line-blot technique using a 16-antigen panel. Cases were divided into different autoantibody subgroups. Various clinical, demographic, and muscle biopsy features were studied with respect to each MSA and MAA.
UNASSIGNED: There were a total of 64 cases. Mi2 (N = 18) was the most common autoantibody. Some of the salient observations included PFA with perivascular inflammation in Mi2; pediatric cases and microinfarcts in NXP2; no PFA or inflammation in MDA5; perifascicular necrosis in JO1; extensive necrosis with sparse inflammation in SRP; more inflammation in overlap myositis; MxA positivity in DM; and absent in ASS.
UNASSIGNED: This is a pilot study documenting differences in biopsy phenotype with each MSA and MAA which is comparable to the literature. These findings can be used to characterize IIM in seronegative biopsies.
UNASSIGNED: To analyze muscle biopsy features with respect to each MSA and MAA to understand the frequency of findings in each entity.
UNASSIGNED: Biopsy-proven cases of IIM where myositis profile was available were included in the study after obtaining Institutional Ethics Committee (IEC) approval. In addition to the stains and enzyme histochemistry, immunohistochemistry with MHC class I and II and MxA was performed. Features like perifascicular atrophy, perifascicular necrosis, scattered necrosis, inflammation, etc. were analyzed. Myositis profile was performed by line-blot technique using a 16-antigen panel. Cases were divided into different autoantibody subgroups. Various clinical, demographic, and muscle biopsy features were studied with respect to each MSA and MAA.
UNASSIGNED: There were a total of 64 cases. Mi2 (N = 18) was the most common autoantibody. Some of the salient observations included PFA with perivascular inflammation in Mi2; pediatric cases and microinfarcts in NXP2; no PFA or inflammation in MDA5; perifascicular necrosis in JO1; extensive necrosis with sparse inflammation in SRP; more inflammation in overlap myositis; MxA positivity in DM; and absent in ASS.
UNASSIGNED: This is a pilot study documenting differences in biopsy phenotype with each MSA and MAA which is comparable to the literature. These findings can be used to characterize IIM in seronegative biopsies.
摘要：
■特发性炎性肌病（IIM），也被称为自身免疫性肌炎，是异质的。这些包括皮肌炎（DM），包涵体肌炎，免疫介导性坏死性肌病（IMNM），抗合成酶综合征（ASS），与多发性肌炎重叠。随着肌炎特异性抗体（MSA）和肌炎相关抗体的发现，IIM的分类已从临床发展到临床病理学，再到最近的临床血清病理学。各种抗体已显示与特定表型的关联。
■分析每个MSA和MAA的肌肉活检特征，以了解每个实体中发现的频率。
■在获得机构伦理委员会（IEC）批准后，将活检证实的肌炎病例纳入研究。除了染色和酶组织化学，使用MHCI类和II类以及MxA进行免疫组织化学。像束状周围萎缩，束状周围坏死，散见性坏死,炎症,等。进行了分析。使用16抗原组通过线印迹技术进行肌炎概况。病例分为不同的自身抗体亚组。各种临床，人口统计学,并对每种MSA和MAA进行了肌肉活检特征研究。
■共64例。Mi2(N=18)是最常见的自身抗体。一些重要的观察结果包括Mi2中PFA伴血管周围炎症；NXP2中的儿科病例和微梗塞；MDA5中没有PFA或炎症；JO1中的束状坏死；SRP中广泛坏死伴稀疏炎症；重叠肌炎中炎症更多；DM中MxA阳性；ASS中不存在。
■这是一项初步研究，记录了每种MSA和MAA的活检表型差异，与文献相当。这些发现可用于表征血清阴性活检中的IIM。
■分析每个MSA和MAA的肌肉活检特征，以了解每个实体中发现的频率。
■在获得机构伦理委员会（IEC）批准后，将活检证实的肌炎病例纳入研究。除了染色和酶组织化学，使用MHCI类和II类以及MxA进行免疫组织化学。像束状周围萎缩，束状周围坏死，散见性坏死,炎症,等。进行了分析。使用16抗原组通过线印迹技术进行肌炎概况。病例分为不同的自身抗体亚组。各种临床，人口统计学,并对每种MSA和MAA进行了肌肉活检特征研究。
■共64例。Mi2(N=18)是最常见的自身抗体。一些重要的观察结果包括Mi2中PFA伴血管周围炎症；NXP2中的儿科病例和微梗塞；MDA5中没有PFA或炎症；JO1中的束状坏死；SRP中广泛坏死伴稀疏炎症；重叠肌炎中炎症更多；DM中MxA阳性；ASS中不存在。
■这是一项初步研究，记录了每种MSA和MAA的活检表型差异，与文献相当。这些发现可用于表征血清阴性活检中的IIM。
