Anti-nuclear matrix protein 2 antibody-positive inflammatory myopathies represent extensive myositis without dermatomyositis-specific rash

被引：41

作者：

Ichimura, Yuki ^{[1
]}

Konishi, Risa ^{[1
]}

Shobo, Miwako ^{[1
]}

Inoue, Sae ^{[1
]}

Okune, Mari ^{[1
]}

Maeda, Akemi ^{[1
]}

Tanaka, Ryota ^{[1
]}

Kubota, Noriko ^{[1
]}

Matsumoto, Isao ^{[2
]}

Ishii, Akiko ^{[3
]}

Tamaoka, Akira ^{[3
]}

Shimbo, Asami ^{[4
]}

Mori, Masaaki ^{[5
]}

Morio, Tomohiro ^{[4
]}

Kishi, Takayuki ^{[6
,7
]}

Miyamae, Takako ^{[6
,7
]}

Tanboon, Jantima ^{[8
,9
]}

Inoue, Michio ^{[8
,9
]}

Nishino, Ichizo ^{[8
,9
]}

Fujimoto, Manabu ^{[1
,10
]}

Nomura, Toshifumi ^{[1
]}

Okiyama, Naoko ^{[1
]}

机构：

[1] Univ Tsukuba, Fac Med, Dept Dermatol, Tennodai 1-1-1, Tsukuba, Ibaraki, Japan

[2] Univ Tsukuba, Fac Med, Dept Internal Med, Div Rheumatol, Ibaraki, Japan

[3] Univ Tsukuba, Fac Med, Dept Internal Med, Div Neurol, Ibaraki, Japan

[4] Tokyo Med & Dent Univ, Grad Sch Med & Dent Sci, Dept Pediat & Dev Biol, Tokyo, Japan

[5] Tokyo Med & Dent Univ, Grad Sch Med & Dent Sci, Dept Lifetime Clin Immunol, Tokyo, Japan

[6] Tokyo Womens Med Univ, Inst Rheumatol, Tokyo, Japan

[7] Tokyo Womens Med Univ, Dept Pediat, Tokyo, Japan

[8] Natl Ctr Neurol & Psychiat, Natl Inst Neurosci, Dept Neuromuscular Res, Tokyo, Japan

[9] Natl Ctr Neurol & Psychiat, Med Genome Ctr, Tokyo, Japan

[10] Osaka Univ, Grad Sch Med, Dept Dermatol, Course Integrated Med, Osaka, Japan

来源：

RHEUMATOLOGY | 2022年 / 61卷 / 03期

基金：

日本学术振兴会;

关键词：

DM; PM; myositis-specific antibodies; anti-nuclear matrix protein 2 antibody; muscle involvement; skin manifestation; juvenile; adult; AUTOANTIBODIES; ADULT;

D O I：

10.1093/rheumatology/keab518

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Objectives Myositis-specific autoantibodies (MSAs) define distinct clinical subsets of idiopathic inflammatory myopathies (IIMs). The anti-nuclear matrix protein 2 (NXP2) antibody, a MSA detected in juvenile/adult IIMs, has been reported to be associated with a high risk of subcutaneous calcinosis, subcutaneous oedema and internal malignancies. The study aimed to clarify the clinical features of anti-NXP2 antibody-positive IIMs in detail. Methods This was a multicentre retrospective observational study on 76 anti-NXP2 antibody-positive patients. The antibody was detected via a serological assay using immunoprecipitation and western blotting. The patients were selected from 162 consecutive Japanese patients with IIMs. Results The cohort of anti-NXP2 antibody-positive IIMs included 29 juvenile patients and 47 adult patients. Twenty-seven (35.5%) patients presented with polymyositis phenotype without dermatomyositis-specific skin manifestations (heliotrope rash or Gottron sign/papules); this was more common in the adults than children (48.9% vs 15.8%, P < 0.01). Nine (11.8%) patients had subcutaneous calcinosis, and 20 (26.3%) patients had subcutaneous oedema. In addition, the proportion of patients with muscle weakness extending to the distal limbs was high (36 patients [47.4%]) in this cohort. Adult patients had a higher prevalence of malignancy than the general population (age-standardized incidence ratio of malignancies: 22.4). Conclusion Anti-NXP2 antibody-positive IIMs, which include dermatomyositis sine dermatitis, are characterized by atypical skin manifestations and extensive muscular involvement.

引用

页码：1222 / 1227

页数：6

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