Anti-PM-Scl antibodies-positive patients encompass three different groups with distinct prognoses

被引：10

作者：

Breillat, Paul ^{[1
,2
]}

Mariampillai, Kuberaka ^{[3
]}

Legendre, Paul ^{[4
]}

Martins, Pauline ^{[5
]}

Dunogue, Bertrand ^{[1
]}

Charuel, Jean Luc ^{[6
]}

Miyara, Makoto ^{[6
]}

Goulvestre, Claire ^{[7
]}

Paule, Romain ^{[8
]}

Vanquaethem, Helene ^{[9
]}

Ackermann, Felix ^{[8
]}

Benveniste, Olivier ^{[3
,10
]}

Nunes, Hilario ^{[11
]}

Mouthon, Luc ^{[1
]}

Allenbach, Yves ^{[3
,10
]}

Uzunhan, Yurdagul ^{[11
]}

机构：

[1] Hop Cochin, AP HP, Ctr Reference Malad Autoimmunes Rares, Dept Med Interne, Paris, France

[2] Sorbonne Univ, Paris, France

[3] Sorbonne Univ, Ctr Rech Myol, Assoc Inst Myol, INSERM,UMRS 974, Paris, France

[4] Ctr Hosp Mans, Dept Immunol Clin, Le Mans, France

[5] Hop Rochelle Re Aunis, Dept Med Interne, La Rochelle, France

[6] Grp Hosp Pitie Salpetriere, AP HP, Lab Immunochim, Dept Immunol, Paris, France

[7] Grp Hosp Cochin, AP HP, Lab Immunol, Paris, France

[8] Hop Foch, Dept Med Interne, Suresnes, France

[9] Hop Instruct Armees Begin, Clin Med, Dept Med Interne, St Mande, France

[10] Grp Hosp Pitie Salpetriere, AP HP, Ctr Reference Malad Neuromusculaires, Dept Med Interne & Immunol Clin, Paris, France

[11] Univ Sorbonne Paris Nord, Hop Avicenne, AP HP, Dept Pneumol,INSERM U1272, Bobigny, France

来源：

RHEUMATOLOGY | 2023年 / 62卷 / 04期

关键词：

SSc; idiopathic inflammatory myopathies; anti-PM-Scl antibodies; cluster study; INTERSTITIAL LUNG-DISEASE; CONNECTIVE-TISSUE DISEASE; CLASSIFICATION CRITERIA; SYSTEMIC-SCLEROSIS; NUCLEOLAR ANTIGEN; RHEUMATOLOGY/EUROPEAN LEAGUE; PM/SCL ANTIBODIES; AMERICAN-COLLEGE; AUTOANTIBODIES; AUTOANTIGEN;

D O I：

10.1093/rheumatology/keac508

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Objective To help identify homogeneous subgroups among patients with anti-PM-scleroderma-antibodies (PM-Scl-Abs) positive auto-immune diseases regardless of diagnostic classifications. Material and methods This multicentric (four hospitals) retrospective study collected all consecutive patients (from 2011 to 2021) with positive testing for anti-PM-Scl-Abs in a context of CTD. Subgroups of patients with similar clinico-biological phenotypes were defined using unsupervised multiple correspondence analysis and hierarchical clustering analysis of the features recorded in the first year of follow-up. Results One hundred and forty-two patients with anti-PM-Scl-Abs were evaluated and 129 patients were included in the clustering analysis and divided into three clusters. Cluster 1 (n = 47) included patients with frequent skin thickening, digestive involvement and interstitial lung disease (ILD) with non-specific interstitial pneumonia (NSIP). They were more likely to develop progressive fibrosing ILD. Cluster 2 (n = 36) included patients who all featured NSIP with frequent organizing pneumonia-associated pattern and mechanic's hands. This subgroup had increased risk of relapse and ILD was characterized by a good functional outcome. Cluster 3 (n = 46) was characterized by predominant or isolated musculoskeletal involvement and frequently matched UCTD criteria. Although very frequent among anti-PM-Scl-Abs positive patients, muscle involvement was less discriminating compared with skin thickening and ILD pattern to classify patients into subgroups. Conclusion Anti-PM-Scl-Abs associated auto-immune diseases are segregated into three subgroups with distinct clinical phenotype and outcomes. Skin thickening and NSIP are determinant predictors in segregation of theses populations.

引用

页码：1467 / 1475

页数：9

共 40 条

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