Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

被引：0

作者：

Cavagna, L. ^{[1
,2
]}

Meloni, F. ^{[3
,4
]}

Meyer, A. ^{[5
]}

Sambataro, G. ^{[6
]}

Belliato, M. ^{[7
]}

De Langhe, E. ^{[8
]}

Cavazzana, I ^{[9
,10
]}

Pipitone, N. ^{[11
]}

Triantafyllias, K. ^{[12
]}

Mosca, M. ^{[13
]}

Barsotti, S. ^{[13
]}

Zampogna, G. ^{[14
]}

Biglia, A. ^{[1
,2
]}

Emmi, G. ^{[15
,16
]}

de Visser, M. ^{[17
]}

van der Kooi, A. ^{[17
]}

Parronchi, P. ^{[18
]}

Hirschi, S. ^{[19
]}

da Silva, J. A. P. ^{[20
]}

Scire, C. A. ^{[21
]}

Furini, F. ^{[21
]}

Giannini, M. ^{[5
]}

Martinez Gonzalez, O. ^{[22
]}

Damian, L. ^{[23
]}

Piette, Y. ^{[24
,25
,26
]}

Smith, V ^{[24
,25
,26
]}

Mera-Varela, A. ^{[27
]}

Bachiller-Corral, J. ^{[28
]}

Cabezas Rodriguez, I ^{[29
]}

Brandy-Garcia, A. M. ^{[30
]}

Maurier, F. ^{[31
]}

Perrin, J. ^{[32
]}

Gonzalez-Moreno, J. ^{[33
]}

Drott, U. ^{[34
]}

Delbruck, C. ^{[34
]}

Schwarting, A. ^{[35
]}

Arrigoni, E. ^{[36
]}

Sebastiani, G. D. ^{[37
]}

Iuliano, A. ^{[37
]}

Nannini, C. ^{[38
]}

Quartuccio, L. ^{[39
]}

Rodriguez Cambron, A. B. ^{[40
]}

Blazquez Canamero, M. A. ^{[40
]}

Blanco, I. Villa ^{[41
]}

Cagnotto, G. ^{[42
]}

Pesci, A. ^{[43
]}

Luppi, F. ^{[43
]}

Dei, G. ^{[43
]}

Romero Bueno, F., I ^{[44
]}

Franceschini, F. ^{[9
,10
]}

机构：

[1] Univ Pavia, Rheumatol, Via Golgi 19, I-27100 Pavia, Italy

[2] IRCCS Policlin S Matteo Fdn, Via Golgi 19, I-27100 Pavia, Italy

[3] IRCCS San Matteo Fdn, Transplant Ctr Unit, Pavia, Italy

[4] Univ Pavia, Pavia, Italy

[5] Hop Univ Strasbourg, Ctr Reference Malad Autoimmunes Rares, Serv Physiol & Explorat Fonctionnelles Musculaire, Rheumatol, Rheumatology, France

[6] Azienda Osped Univ Catania, Pulmonol, Catania, Italy

[7] Fdn IRCCS Policlin S Matteo, UOC Anestesia & Rianimazione Cardiopolmonare 2, Pavia, Italy

[8] Univ Hosp, Rheumatol, Leuven, Belgium

[9] Univ Brescia, Rheumatol, Brescia, Italy

[10] AO Spedali Civili, Brescia, Italy

[11] S Maria Hosp IRCCS, Rheumatol, Reggio Emilia, Italy

[12] Ctr Rheumat Dis, Rheumatol, Bad Kreuznach, Germany

[13] Azienda Osped Univ Pisana, Rheumatol, Pisa, Italy

[14] Azienda Osped Brunico, Rheumatol, Brunico, Italy

[15] Univ Firenze, Careggi Univ Hosp, Internal Interdisciplinary Unit, Lupus Clin, Florence, Italy

[16] Univ Firenze, Dept Expt & Clin Med, Florence, Italy

[17] Amsterdam Univ Med Ctr, Neurol, Amsterdam, Netherlands

[18] Univ Florence, Dept Expt & Clin Med, Florence, Italy

[19] Strasbourg Univ, Strasbourg Univ Hosp, NHC, Pneumol, Strasbourg, France

[20] Ctr Hosp & Univ Coimbra, Rheumatol, Coimbra, Portugal

[21] Azienda Osped Univ S Anna, Rheumatol, Ferrara, Italy

[22] Complejo Hosp Salamanca, Rheumatol, Salamanca, Spain

[23] Emergency Cty Teaching Hosp, Rheumatol, Cluj Napoca, Romania

[24] Ghent Univ Hosp, Dept Rheumatol, Ghent, Belgium

[25] Univ Ghent, Dept Internal Med, Ghent, Belgium

[26] VIB Inflammat Res Ctr IRC, Unit Mol Immunol & Inflammat, Ghent, Belgium

[27] Hosp Clin Univ Santiago de Compostela, Rheumatol, Santiago, Spain

[28] Hosp Univ Ramon y Cajal, Rheumatol, Madrid, Spain

[29] Hosp Univ Rio Hortega, Rheumatol, Valladolid, Spain

[30] Hosp Univ San Augustin, Rheumatol, Aviles, Spain

[31] Hop Belle Ile, HPMetz, Rheumatol, Metz, France

[32] Hop Belle Ile, HPMetz, Pneumol, Metz, France

[33] Hosp Univ Son Llatzer, Internal Med Dept, Rheumatol, Palma De Mallorca, Spain

[34] Goethe Univ Frankfurt, Rheumatol, Frankfurt, Germany

[35] Johannes Gutenberg Univ Mainz, Rheumatol, Mainz, Germany

[36] Osped Guglielmo Saliceto, Rheumatol, Piacenza, Italy

[37] Osped San Camillo, Rheumatol, Rome, Italy

[38] Prato Hosp, Rheumatol, Prato, Italy

[39] Univ Udine, Acad Hosp Santa Maria della Misericordia, Dept Med DAME, Rheumatol Unit, Udine, Italy

[40] Severo Ochoa Hosp, Rheumatol, Madrid, Spain

[41] Sierralana Hosp, Rheumatol, Torrellavega, Spain

[42] Skane Univ Hosp, Rheumatol, Malmo, Sweden

[43] Univ Milano Bicocca, San Gerardo Hosp, Pneumol, Monza, Italy

[44] Univ Hosp J Diaz, Rheumatol, Madrid, Spain

[45] Univ Pavia, Pneumol, Pavia, Italy

[46] Univ Genoa, IRCCS San Martino, DIMI, Rheumatol, Genoa, Italy

[47] Univ Clin Cagliari, Rheumatol, Cagliari, Italy

[48] Azienda Osped Univ Cagliari, Cagliari, Italy

[49] Hosp Clin Barcelona, Internal Med, Barcelona, Spain

[50] Univ Hosp Lisboa Norte, Rheumatol, Lisbon, Portugal

来源：

CLINICAL AND EXPERIMENTAL RHEUMATOLOGY | 2022年 / 40卷 / 02期

关键词：

melanoma differentiation-associated protein 5 antibody; rapidly progressive interstitial lung diseases; idiopathic inflammatory myopathies; INTERSTITIAL LUNG-DISEASE; GENE; 5; ANTIBODY; IDIOPATHIC INFLAMMATORY MYOPATHIES; CLASSIFICATION CRITERIA; DERMATOMYOSITIS; COLLEGE; UTILITY; LEAGUE; VIRUS;

D O I：

暂无

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

引用

页码：274 / 283

页数：10

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