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


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  1. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. lorenzo.cavagna@unipv.it
  2. Transplant Centre Unit, IRCCS San Matteo Foundation and University of Pavia, Italy.
  3. Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Hôpitaux Universitaires de Strasbourg, France.
  4. Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy.
  5. UOC Anestesia e Rianimazione 2 Cardiopolmonare, Foundation IRCCS Policlinico S. Matteo, Pavia, Italy.
  6. Rheumatology, University Hospitals, Leuven, Belgium.
  7. Rheumatology, University and AO Spedali Civili, Brescia, Italy.
  8. Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy.
  9. Rheumatology, Centre for Rheumatic Diseases, Bad Kreuznach, Germany.
  10. Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy.
  11. Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy.
  12. Rheumatology, Azienda Ospedaliera Brunico, Italy.
  13. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  14. Internal Interdisciplinary Unit, Lupus Clinic, Careggi University Hospital and Department of Experimental and Clinical Medicine, University of Firenze, Italy.
  15. Neurology, Amsterdam University Medical Centres, The Netherlands.
  16. Neurology, Amsterdam University Medical Centres, The Netherlands.
  17. Department of Experimental and Clinical Medicine, University of Florence, Italy.
  18. Pneumology, NHC, Strasbourg University Hospital, Strasbourg University, France.
  19. Rheumatology, Centro Hospitalar e Universitário de Coimbra, Portugal.
  20. Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy.
  21. Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy.
  22. Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Hôpitaux Universitaires de Strasbourg, France.
  23. Rheumatology, Complejo Hospitalario de Salamanca, Spain.
  24. Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
  25. Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium.
  26. Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium.
  27. Rheumatology, Hospital Clínico Universitario de Santiago de Compostela, Spain.
  28. Rheumatology, Hospital Universitario Ramon y Cajal, Spain.
  29. Rheumatology, Hospital Universitario Rio Hortega, Valladolid, Spain.
  30. Rheumatology, Hospital Universitario San Augustin, Aviles, Spain.
  31. Rheumatology, HPMetz, Hopital Belle-Ile, Metz, France.
  32. Pneumology HPMetz, Hopital Belle-Ile, Metz, France.
  33. Rheumatology, Internal Medicine Department, Hospital Universitario Son Llàtzer, Palma de Mallorca, Spain.
  34. Rheumatology, Johann Wolfgang Goethe-Universität, Frankfurt, Germany.
  35. Rheumatology, Johann Wolfgang Goethe-Universität, Frankfurt, Germany.
  36. Rheumatology, Johannes Gutenberg-University, Mainz, Germany.
  37. Rheumatology, Ospedale Guglielmo da Saliceto, Piacenza, Italy.
  38. Rheumatology, Ospedale San Camillo, Roma, Italy.
  39. Rheumatology, Ospedale San Camillo, Roma, Italy.
  40. Rheumatology, Prato Hospital, Italy.
  41. Rheumatology Unit, Department of Medicine (DAME), University of Udine, Academic Hospital “Santa Maria della Misericordia”, Udine, Italy.
  42. Rheumatology, Severo Ochoa Hospital, Madrid, Spain.
  43. Rheumatology, Severo Ochoa Hospital, Madrid, Spain.
  44. Rheumatology, Sierralana Hospital, Torrellavega, Spain.
  45. Rheumatology, Skane University Hospital, Sweden.
  46. Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
  47. Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
  48. Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
  49. Rheumatology, University Hospital J. Dìaz, Madrid, Spain.
  50. Rheumatology, University and AO Spedali Civili, Brescia, Italy.
  51. Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy.
  52. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  53. Pneumology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  54. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  55. Rheumatology, University of Genova, DIMI, IRCCS San Martino, Genova, Italy.
  56. Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of Cagliari, Italy.
  57. Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of Cagliari, Italy.
  58. Internal Medicine, Hospital Clínic, Barcelona, Spain.
  59. Rheumatology, University Hospital Lisboa Norte, Portugal.
  60. Serviço Reumatologia, Centro Hospitalar Lisboa Norte and Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Portugal.
  61. Rheumatology, University Hospital, Alicante, Spain.
  62. Rheumatology, University La Sapienza and Policlinico Umberto I, Rome, Italy.
  63. Scleroderma Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
  64. Pneumology, Mater Dei Hospital, Msida, Malta.
  65. Pneumology, Mater Dei Hospital, Msida, Malta.
  66. Rheumatology, Azienda Ospedaliero-Universitaria di Sassari, Italy.
  67. Rheumatology, University of Perugia, Italy.
  68. Rheumatology, University of Perugia, Italy.
  69. Biostatistics Unit, Department of Public Health, University of Pavia, Italy.
  70. Rheumatology, University of Heidelberg, Germany.
  71. Artroreuma srl, Outpatient of Rheumatology accredited with the Italian National Health System, Mascalucia, Catania, Italy.
  72. Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy.
  73. Rheumatology, Charité - Universitätsmedizin Berlin, Germany.
  74. Pneumology, Hospital Universitario de la Princesa, IIS-Princesa, Cátedra UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain.
  75. Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
  76. Pneumology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
  77. Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, Mexico.
  78. Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, Mexico.
  79. Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, Freiburg, Germany.
  80. Rheumatology, University of Bari, Italy.
  81. Rheumatology, Università Vita-Salute, San Raffaele, Milano, Italy
  82. Rheumatology, University of Verona, Italy.
  83. Rheumatology, University of Verona, Italy.
  84. Rheumatology, Hôpital Bichat-Claude Bernard, Université Paris Diderot, Paris, France.
  85. Rheumatology, University of Erlangen, Germany.
  86. Rheumatology, Hospital Universitario de la Princesa, IIS-Princesa, Cátedra UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain.
  87. Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, Freiburg, Germany.
  88. Rheumatology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México.
  89. Radiology, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  90. Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy.
  91. Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy.
  92. Rheumatology, Charité - Universitätsmedizin Berlin, Germany.
  93. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  94. Rheumatology, University of Bari, Italy.
  95. Rheumatology, University of Erlangen, Germany.
  96. Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
  97. Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.

on behalf of the AENEAS collaborative group

CER15088
2022 Vol.40, N°2
PI 0274, PF 0283
Full Papers

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PMID: 35200123 [PubMed]

Received: 21/08/2021
Accepted : 13/10/2021
In Press: 16/02/2022
Published: 25/02/2022

Abstract

OBJECTIVES:
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.
CONCLUSIONS:
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.

DOI: https://doi.org/10.55563/clinexprheumatol/di1083

Rheumatology Article

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