Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies-Reference-Cited by-同舟云学术

Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies

Published:2022-04-06 Issue:1 Volume:62 Page:243-255
ISSN:1462-0324
Container-title:Rheumatology
language:en
Short-container-title:

Author:

Hernández-Molina Gabriela¹^ORCID,Kostov Belchin²³,Brito-Zerón Pilar⁴,Vissink Arjan⁵,Mandl Thomas⁶,Hinrichs Anneline C⁷,Quartuccio Luca⁸,Baldini Chiara⁹^ORCID,Seror Raphaele¹⁰,Szántó Antonia¹¹,Isenberg David¹²^ORCID,Gerli Roberto¹³^ORCID,Nordmark Gunnel¹⁴^ORCID,Rasmussen Astrid¹⁵,Solans-Laque Roser¹⁶,Hofauer Benedikt¹⁷,Sène Damien¹⁸,Pasoto Sandra G¹⁹,Rischmueller Maureen²⁰,Praprotnik Sonja²¹,Gheita Tamer A²²^ORCID,Danda Debashish²³,Armağan Berkan²⁴^ORCID,Suzuki Yasunori²⁵,Valim Valeria²⁶,Devauchelle-Pensec Valerie²⁷,Retamozo Soledad²⁸²⁹³⁰,Kvarnstrom Marika³¹³²,Sebastian Agata³³,Atzeni Fabiola³⁴,Giacomelli Roberto³⁵,Carsons Steven E³⁶,Kwok Seung-Ki³⁷,Nakamura Hideki³⁸,Fernandes Moça Trevisani Virginia³⁹,Flores-Chávez Alejandra⁴⁰⁴¹,Mariette Xavier¹⁰,Ramos-Casals Manuel⁴¹⁴²^ORCID,Brito-Zerón P,Flores-Chávez A,Ramos-Casals M,Horvath I F,Szántó A,Tarr T,Ng F,Rasmussen A,Farris D A,Dong X,Yan Z,Li X,Xu B,Baldini C,Bombardieri S,Mandl T,Olsson P,Priori R,Giardina F,Izzo R,Seror R,Mariette X,Gottenberg J E,Kruize A A,Hinrichs A,Bootsma H,Vissink A,Danda D,Sandhya P,Hernandez-Molina G^ORCID,Sánchez-Guerrero J,Armagan B^ORCID,Kilic L,Kalyoncu U,Quartuccio L,Gandolfo S,De Vita S,Praprotnik S,Sebastian A,Wiland P,Gerli R,Bartoloni E,Kwok S-K,Park S-H,Kvarnstrom M,Wahren-Herlenius M,Rischmueller M,Downie-Doyle S,Solans-Laque R,Sene D,Pasoto S G,Suzuki Y,Kawano M,Isenberg D A,Nordmark G,Valim V,Nakamura H,Shimizu T,Nishihata S -Y,Nakamura T,Takagi Y,Fernandes Moça Trevisani V,Retamozo S,Hofauer B,Knopf A,Fraile G,Giacomelli R,Devauchelle-Pensec , V,Saraux A,Bombardieri M,Astorri E,Atzeni F,Hammenfors D,Brun J G,Carsons S E,Maure Noia B,Argibay Filgueira A B,Gheita T A,Sánchez Berná I,López Dupla M,Alberto Rojas R,Febrer Nafria A M,Morel J,Fonseca Aizpuru E,Santos Seoane S,Brito-Zerón P,Morcillo C,Melchor Díaz S,Carreira P,Vollenveider C,Vázquez M,Ericka Díaz Cuiza P,Herrera B E,Andrea Consani S,Comotto A,de Miguel Campo B,Kostov B,Sisó-Almirall A,Kostov B,Acar-Denizli N,

Affiliation:

1. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán , Mexico City, Mexico

2. Primary Healthcare Transversal Research Group, IDIBAPS

3. Department of Statistics and Operations Research, Universitat Politècnica de Catalunya (UPC)

4. Department of Medicine, Autoimmune Diseases Unit, Hospital CIMA- Sanitas , Barcelona, Spain

5. Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen , Groningen, The Netherlands

6. Department of Clinical Sciences Malmö, Division of Rheumatology, Lund University , Malmö, Sweden

7. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht , Utrecht, The Netherlands

8. Clinic of Rheumatology, Department of Medicine (DAME), University of Udine, ASU FC , Udine

9. Rheumatology Unit, University of Pisa , Pisa, Italy

10. Rheumatology Department, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpitaux Universitaires Paris-Sud - Hôpital Bicêtre, Paris-Saclay University, INSERM UMR1184: Centre for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin-Bicêtre, France

11. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen , Debrecen, Hungary

12. Centre for Rheumatology, Division of Medicine, University College London , London, UK

13. Rheumatology Unit, Department of Medicine & Surgery, University of Perugia , Perugia, Italy

14. Rheumatology, Department of Medical Sciences, Uppsala University , Uppsala, Sweden

15. Genes and Human Disease Research Program, Oklahoma Medical Research Foundation , Oklahoma City, OK, USA

16. Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Vall d’Hebron, Barcelona, Spain

17. Otorhinolaryngology/Head and Neck Surgery, Klinikum rechts der Isar, Technical University of Munich , Munich, Germany

18. Université de Paris, Departement of Internal Medicine, Hôpital Lariboisière, Assistance Publique- Hôpitaux de Paris , Paris, France

19. Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de Sao Paulo , Sao Paulo, Brazil

20. Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine, University of Adelaide , Adelaide, Australia

21. Department of Rheumatology, University Medical Centre , Ljubljana, Slovenia

22. Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University , Cairo, Egypt

23. Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital , Vellore, India

24. Department of Internal Medicine, Faculty of Medicine, Hacettepe University , Ankara, Turkey

25. Division of Rheumatology, Kanazawa University Hospital , Kanazawa, Ishikawa, Japan

26. Department of Medicine, Federal University of Espírito Santo , Vitória, Brazil

27. Rheumatology Department, Brest University Hospital , Brest, France

28. Rheumatology Department, Hospital Universitari Parc Taulí , Sabadell, Barcelona, Spain

29. Instituto Modelo de Cardiología Privado SRL , Córdoba, Argentina

30. Instituto Universitario de Ciencias Biomédicas de Córdoba (IUCBC) , Córdoba, Argentina

31. Division of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital

32. Academic Specialist Center, Center for Rheumatology, Stockholm Health Services , Stockholm, Sweden

33. Department of Rheumatology and Internal Medicine, Wroclaw Medical University , Wroclaw, Poland

34. IRCCS Galeazzi Orthopaedic Institute, Milan and Rheumatology Unit, University of Messina , Messina

35. Department of Clinical Immunology and Rheumatology, School of Medicine, University of Rome ‘Campus Biomedico’ , Rome, Italy

36. Division of Rheumatology, Allergy and Immunology, NYU Long Island School of Medicine , Mineola, NY, USA

37. Division of Rheumatology, Department of Internal Medicine, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea , Seoul, South Korea

38. Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences , Nagasaki, Japan

39. Federal University of São Paulo , Sao Paulo, Brazil

40. CONACYT , Mexico City, Mexico

41. Department of Diseases, ICMiD

42. Department of Medicine, University of Barcelona, Hospital Clinic , Barcelona, Spain

Abstract

Abstract Objective To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.

Publisher

Oxford University Press (OUP)

Subject

Pharmacology (medical),Rheumatology

Link

https://academic.oup.com/rheumatology/advance-article-pdf/doi/10.1093/rheumatology/keac205/43447798/keac205.pdf

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