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

Gabriela Hernández-Molina, Belchin Kostov, Pilar Brito-Zerón, Arjan Vissink, Thomas Mandl, Anneline C Hinrichs, Luca Quartuccio, Chiara Baldini, Raphaele Seror, Antonia Szántó, David Isenberg, Roberto Gerli, Gunnel Nordmark, Astrid Rasmussen, Roser Solans-Laque, Benedikt Hofauer, Damien Sène, Sandra G Pasoto, Maureen Rischmueller, Sonja PraprotnikTamer A Gheita, Debashish Danda, Berkan Armağan, Yasunori Suzuki, Valeria Valim, Valerie Devauchelle-Pensec, Soledad Retamozo, Marika Kvarnstrom, Agata Sebastian, Fabiola Atzeni, Roberto Giacomelli, Steven E Carsons, Seung-Ki Kwok, Hideki Nakamura, Virginia Fernandes Moça Trevisani, Alejandra Flores-Chávez, Xavier Mariette, Manuel Ramos-Casals, Sjögren Big Data Consortium

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskriftPeer review


OBJECTIVE: To characterize 414 patients with primary SS who developed hematological malignancies and to analyze 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. Hematological 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 hematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). 376 (91%) patients had mature B cell malignancy, nearly half MALT lymphoma (n = 197), followed by DLBCL (n = 67), nodal MZL lymphoma (n = 29), CLL/SLL (n = 19) and follicular lymphoma (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 hematological 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%.

Sidor (från-till)243-255
TidskriftRheumatology (Oxford, England)
Tidigt onlinedatum2022
StatusPublished - 2022

Bibliografisk information

© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email:

Ämnesklassifikation (UKÄ)

  • Cancer och onkologi
  • Reumatologi och inflammation
  • Hematologi


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