Titre du document / Document title

Malignant lymphoma in primary Sjögren's syndrome: A multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's syndrome

Auteur(s) / Author(s)

European Concerted Action on Sjögren's Syndrome, EUROPE
VOULGARELIS M. ⁽¹⁾ ; DAFNI U. G. ⁽¹⁾ ; ISENBERG D. A. ⁽²⁾ ; MOUTSOPOULOS H. M. ⁽¹⁾ ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

⁽¹⁾ National University of Athens, Athens, GRECE
⁽²⁾ University College, London, ROYAUME-UNI

Résumé / Abstract

Objective. Several reports have noted an increased incidence of malignant lymphoma in patients with Sjögren's syndrome (SS). Each case series has consisted of a limited number of patients with malignant non-Hodgkin's lymphoma (MNHL). In this report, we describe the disease characteristics, the clinical course, and the evolution in 33 patients followed up in 9 European medical centers. Methods. The pool of MNHL patients from participating centers in a European Concerted Action on SS were analyzed. We report on the disease characteristics, its evolution, prognosis, current treatment practices, and survival. Results. The MNHLs in this study were primarily situated in the marginal zone (48.5%), with the manifestations mostly extranodal (78.8%) and most often identified in the salivary glands (54.6%). Lymphadenopathy (65.6%), skin vasculitis (33.3%), peripheral nerve involvement (24.2%), low-grade fever (25.0%), anemia (48.1%), and lymphopenia (78.6%) were observed significantly more frequently than in the general SS population. Patients with high-to-intermediate grade lymphoma had significantly worse survival (P = 0.041). The presence of B symptoms (fever, night sweats, and weight loss) and a large tumor diameter (>7 cm) were additional independent risk factors for death. Conclusion. The novel observations of this study were those related to the type of MNHL, the survival prognosis, and the very high frequency of skin vasculitis, peripheral nerve involvement, anemia, and lymphopenia. Some of the previously reported results on extranodal manifestations were confirmed.

Revue / Journal Title

Arthritis and rheumatism   ISSN 0004-3591   CODEN ARHEAW 

Source / Source

1999, vol. 42, n^o8, pp. 1765-1772 (40 ref.)

Langue / Language

Anglais

Editeur / Publisher

Wiley, Hoboken , NJ, ETATS-UNIS  (1958) (Revue)

Mots-clés anglais / English Keywords

Sjögren syndrome ; Primary ; Association ; Non Hodgkin lymphoma ; Malignant lymphoma ; Symptomatology ; Evolutivity ; Treatment ; Prognosis ; Human ; Multicenter study ; Eye disease ; Stomatology ; Systemic disease ; Immunopathology ; Autoimmune disease ; Malignant hemopathy ; Lymphoproliferative syndrome ;

Mots-clés français / French Keywords

Sjögren syndrome ; Primaire ; Association ; Lymphome non hodgkinien ; Lymphome malin ; Symptomatologie ; Evolutivité ; Traitement ; Pronostic ; Homme ; Etude multicentrique ; Oeil pathologie ; Stomatologie ; Maladie système ; Immunopathologie ; Maladie autoimmune ; Hémopathie maligne ; Lymphoprolifératif syndrome ;

Mots-clés espagnols / Spanish Keywords

Sjögren síndrome ; Primario ; Asociación ; Linfoma no Hodgkin ; Linfoma maligno ; Sintomatología ; Evolutividad ; Tratamiento ; Pronóstico ; Hombre ; Estudio multicéntrico ; Ojo patología ; Estomatología ; Enfermedad sistémica ; Inmunopatología ; Enfermedad autoinmune ; Hemopatía maligna ; Linfoproliferativo síndrome ;

Localisation / Location

INIST-CNRS, Cote INIST : 8711, 35400008940000.0280