Kikuchi-Fujimoto Disease and primary Sjögren's Syndrome coexisting: a case-based literature review.
Mod Rheumatol Case Rep
; 2024 Sep 21.
Article
em En
| MEDLINE
| ID: mdl-39305128
ABSTRACT
Kikuchi-Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotizing lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterized by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with NSAIDs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with Systemic Lupus Erythematosus, and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren's Syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren Syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Idioma:
En
Revista:
Mod Rheumatol Case Rep
/
Modern rheumatology case reports
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Portugal
País de publicação:
Reino Unido