Sinus histocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sjogren's syndrome.
Sinus histocytosis with massive lymphadenopathy is a rare disease that has been described by Rosai and Dorfman. It is characterized by massive, cervical lymphadenopathy, with extranodal manifestations in about 40% of patients. It occurs as a distinct entity, never associated with other diseases, and in most cases the prognosis is good. Lymphadenopathy is also a frequent sign of patients with primary Sjogren's syndrome (SS), usually associated with disease activity or concurrent infection. However, excessive lymphadenopathy in SS patients is a sign of lymphoproliferative disorder development. In this report, we describe a patient with primary SS, and excessive lymphadenopathy and splenomegaly who developed Rosai-Dorfman disease, and we discuss the possible aetiopathophysiological mechanism linking these two entities.
Drosos
A A
AA
Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, Greece. adrosos@cc.uoi.gr
Georgiadis
A N
AN
Metafratzi
Z M
ZM
Voulgari
P V
PV
Efremidis
S C
SC
Bai
M
M
D001707:Biopsy, Needle; D005500:Follow-Up Studies; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D018570:Risk Assessment; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D008775:Methylprednisolone