Background Multicentric Castleman's disease (MCD) is a disorder characterized by polyclonal proliferation of B lymphocytes that is frequently associated with autoimmune manifestations and connective tissue diseases. MCD presents high levels of IL-6 and systemic symptoms such as fever, arthralgia, hepatosplenomegaly and serositis, so it is recommended to include MCD in the differential diagnosis of adult-onset Still's disease (AOSD). However, there are no studies comparing both groups of patients.
Objectives To compare the clinical and laboratory features at onset between patients with MCD and AOSD seen in a Madrid tertiary care hospital.
Methods We performed a retrospective observational study in patients with diagnosis MCD and AOSD attended our center between January 1989 and December 2015. The variables included demographics, clinical manifestations, laboratory tests and Yamaguchi's criteria.
Results A total of 34 patients were included, 17 with MCD and 17 with AOSD. The comparison of the characteristics of both groups is presented in the table. There were no differences in age, uration of disease (MCD 158,6 days and AOSD 250,5 days, p=0,3919), diagnostic delay (MCD 18,4 and AOSD 52,2, p=0,2711), arthritis, myalagias, pleuritis or macrophagic activation syndrome, but persistent fever, rash, arthralgia, pharyngitis and pericarditis were significantly more frequent in ESA, whereas male gender, human immunodeficiency virus (HIV) and/or human herpes virus 8 (HHV8) infection, hepatosplenomegaly and lymphadenopathy were significantly higher in MCD. In the laboratory test results, leukocytosis and hypertransaminasemia were significantly higher in AOSD. 52.9% of patients with MCD met 5 or more Yamaguchi's criteria for AOSD.
Conclusions Patients with MCD may present common systemic manifestations and laboratory abnormalities at onset with AOSD and up to 50% of them may fulfill Yamaguchi's criteria for this disease, so MCD should be taken into account in its differential diagnosis.
Disclosure of Interest None declared