THU0545 Comparison of the clinical and laboratory features at onset between multicentric castleman's disease and adult-onset still's disease

Abstract

BackgroundMulticentric 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.ObjectivesTo compare the clinical and laboratory features at onset between patients with MCD and AOSD seen in a Madrid tertiary care hospital.MethodsWe 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.ResultsA 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.Multicentric Castleman's disease (n=17)Adult-onset Still's disease (n=17)p-value Male:female ratio2.4:10.4:10.0163Age at diagnosis (mean ± SD)46.4±13.137.5±13.50.0598HIV infection35.2%0%0.0154HHV8 infection35.2%0%0.0154Persistent fever47.1%94.1%0.0078Rash29.4%64.7%0.0392Arthralgias35,3%88.2%0.0014Arthritis0%17.6%0.0696Pharyngitis0%41.2%0.0029Hepatosplenomegaly47.1%5.9%0.0065Pericarditis0%23.5%0.0332Pleuritis17.6%11.7%0.6217Lymphadenopathy100%17.6%0.0001Macrophage activation syndrome11.7%17.6%0.6282Leucocytes >10000/mm335.2%76.5%0.0156Increased transaminases0%58.8%0.0002Erythrosedimentation rate (mm/Hg)5277.50.1104C-reactive protein (md/dl)75.1109.50.3699Ferritin (ng/ml)24019260.1234ConclusionsPatients 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 InterestNone declared

A challenging diagnosis of idiopathic multicentric Castleman disease with complex systemic presentation: A case report

“…Still's disease is a disorder that commonly poses challenges in clinical differentiation from iMCD. Clinical similarities between these diseases have been previously reported, with patients with MCD fulfilling several diagnostic criteria for Still's disease 20 . Our patient was treated with tocilizumab, which is an anti‐IL‐6 receptor antibody commonly used in the treatment of Still's disease 16,21 .…”

“…Clinical similarities between these diseases have been previously reported, with patients with MCD fulfilling several diagnostic criteria for Still's disease. 20 Our patient was treated with tocilizumab, which is an anti‐IL‐6 receptor antibody commonly used in the treatment of Still's disease. 16 , 21 Despite initial short‐term resolution of symptoms and normalization of inflammatory parameters, treatment with tocilizumab did not result in long‐term clinical improvement in our patient and was therefore discontinued.…”