Background Castleman's disease (CD) is a lymphoproliferative disorder characterized by polyclonal expansion of B lymphocytes, which features 2 clinical subtypes: unicentric and multicentric, and 3 histopathological variants: hyaline vascular, plasma cell, and mixed. Multicentric CD (MCD) occurs as a systemic disease and is characterized by elevated levels of IL6, generalized lymphadenopathy, hepatosplenomegaly, and constitutional symptoms. These patients may develop autoimmune manifestations, and some of them may associate systemic or organ-specific autoimmune diseases (AD), however, there are few studies about this association.
Objectives To study the spectrum of autoimmune manifestations in patients with MCD diagnosed in a Spanish tertiary hospital.
Methods All the patients diagnosed with MCD during the period 1985-2013 were included. Demographic, clinical, immunologic, therapeutic and outcome data were analyzed using the statistical software SPSS 21.0.
Results A total number of 16 patients were included, 81% males, with a mean age at diagnosis of 47 years (range: 23-68) and mean disease duration of 6.1 months. The most frequent histological subtype was the plasma cell (63%). There was history of HIV infection in 8 cases, 7 of them had positive serology for HHV8, associated Kaposi sarcoma in 3 cases and lymphoma in 1 case. All the patients had peripheral lymphadenopathy and mediastinal in 81%. Over 60% presented with fever, hepatosplenomegaly and increased acute phase reactants (AFR), with mean value of ESR 83 mm/h and CRP 80 mg/dL. All them also had hypergammaglobulinemia, being monoclonal in 25% of cases. Among the clinical manifestations suggestive of AD, 4 patients had arthralgia, 2 Raynaud's phenomena, 2 maculopapular rash, 1 photosensitivity, 1 sicca syndrome, and 1 purpura. The immunological abnormalities observed were thrombocytopenia in 6 cases, hemolytic anemia in 2 cases, positive rheumatoid factor in 4 cases, cryoglobulins in 2 cases, and ANA plus anti-DNA in 1 case. AD was diagnosed in 7 cases, corresponding to systemic lupus erythematosus (1 case), undifferentiated connective tissue disease (1 case), Evans syndrome (ES) (2 cases) and leukocytoclastic vasculitis (3 cases). One of the patients with ES and 3 other without AD were compatible with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes). The treatments administered in these patients included antiretrovirals in 7 cases (HIV-positive), corticosteroids in 7 cases, rituximab in 7 cases, and other chemotherapeutic agents in 3 cases. During the follow-up period, 10 patients achieved complete remission of MCD and autoimmune manifestations. There were 4 deaths and all occurred in patients without associated AD.
Conclusions MCD should be considered in patients with suspected AD who have characteristics manifestations such as generalized lymphadenopathy, hepatosplenomegaly or marked elevation of AFR, as MCD presents frequently autoimmune abnormalities and some cases are associated with defined AD. The prognosis seems to be better in this latter group. Because MCD an AD share common pathogenic mechanisms, therapies directed against B lymphocytes (anti-CD20) and cytokines (anti-IL6) may be effective for both entities
Disclosure of Interest : None declared