Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases

Abstract

Purpose To explore the clinical features and immunological mechanisms of Castleman disease (CD) complicated with autoimmune diseases (AID). Methods We explored the prevalence and clinical manifestations of CD complicated with AID by reviewing clinical, pathological, and laboratory data of 40 CD patients retrospectively, and then explored abnormal immune mechanisms in the co-existence of the two entities by monitoring lymphocyte subsets in peripheral blood. Results Paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren’s syndrome, myasthenia gravis, and psoriasis were found to be coexisted with CD in 9/40 (22.5%) patients with different sequence of onset. No bias in the clinical and histological type of CD was observed for the occurrence of AID. CD patients with AID were more likely to have skin and/or mucous membrane damage and pulmonary complications, and presented elevated erythrocyte sedimentation rate, hypergammaglobulinemia, and positive autoantibodies than those without AID (p < 0.05). Deregulated cellular and innate immune responses as indicated by decreased CD3+ T cells and increased natural killer cells were observed in peripheral blood of CD patients with AID (p < 0.05). UCD patients with AID were successfully treated with surgery and immunosuppressive therapy. MCD complicated by AID relieved with immunosuppressors, cytotoxic chemotherapy, and rituximab. Conclusion Systemic inflammation/immunological abnormalities and organ dysfunction were associated with the occurrence of AID in CD. Impairment of cellular and innate immunity may be a candidate etiology for the coexistence of the two entities.

Comprehensive analysis of 65 patients with Castleman disease in a single center in China

“…Among the comorbidities, liver diseases and splenomegaly appeared more frequent in CD, which may have a relationship with immunity decrease. Besides, paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren’s syndrome, myasthenia gravis, and psoriasis were also reported to be with CD 31 . Due to the various symptoms or locations of the disease, the admission departments were also widely distributed, and the department where patients were most likely to be treated was the Dept.…”

Idiopathic Multicentric Castleman Disease with Strikingly Elevated IgG4 Concentration in the Serum and Abundant IgG4-Positive Cells in the Tissue: A Case Report

“…As a B-cell activator, IL-6 induces B-cell differentiation into immunoglobulin-producing cells, resulting in polyclonal gammopathies or autoantibodies [ 17 ]. Thus, various autoantibodies could be found in iMCD patients [ 18 ].…”

Oral lichenoid lesion simultaneously associated with Castleman’s disease and papillary thyroid carcinoma: a rare case report

“…A retrospective study has shown that 47% cases of CD were accompanied with cutaneous/mucosal lichen planus-like lesions [ 12 ]. The underlying mechanism is not fully understood, which may be related to the autoantibody production, epitope spreading, antigen mimicry and interleukin-6 upregulation [ 22 , 23 ]. Nonetheless, to our knowledge, no study has reported a definite OLL case associated with CD.…”