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Clinical characteristics and immunological abnormalities of Castleman disease complicated with autoimmune diseases
Dao-Ping Sun1,2, Wen-Ming Chen3, Li Wang1,4
1Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, 210029, China.
Insights
Castleman disease (CD) can coexist with autoimmune diseases (AID), affecting over 20% of patients. Impaired cellular and innate immunity may contribute to this overlap, leading to systemic inflammation and organ dysfunction.
Area of Science:
- Immunology
- Hematology
- Rheumatology
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder.
- Autoimmune diseases (AID) involve the immune system attacking the body's own tissues.
- The co-occurrence of CD and AID presents complex clinical challenges.
Purpose of the Study:
- To investigate the clinical characteristics of Castleman disease (CD) when complicated by autoimmune diseases (AID).
- To elucidate the underlying immunological mechanisms involved in the co-existence of CD and AID.
Main Methods:
- Retrospective analysis of clinical, pathological, and laboratory data from 40 CD patients.
- Monitoring of peripheral blood lymphocyte subsets to assess immune responses.
- Comparison of clinical and immunological features between CD patients with and without AID.
Main Results:
- Over 22% of CD patients had coexisting AID, including paraneoplastic pemphigus, autoimmune hemolytic anemia, Sjogren's syndrome, myasthenia gravis, and psoriasis.
- CD patients with AID exhibited higher rates of skin/mucous membrane damage, pulmonary complications, elevated inflammatory markers, and positive autoantibodies.
- Peripheral blood analysis revealed deregulated immune responses in CD patients with AID, characterized by decreased CD3+ T cells and increased natural killer cells.
Conclusions:
- Systemic inflammation, immunological abnormalities, and organ dysfunction are linked to AID occurrence in CD.
- Impaired cellular and innate immunity are potential etiological factors for the co-existence of CD and AID.
- Treatment strategies involving surgery, immunosuppression, chemotherapy, and rituximab were effective for CD with AID.
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.
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