Related Experiment Video
Updated: Mar 12, 2026
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
Clinical Features of Patients with Castleman's Disease Complicated Systemic Lupus Erythematosus
Lu Zhang1, Xin-Xin Cao1, Shu-Jie Wang1
1Department of Hematology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.
Insights
Castleman's disease (CD) and systemic lupus erythematosus (SLE) co-occurrence is rare. Patients with both conditions show a higher incidence of autoimmune thrombocytopenia and less central nervous system involvement compared to general SLE patients.
Area of Science:
- Rheumatology
- Hematology
- Oncology
Background:
- Castleman's disease (CD) is a rare lymphoproliferative disorder.
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease.
- The co-occurrence of CD and SLE presents unique clinical challenges.
Purpose of the Study:
- To investigate the clinical features of patients diagnosed with both Castleman's disease and systemic lupus erythematosus.
- To compare the clinical presentation of this subgroup with the general SLE population.
Main Methods:
- Retrospective analysis of patients diagnosed with CD and SLE between 1994-2014 from Peking Union Medical College Hospital.
- Comprehensive literature review of PubMed using keywords: "Castleman's disease", "systemic lupus erythematosus", "SLE", and "lupus".
Main Results:
- Nine cases worldwide were analyzed, with 2 from PUMCH.
- The median age at CD diagnosis was 39.0 years; all had multicentric CD.
- Common symptoms included fever (88.9%), blood system involvement (88.9%), and kidney involvement (88.9%). Autoimmune thrombocytopenia (AITP) was observed in 55.6% of patients, significantly higher than in general SLE patients (15.0%). No central nervous system involvement was reported.
Conclusions:
- Castleman's disease complicated by SLE is a rare condition.
- This subgroup of patients exhibits a potentially higher rate of autoimmune thrombocytopenia.
- A lower incidence of central nervous system involvement may characterize CD patients with SLE.
Abstract:
Objective To investigate the clinical features of patients with Castleman's disease (CD) and systemic lupus erythematosus (SLE). Methods According to the diagnostic information between 1994 to 2014 extracted from the database of the Medical Record Department of Peking Union Medical College Hospital (PUMCH),patients with CD and SLE were included. A thorough literature review utilizing the key words of "Castleman's disease","systemic lupus erythematosus","SLE",and "lupus" was performed in PubMed during the same period. Cases with detailed clinical information were included while cases without detailed information were excluded from the analysis of this study. Results Nine patients worldwide were available for analysis [2 cases from PUMCH,accounted for 0.03%(2/6502) of all patients diagnosed as SLE and 1.0% (2/100) of patients diagnosed as CD during the same period] with a male-to-female ratio of2:7. The median age at diagnosis of CD was 39.0 years (range:21- 60 years). All patients were diagnosed as multicentric CD with generalized peripheral lymphadenopathy. Pathologic examination showed a balanced distribution:plasma cell variant:hyaline-vascular variant:mixed variant=3:3:3. Fever was the most common symptom (88.9%,8/9). Blood system was the most commonly involved system (88.9%,8/9) and kidneys were the most commonly involved organ (88.9%,8/9). Autoimmune thrombocytopenia (AITP) was observed in 55.6% (5/9) of patients,which was significantly higher than the general SLE patients (15.0%) (P<0.01). None of the 9 patients had evidence of central nervous system involvement. Conclusions CD complicated by SLE is a rare clinical condition. Compared to the general SLE population,this subgroup of patients may have higher rate of AITP and lower rate of central nervous system involvement.
Related Concept Videos
01:24Chronic Kidney Disease II: Clinical Manifestations
01:25Endocarditis II: Clinical Features of Infective Endocarditis
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:25Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
01:20COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
01:20Renal Corpuscle
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...