# Retrospective cohort evaluation of renal involvement in non-HIV castleman disease patients from a single academic center in Beijing, China

**Authors:** Hongtao Ling, Lihong Wang, Wei Wang, Xiaoying Yang, Wenqiong Wang, Shuanglian Xie, Yiming Zhao, Shujing Guo, Weiwei Xie, Zhizhen Lai, Huihui Liu, Xiaodi Yang, Xiaojuan Yu, Yujun Dong

PMC · DOI: 10.1007/s00277-026-06739-1 · 2026-01-15

## TL;DR

This study examines kidney involvement in non-HIV Castleman disease patients, finding it more common in multicentric cases and showing no negative impact on survival.

## Contribution

The study provides a detailed analysis of renal involvement in non-HIV Castleman disease, highlighting its prevalence and outcomes in a large cohort.

## Key findings

- Renal involvement occurred in 6.03% of unicentric and 55.22% of multicentric Castleman disease patients.
- Thrombotic microangiopathy was the most frequent renal pathology in multicentric cases.
- Renal involvement did not significantly affect overall survival in Castleman disease patients.

## Abstract

Castleman disease (CD), a rare and clinically heterogeneous condition, frequently involves renal impairment, though this relationship remains poorly characterized. This large cohort study of 183 patients (116 unicentric [UCD], 67 multicentric [MCD]) investigated renal involvement (RI). RI occurred in 6.03% (7/116) of UCD and 55.22% (37/67) of MCD patients. In UCD-RI, 4 underwent renal biopsy, revealing varied pathological results, and 1 underwent total left nephroureterectomy. In MCD-RI, common manifestations included edema, nephrotic syndrome, and acute renal failure. Thrombotic microangiopathy (TMA) was the most frequent renal pathology (9/19 biopsies). Acute renal failure often responded well to treatment, with 60% (9/15) achieving complete recovery. The myeloma-like treatment regimen demonstrated superior efficacy compared to the lymphoma-like regimen and immunomodulatory therapy (P = 0.039). The 5-year renal survival rate in the MCD-RI group was 88.9%, not significantly different from UCD-RI (P = 0.45). Furthermore, the 5-year overall survival in the CD-RI group was 81.9%, showing no statistically significant difference from CD patients without renal involvement (P = 0.11). This study confirms that RI is more common in MCD, with TMA as a key pathological feature, and demonstrates that renal involvement does not negatively impact overall survival.

The online version contains supplementary material available at 10.1007/s00277-026-06739-1.

## Linked entities

- **Diseases:** Castleman disease (MONDO:0015564), nephrotic syndrome (MONDO:0005377), acute renal failure (MONDO:0002492), thrombotic microangiopathy (MONDO:0019737)

## Full-text entities

- **Genes:** IL6 (interleukin 6) [NCBI Gene 3569] {aka BSF-2, BSF2, CDF, HGF, HSF, IFN-beta-2}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, SDC1 (syndecan 1) [NCBI Gene 6382] {aka CD138, SDC, SYND1, syndecan}, FLT1 (fms related receptor tyrosine kinase 1) [NCBI Gene 2321] {aka FLT, FLT-1, VEGFR-1, VEGFR1}, ALB (albumin) [NCBI Gene 213] {aka FDAHT, HSA, PRO0883, PRO0903, PRO1341}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, VEGFA (vascular endothelial growth factor A) [NCBI Gene 7422] {aka L-VEGF, MVCD1, VEGF, VPF}
- **Diseases:** tumors (MESH:D009369), reticulin fibrosis (MESH:D005355), membranous nephropathy (MESH:D015433), Infectious Diseases (MESH:D003141), respiratory failure (MESH:D012131), BO (MESH:D001989), thrombocytopenia (MESH:D013921), proteinuria (MESH:D011507), fatigue (MESH:D005221), HIV (MESH:D015658), Fanconi syndrome (MESH:D005198), Small vessel damage (MESH:D059345), diabetic nephropathy (MESH:D003928), lymphoma (MESH:D008223), Renal TMA (MESH:D057049), systemic lupus erythematosus (MESH:D008180), eosinophilia (MESH:D004802), IgA nephropathy (MESH:D005922), pancreatic cancer (MESH:D010190), hydronephrosis (MESH:D006869), ascites (MESH:D001201), solid (MESH:D018250), PNP (MESH:D010392), infection (MESH:D007239), lymphoproliferative disorder (MESH:D008232), ANCA (MESH:D056648), focal segmental glomerulosclerosis (MESH:D005923), rash (MESH:D005076), pericardial effusion (MESH:D010490), Acute renal failure (MESH:D058186), aMCD (MESH:D012514), plasmacytic lymphadenopathy (MESH:D007952), decreased appetite (MESH:D001068), hypertension (MESH:D006973), splenomegaly (MESH:D013163), POEMS syndrome (MESH:D016878), preeclampsia (MESH:D011225), myeloma (MESH:D009101), renal failure (MESH:D051437), RI (MESH:C565423), RPGN (MESH:C538458), anemia (MESH:D000740), MCD-RI (MESH:C537372), fever (MESH:D005334), chronic renal insufficiency (MESH:D051436), hematuria (MESH:D006417), microvascular damage (MESH:D017566), nephrotic syndrome (MESH:D009404), ESRD (MESH:D007676), inflammatory (MESH:D007249), glomerulonephritis (MESH:D005921), ischemic kidney injury (MESH:D007674), renal (MESH:D006030), Amyloidosis (MESH:D000686), pleural effusion (MESH:D010996), lymphadenopathy (MESH:D008206), hypoalbuminemia (MESH:D034141), ureteral mass (MESH:D014515), anti-GBM disease (MESH:D019867), COVID-19 (MESH:D000086382)
- **Chemicals:** HE (-), creatinine (MESH:D003404), Congo red (MESH:D003224), dexamethasone (MESH:D003907), siltuximab (MESH:C504234), formaldehyde (MESH:D005557), epon (MESH:C004875), cyclophosphamide (MESH:D003520), sirolimus (MESH:D020123), Hematoxylin (MESH:D006416), paraformaldehyde (MESH:C003043), pomalidomide (MESH:C467566), azathioprine (MESH:D001379), eosin (MESH:D004801), uranyl acetate (MESH:C005460), steroid (MESH:D013256)
- **Species:** Homo sapiens (human, species) [taxon 9606], Human gammaherpesvirus 8 (no rank) [taxon 37296]

## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12804195/full.md

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Source: https://tomesphere.com/paper/PMC12804195