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

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.

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.

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