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Background <p>Whether IgA nephropathy (IgAN) in patients with systemic lupus erythematosus (SLE) represents a coincidental comorbidity or a distinct clinico-pathological entity remains unclear. This study aimed to characterise the demographic, clinical, pathological, and prognostic features of this rare association.</p> Methods <p>We conducted a systematic review of the PubMed and Embase databases to 31 May 2025 were conducted to identify all reported cases of biopsy-proven IgAN in patients with SLE, excluding cases with concomitant thrombotic microangiopathy, negative Gd-IgA1 immunostaining, prior IgA vasculitis, or ANA-negative disease. Study quality was assessed using the JBI checklist. Individual patient data were pooled; descriptive statistics summarized clinical features, and group comparisons were performed using Mann-Whitney U and Fisher’s exact tests. Individual patient data were extracted and analyzed as a single cohort. Renal outcomes were defined as either a ≥ 40% decline in estimated glomerular filtration rate (eGFR) from baseline or progression to end-stage renal disease.</p> Results <p>Thirty-one patients (25.8% male; mean age 40.8 ± 15.8 years; 67.7% Asian) were included. Median proteinuria was 1.10&#xa0;g/24&#xa0;h, and 12.9% presented with nephrotic syndrome. Microscopic haematuria was observed in 93.5% of patients. Mean eGFR at biopsy was 84.0 ± 53.4 mL/min/1.73&#xa0;m², with 12.9% exhibiting acute kidney injury (AKI). All patients were antinuclear antibody-positive, 64.5% were anti-dsDNA-positive, and 55.2% had hypocomplementaemia. Renal biopsy revealed dominant mesangial IgA and complement C3 deposition, with electron-dense deposits confined to the mesangium in all but one case. Immunosuppression (glucocorticoids 87.1%; cyclophosphamide 22.6%) yielded complete remission in 54.8% of patients. Over a median follow-up of 16.5 months, 27.8% of patients reached the renal endpoint, including three of four patients with AKI.</p> Conclusions <p>Limited by retrospective case report methodology, this largest cohort to date suggests IgAN complicating SLE manifests a distinctive phenotype that bridges features of primary IgAN and lupus nephritis. Although immunosuppression is effective, long-term renal risk remains non-negligible. Early recognition and targeted therapy may improve outcomes.</p> Clinical trial number <p>Not applicable.</p>

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Clinicopathological characteristics and renal outcomes of IgA nephropathy in systemic lupus erythematosus: a case report and systematic literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年5月29日

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  • XiXi Zhao,
  • ZhenZhen Li,
  • GuangYan Cai,
  • FengKun Chen,
  • Jian Zhang,
  • XuanLi Tang,
  • Xu Zhang,
  • ZhiYu Duan,
  • Yan Song

摘要

Background

Whether IgA nephropathy (IgAN) in patients with systemic lupus erythematosus (SLE) represents a coincidental comorbidity or a distinct clinico-pathological entity remains unclear. This study aimed to characterise the demographic, clinical, pathological, and prognostic features of this rare association.

Methods

We conducted a systematic review of the PubMed and Embase databases to 31 May 2025 were conducted to identify all reported cases of biopsy-proven IgAN in patients with SLE, excluding cases with concomitant thrombotic microangiopathy, negative Gd-IgA1 immunostaining, prior IgA vasculitis, or ANA-negative disease. Study quality was assessed using the JBI checklist. Individual patient data were pooled; descriptive statistics summarized clinical features, and group comparisons were performed using Mann-Whitney U and Fisher’s exact tests. Individual patient data were extracted and analyzed as a single cohort. Renal outcomes were defined as either a ≥ 40% decline in estimated glomerular filtration rate (eGFR) from baseline or progression to end-stage renal disease.

Results

Thirty-one patients (25.8% male; mean age 40.8 ± 15.8 years; 67.7% Asian) were included. Median proteinuria was 1.10 g/24 h, and 12.9% presented with nephrotic syndrome. Microscopic haematuria was observed in 93.5% of patients. Mean eGFR at biopsy was 84.0 ± 53.4 mL/min/1.73 m², with 12.9% exhibiting acute kidney injury (AKI). All patients were antinuclear antibody-positive, 64.5% were anti-dsDNA-positive, and 55.2% had hypocomplementaemia. Renal biopsy revealed dominant mesangial IgA and complement C3 deposition, with electron-dense deposits confined to the mesangium in all but one case. Immunosuppression (glucocorticoids 87.1%; cyclophosphamide 22.6%) yielded complete remission in 54.8% of patients. Over a median follow-up of 16.5 months, 27.8% of patients reached the renal endpoint, including three of four patients with AKI.

Conclusions

Limited by retrospective case report methodology, this largest cohort to date suggests IgAN complicating SLE manifests a distinctive phenotype that bridges features of primary IgAN and lupus nephritis. Although immunosuppression is effective, long-term renal risk remains non-negligible. Early recognition and targeted therapy may improve outcomes.

Clinical trial number

Not applicable.