Key result
Gitelman syndrome patients with proteinuria and renal dysfunction have ~74% higher plasma angiotensin II.
Why the study?
The incidence, risk factors, pathological features, and prognosis of proteinuria and renal dysfunction in Gitelman syndrome were unclear.
Cohort (n=116)
Absolute Event Rate: 440.2% vs 253.2%
p-value: p=0.031
Proteinuria and renal dysfunction in Gitelman syndrome are associated with elevated angiotensin II levels and may indicate underlying glomerulopathy and vascular lesions, though renal function generally remains stable with therapy.
May indicate glomerulopathy in Gitelman syndrome; leaves open whether angiotensin II-targeted therapy alters renal progression.
INTRODUCTION: Gitelman syndrome (GS) is a rare renal tubular salt-wasting disorder. Besides kidney electrolyte loss, proteinuria and renal dysfunction were also observed. However, their incidence, risk factors, pathological features, and prognosis were unclear. METHODS: We retrospectively reviewed 116 GS patients and analyzed their clinical, genetic, and pathological characteristics. We also systematically reviewed articles on GS with proteinuria and renal dysfunction. RESULTS: Twenty-three GS patients had proteinuria (69.6%) and renal dysfunction (43.5%) with a mean age of 35.3 ± 13.2 years, and 65.2% were male. Compared to patients without proteinuria or renal dysfunction, these patients had elevated plasma angiotensin II level (440.2 ± 351.7 vs. 253.2 ± 187.4 pg/mL, p = 0.031) and three times higher incidence of diabetes. The renal pathology of nine biopsied patients indicated hypertrophy of the juxtaglomerular apparatus (100%), chronic tubulointerstitial changes (66.7%), intrarenal vascular changes (66.7%), and glomerulopathy (55.6%). More extensive renin staining was observed in patients with GS than in the control group with glomerular minor lesion (p < 0.001). During a median of 85 months (range, 11-205 months) of follow-up for 19 out of the 23 GS-renal patients, the renal function was generally stable, except one died of cancer and one developed end-stage renal disease because of concomitant membranous nephropathy and IgA nephropathy. CONCLUSION: Proteinuria and renal dysfunction were more common than expected and might indicate glomerulopathy and vascular lesions besides a tubulointerstitial injury in GS. Renal function may maintain stable with effective therapy in most cases.
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Zhang et al. (2023) conducted a cohort in Gitelman syndrome (n=116). Proteinuria and renal dysfunction vs. Patients without proteinuria or renal dysfunction was evaluated on Plasma angiotensin II level (pg/mL) (p=0.031). Gitelman syndrome patients with proteinuria and renal dysfunction had significantly elevated plasma angiotensin II levels (440.2 vs. 253.2 pg/mL, p=0.031) compared to those without.
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