Glomerular Diseases · Published 2026-07-09 · DOI 10.1159/000553335
Thanawat Vongchaiudomchoke, Boonyarit Cheunsuchon, Nuttaya Wachiraphansakul
Introduction: Primary membranous nephropathy, one of the most common primary nephrotic syndromes in adults, rarely has tubular involvement. Two case reports have previously illustrated atypical tubular manifestations, including Liddle-like syndrome and proximal tubulopathy. Hypokalemic distal tubulopathy in anti-phospholipase A2 receptor antibody-positive membranous nephropathy has not yet been recognized. Case presentation: We report a case of a 37-year-old Thai male presenting with nephrotic syndrome and rhabdomyolysis secondary to severe hypokalemia. Kidney biopsy and serologic testing confirmed anti-phospholipase A2 receptor antibody-positive membranous nephropathy. Urinary indices showed a distal tubulopathy pattern. Hormonal studies, including plasma renin, plasma aldosterone and a dexamethasone suppression test, were all negative. The patient responded well to prednisolone and oral cyclophosphamide. With nephrotic syndrome remission, hypokalemia was alleviated simultaneously. Conclusion: This case suggests a recognition and expansion of the knowledge of an extremely rare tubular involvement in relation to anti-phospholipase A2 receptor antibody-positive membranous nephropathy.
Abstract from DOAJ. Public domain (CC0 1.0).
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Vongchaiudomchoke, T., Cheunsuchon, B., Wachiraphansakul, N. (2026). Hypokalemic distal tubulopathy secondary to PLA2R-positive membranous nephropathy: an exceptional case. Glomerular Diseases. https://doi.org/10.1159/000553335