Ten tips for the diagnosis and management of nephrotic syndrome
Ilay Berke, Annette Bruchfeld, Eleni Frangou, Sarah M Moran, Luis F Quintana, Kate I Stevens, YK Onno Teng, Stefanie Steiger, Andreas Kronbichler, Safak MiriogluAbstract
Nephrotic syndrome (NS) encompasses a heterogeneous group of glomerular diseases characterized by heavy proteinuria, hypoalbuminemia, edema, and multiple systemic complications. Despite substantial advances in diagnostic techniques and targeted therapies, the management of NS remains challenging in clinical practice. In addition to complex treatment decisions, clinicians must address diagnostic uncertainty, recognize secondary causes, and prevent potentially serious complications including thrombosis, infections, cardiovascular disease, and progressive kidney dysfunction. Adverse outcomes in NS frequently arise not only from inappropriate management but also from overlooked steps in diagnosis, monitoring, and long-term care. Here, we present 10 practical tips to improve clinical decision-making in the diagnosis and management of NS. Key positive strategies include appropriate use of kidney biopsy and modern immunopathologic techniques, integration of immunologic biomarkers such as anti-phospholipase A2 receptor antibodies in the evaluation of membranous nephropathy, systematic exclusion of secondary causes, and comprehensive supportive care including blood pressure control, antiproteinuric therapy, and thrombosis prophylaxis in high-risk patients. In addition, evidence-based use of immunosuppressive therapies (IST) tailored to specific disease entities is emphasized. Equally important are commonly overlooked pitfalls that may compromise outcomes, including delayed genetic testing in steroid-resistant disease, prolonged ineffective immunosuppression, inadequate post-transplant surveillance for recurrence, under-recognition of cardiovascular risk, and failure to prevent complications associated with IST. Highlighting these practical considerations may help increase awareness of common pitfalls and support more attentive clinical management of patients with NS.