Glucocorticoids are indicated for this patient who most likely has minimal change glomerulopathy (MCG). MCG is the most common cause of idiopathic nephrotic syndrome in children and accounts for approximately 10% of cases in adults. Although the mechanism of MCG is not well understood, it results in fusion and dysfunction of the epithelial foot processes of the glomerulus, causing significant loss of protein and other macromolecules into the urine. Patients with MCG typically present with acute onset of edema and weight gain due to fluid retention. Urine protein levels tend to be significantly elevated (urine protein-creatinine ratio typically 5000-10,000 mg/g). The abrupt onset of the full nephrotic syndrome, a history of kidney disease in childhood that remitted, negative serologic tests, and a kidney biopsy showing normal light microscopic findings and negative immunofluorescence are diagnostic of MCG. Electron microscopy is confirmatory and usually demonstrates the extensive effacement of the podocyte foot processes. Most patients with MCG are symptomatic, and the disease does not remit spontaneously in the weeks or months after presentation. Treatment with immunosuppressive medications is recommended to prevent complications of severe nephrotic syndrome, including severe symptomatic edema, thromboembolic events, and infections. Glucocorticoids such as prednisone are recommended as first-line therapy unless there are contraindications. More than 80% of patients respond within 16 weeks of treatment. Alternative first-line therapy for patients with contraindications to glucocorticoids (for example, obesity, impaired glucose tolerance or diabetes mellitus, or psychiatric conditions) includes calcineurin inhibitors such as cyclosporine.
Alkylating agents such as cyclophosphamide are reserved for frequently relapsing or glucocorticoid-dependent patients with MCG.
ACE inhibitors such as lisinopril or angiotensin receptor blockers (typically used to inhibit the progression of chronic kidney disease) are typically not indicated to treat MCG because the duration of disease is short with glucocorticoid therapy, and patients are not hypertensive.
Although MCG is not a common cause of end-stage kidney disease in adults, and untreated patients may slowly improve, the nephrotic-range proteinuria of MCG is associated with a significantly increased risk for thromboembolism and infection. Therefore, because most patients treated with glucocorticoid therapy recover with a favorable prognosis, not providing treatment is inappropriate.