Guideline sources The following summarized guidelines for the evaluation and management of minimal change disease (MCD) are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021; 2012) and the Japanese Society of Nephrology (JSN 2016). 1 2 3 Guidelines 1. Classification and risk stratification Prognosis: recognize that long-term kidney survival is excellent in patients with MCD responding to corticosteroids, but less certain for patients not responding. B 2. Diagnostic procedures Kidney biopsy: perform kidney biopsy to diagnose MCD in adult patients. B 3. Medical management https://web.pathway.md/diseases/recvzefEM0zmUSMJD 1/3 6/30/23, 1:04 AM Minimal change disease Pathway Corticosteroids: As per KDIGO 2021 guidelines, initiate high-dose corticosteroids for no longer than 16 weeks as initial treatment in adult patients with MCD unless contraindicated. B Show 2 more As per JSN 2016 guidelines, administer oral corticosteroids to reduce proteinuria in the initial treatment of patients with MCD. B Show 2 more Immunosuppressants: consider initiating cyclophosphamide, a calcineurin inhibitor, or mycophenolate mofetil as initial therapy in adult patients with relative contraindications to corticosteroids. C Supportive therapy: Avoid using statins for hyperlipidemia in adult patients with an initial episode of nephrotic syndrome associated with MCD. D Avoid using ACEIs or ARBs for proteinuria in normotensive adult patients with an initial episode of nephrotic syndrome associated with MCD. D 4. Therapeutic procedures Renal replacement therapy: consider initiating RRT as indicated in adult patients with MCD and AKI, together with corticosteroids as for a first episode. C 5. Follow-up and surveillance Assessment of treatment response: define complete remission as reduction of proteinuria to < 0.3 g/day or protein-to-creatinine ratio < 300 mg/g (or < 30 mg/mmol), stable serum creatinine and serum albumin > 3.5 g/dL (or 35 g/L). B Show 5 more Management of relapse (corticosteroids): treat infrequent relapses with corticosteroids. B Management of relapse, immunosuppressants: As per KDIGO 2021 guidelines, initiate cyclophosphamide, rituximab, calcineurin inhibitors, or mycophenolic acid analogs in adult patients with frequently relapsing/corticosteroid-dependent MCD. B Prefer cyclophosphamide in cyclophosphamide-naive patients, and rituximab, calcineurin inhibitors, or mycophenolate mofetil/sodium mycophenolate in patients previously treated with or wishing to avoid cyclophosphamide. B As per JSN 2016 guidelines: Administer cyclosporine with corticosteroids to reduce proteinuria in patients with steroid- resistant and relapsing MCD. B Insufficient evidence to support that cyclosporine prevents the decline of renal function. I https://web.pathway.md/diseases/recvzefEM0zmUSMJD 2/3 6/30/23, 1:04 AM Minimal change disease Pathway Clinical findings Symptoms Past medical history Ankle swelling Nephrotic syndrome Fatigue Neurological exam Headache Malaise Lethargy Puffy eyelids Genitourinary exam Shortness of breath Scrotal edema Vital signs Lab findings Hypertension Hyperlipidemia Abdominal exam serum LDL Abdominal distension serum urea Ascites urine protein serum HDL Gynecologic exam serum albumin Vulvar swelling Hematological findings Anemia References 1. Kidney Disease: Improving Global Outcomes KDIGO Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100 4S S1 S276. Open 2. Nishi S, Ubara Y, Utsunomiya Y et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clin Exp Nephrol. 2016 Jun;20 3 342 70. Open 3. No authors listed. Chapter 5 Minimal-change disease in adults. Kidney Int Suppl 2011 . 2012 Jun;2 2 177 180. Open https://web.pathway.md/diseases/recvzefEM0zmUSMJD 3/3