Guideline sources The following summarized guidelines for the evaluation and management of focal segmental glomerulosclerosis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2021) and the Japanese Society of Nephrology (JSN 2016). 1 2 Guidelines 1. Diagnostic investigations Initial evaluation: Evaluate for secondary causes in adult patients with FSGS without nephrotic syndrome. B Consider referring selected patients with FSGS to specialized centers for genetic testing. C 2. Medical management Corticosteroids: As per KDIGO 2021 guidelines, initiate high-dose oral corticosteroids as first-line immunosuppressive treatment in patients with primary FSGS. B Show 2 more As per JSN 2016 guidelines: Initiate corticosteroids to reduce the urinary protein level and to prevent the decline of renal function in patients with FSGS. B https://web.pathway.md/diseases/recfXCIpSQxTJPNle 1/3 6/29/23, 4:06 AM Focal segmental glomerulosclerosis Pathway Consider administering corticosteroid pulse therapy when absorption of oral corticosteroids seems difficult. Immunosuppressants: As per KDIGO 2021 guidelines, do not initiate immunosuppression in adult patients with FSGS of undetermined cause or in patients with secondary FSGS. D Show 4 more As per JSN 2016 guidelines, initiate a combination of cyclosporine and corticosteroids to reduce the urinary protein level in patients with corticosteroid-resistant FSGS. B Show 3 more 3. Follow-up and surveillance Management of relapse: consider managing adult patients with previous corticosteroid- sensitive primary FSGS experiencing a relapse as adult patients with relapsing minimal change disease. C Clinical findings Patient demographics Symptoms African-american race Ankle swelling Fatigue Past medical history Headache Malaise Nephrotic syndrome Puffy eyelids Vascular exam Weight gain Peripheral edema Vital signs Abdominal exam Hypertension Abdominal distension Neurological exam Ascites Lethargy Gynecologic exam Genitourinary exam Vulvar swelling Scrotal edema Hematological findings Lab findings Anemia Hyperlipidemia serum LDL serum urea urine protein serum HDL serum albumin https://web.pathway.md/diseases/recfXCIpSQxTJPNle 2/3 6/29/23, 4:06 AM Focal segmental glomerulosclerosis Pathway 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 6 Idiopathic focal segmental glomerulosclerosis in adults. Kidney Int Suppl 2011 . 2012 Jun;2 2 181 185. Open 4. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open https://web.pathway.md/diseases/recfXCIpSQxTJPNle 3/3