Adult Onset Autosomal Dominant Familial Focal Segmental Glomerulosclerosis Cn380162
An inherited kidney disease causing gradual kidney failure in adults. Managed with medications and may eventually require kidney transplant.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Proteinuria
(renal)Edema
(renal)Hypertension
(cardiovascular)Hematuria
(renal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
ACE Inhibitor or ARB Therapy
Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers are first-line renoprotective agents that reduce proteinuria, slow glomerulosclerosis progression, and control hypertension.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal Function Surveillance
Regular monitoring of serum creatinine, estimated glomerular filtration rate (eGFR), urine protein-to-creatinine ratio, and lipid panels to track disease progression and treatment response.
Additional treatment options
Immunosuppressive Therapy
Corticosteroids and/or calcineurin inhibitors (cyclosporine, tacrolimus) may be employed in aggressive disease variants showing progressive decline in kidney function despite conservative management.
medicationRenoprotective Dietary Modification
Low-sodium diet to control blood pressure and reduce proteinuria, with moderate protein restriction to decrease glomerular hyperfiltration. Adequate potassium intake should be maintained.
dietaryLifestyle Modification Program
Weight management, smoking cessation, and regular physical activity tailored to preserve remaining kidney function. Smoking cessation is particularly critical as smoking accelerates FSGS progression.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Adult Onset Autosomal Dominant Familial Focal Segmental Glomerulosclerosis Cn380162?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1-S276.
D'Agati VD, Kaskel FJ, Falk RJ. Focal segmental glomerulosclerosis. N Engl J Med. 2011;365(25):2398-2411.
Rosenberg AZ, Kopp JB. Focal segmental glomerulosclerosis: diagnosis and classification. Adv Chronic Kidney Dis. 2017;24(2):90-96.
Savica V, Dinneen O, Singh AK, Hari P, Bagga A. Treatment of focal segmental glomerulosclerosis in adults: a systematic review and meta-analysis. Clin Kidney J. 2020;13(4):558-567.
Medication Comparisons
Compare medications used to treat Adult Onset Autosomal Dominant Familial Focal Segmental Glomerulosclerosis Cn380162. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy