Atypical Hemolytic Uremic Syndrome With Anti Factor H Antibodies C5680286
A form of aHUS caused by antibodies that block Factor H, a key complement regulator. Often affects younger patients. Treated with complement inhibitors and sometimes immunosuppression.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Rapidly progressive glomerulonephritis
Hypertensive encephalopathy
Pulmonary edema
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Schistocytes on blood smear
(hematologic)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
Anti-Complement Inhibitor Therapy
C5 inhibitors form the cornerstone of treatment to block complement-mediated endothelial damage caused by anti-factor H antibodies. Typically continued long-term to prevent relapse.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Plasma Exchange with Immunosuppression
Combines apheresis to remove circulating antibodies with immunosuppressive agents to suppress new antibody production. Often used as initial therapy or adjunct to complement inhibitors.
Additional treatment options
Rituximab
Anti-CD20 monoclonal antibody to deplete B-cells and reduce anti-factor H antibody production. Reserved for refractory cases or those unable to tolerate other therapies.
medicationAntibody Titer and Complement Level Monitoring
Serial measurement of anti-factor H antibody titers and complement CH50, C3, C4 levels to assess disease activity and guide treatment decisions.
monitoringStress Reduction and Immune Support
Mind-body techniques including meditation, adequate sleep, and stress management to support immune regulation. Supplements may be considered to address nutritional deficiencies.
complementaryWhen 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 Atypical Hemolytic Uremic Syndrome With Anti Factor H Antibodies C5680286?
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.
Kavanagh D, Goodship TH, Richards A. Diagnosis and management of complement-mediated kidney diseases. Kidney Int. 2021;100(5):975-988.
Noris M, Remuzzi G. Atypical hemolytic uremic syndrome. N Engl J Med. 2019;381(18):1736-1748.
Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic uremic syndrome. N Engl J Med. 2013;368(23):2169-2181.
Loirat C, Frémeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:60.
Medication Comparisons
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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