Atypical Complement Factor H Deficiency Cn380067
A rare immune system disorder where a protein that controls inflammation is not working properly. It can affect the kidneys and other organs. Treatment involves managing inflammation and preventing complications.
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
Microangiopathic hemolytic anemia
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Proteinuria
(renal)Hypertension
(renal)C3 glomerulopathy
(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
Complement Inhibitors (Eculizumab/Ravulizumab)
Monoclonal antibodies that inhibit terminal complement activation by binding C5. This prevents complement-mediated tissue damage in the kidneys and other affected organs.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Renal Function Surveillance
Regular monitoring of kidney function through estimated GFR, urinalysis for proteinuria and hematuria, and imaging to detect structural abnormalities early and guide management.
Additional treatment options
Plasma Exchange Therapy
Removal of patient plasma containing abnormal complement factor H and replacement with fresh frozen plasma or albumin. This provides temporary functional complement regulation.
medicationInfection Prevention Strategies
Comprehensive infection prevention including vaccinations, meticulous hygiene, prompt treatment of infections, and avoidingcrowded healthcare settings when possible.
lifestyleRenal-Protective Nutrition
Dietary modifications including appropriate protein intake, sodium restriction if hypertensive, and careful potassium management to reduce kidney burden and slow disease progression.
dietaryWhen 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 Complement Factor H Deficiency Cn380067?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Atypical Complement Factor H Deficiency Cn380067. 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