Living with Hypocomplementemic urticarial vasculitis
A rare condition of inflamed small blood vessels with repeated hives and low complement proteins, sometimes affecting joints, lungs, eyes, and kidneys.
Understanding Hypocomplementemic urticarial vasculitis
It causes repeated hives (urticaria) and painful skin sores that itch or burn. Some people also have joint pain, lung disease, eye inflammation, or kidney inflammation. When it is more severe, some scientists call it HUV syndrome (HUVS). It usually happens by chance, but family cases have been reported. It may link to an infection or another disease such as lupus.
Signs & symptoms
Symptoms vary from person to person. GARD lists these among the most common:
- Recurrent hives and swelling
- Itchy or burning skin lesions
- Joint pain (arthritis)
- Breathing difficulty and cough
- Eye inflammation
- Blood in urine
Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.
Diagnosis
- 1Talk with your doctor
Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.
- 2See a specialist
Specialists who may be involved include autoimmune and inflammatory disease specialists, genetic specialists and kidney specialists.
- 3Consider genetic testing
Changes in DNASE1L3 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.
- 4Connect with expert centers
Vasculitis Foundation may keep lists of experts for this condition.
Care & treatment
- Care team
- autoimmune and inflammatory disease specialists (rheumatologists); Genetic specialists (geneticists); Kidney specialists (nephrologists)
- Expert care
- Expert lists: Vasculitis Foundation
- Treatment
- GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
- Research
- Clinical trials and observational studies may be options. See the research section below.
General information, not medical advice. Your care team makes the decisions.
Everyday life
Living with a rare condition is easier with support.
- Connect with Vasculitis Foundation and other patient organizations
- Keep notes on symptoms, changes and questions for appointments
- Ask about patient registries that help research
- Look into financial aid and travel resources for care visits
Quick facts
Both genetic and environmental factors are thought to play a role. It is thought to involve an abnormal immune response. GARD lists DNASE1L3 as a gene and autosomal recessive inheritance.
Also known as: anti-c1q vasculitis; hypocomplementemic vasculitis; mac duffie hypocomplementemic urticarial vasculitis; mac duffie syndrome; mcduffie hypocomplementemic urticarial vasculitis; mcduffie syndrome
Patient organizations
- Vasculitis FoundationHypocomplementemic urticarial vasculitis · United States
- National Kidney FoundationKidney diseases · United States
- Renal Support NetworkKidney diseases · United States
You are more than your diagnosis
Perspectives from people navigating life with Hypocomplementemic urticarial vasculitis.
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