Hypocomplementemic urticarial vasculitis

How might hypocomplementemic urticarial vasculitis be diagnosed?

A diagnosis of HUV requires the presence of the two major criteria, as well as at least two minor criteria. The major criteria are hives (urticaria) for at least 6 months, and low levels of complement system proteins in the blood. The minor criteria are:

  • Inflammation in the small veins of the dermis (diagnosed by biopsy)
  • Joint pain or arthritis
  • Mild kidney inflammation (glomerulonephritis)
  • Eye inflammation (in the uvea or episclera)
  • Recurrent abdominal pain
  • The presence of anti-C1q antibodies (although this test is not widely available)

Additional laboratory studies may include tests for kidney function tests and immunological status. A chest x-ray should be done in individuals found to have low levels of complement system proteins and breathing problems.

Last updated on 05-01-20

What is the long-term outlook for people with hypocomplementemic urticarial vasculitis?

The long-term outlook (prognosis) for people with HUV varies from person to person. The prognosis largely depends on severity of systemic involvement and is influenced primarily by the severity of lung (pulmonary), heart, and kidney (renal) disease. When present, pulmonary disease (COPD) is the major cause of death. Cigarette smoking itself appears to be a strong risk factor for developing lung disease with HUV. Acute swelling of the larynx (laryngeal edema) can also be life-threatening. Although HUV is uncommon in childhood, the prognosis is worse for those affected at younger ages because of more frequent, severe renal involvement.

The prognosis can also be influenced by other underlying disorders that HUV is associated with. When HUV is associated with systemic lupus, the overlap of the two disease processes may cause relatively fast progression and poor prognosis, given that either disease can end fatally. Therefore, all patients diagnosed with HUV should also be examined for lupus (and possibly vice versa).

Because the symptoms and severity of HUV vary, it is not possible to predict the life expectancy for affected individuals.

Last updated on 05-01-20

Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: https://www.vasculitisfoundation.org/contact-us-2/ Url: https://www.vasculitisfoundation.org/

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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