TN 7 (08-12)
DI 23022.765 Hypocomplementemic Urticarial Vasculitis Syndrome
COMPASSIONATE ALLOWANCE INFORMATION
HYPOCOMPLEMENTEMIC URTICARIAL VASCULITIS SYNDROME
Hypocomplementemic Vasculitis; Urticarial Vasculitis; McDuffie Syndrome
Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS) is a rare type of chronic autoimmune inflammation of small blood vessels and abnormally low levels of complement. HUVS usually involves the skin with painful urticaria (hives), and may cause inflammatory changes in joints, kidneys, gastrointestinal tract, lungs, heart, and eyes. HUVS is often associated with systemic diseases such as COPD, systemic lupus, and Sjögren syndrome.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Laboratory tests of serum documenting complement deficiency and positive C1q antibody, and skin or organ biopsy documenting leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis. The clinical criteria for diagnosis of HUVS include characteristic chronic urticaria with residual hyperpigmentation, and inflammatory vasculitis with angioedema, glomerulonephritis, chronic obstructive pulmonary disease, arthritis, or uveitis.
ONSET AND PROGRESSION
The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality, and is made worse by smoking. Kidney involvement with glomerulonephritis may ultimately result in end stage renal disease with need for kidney transplant. Death may also occur due to acute laryngeal edema.
The treatment of HUVS is determined by the organ involved and severity of the disease, and may include combinations of antihistamines, glucocorticoids and other immunosuppressive medications. COPD and cardiac valvular defects may require specific treatment.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation:
Treating physician records of clinical history and physical findings
Skin and/or tissue biopsy report
Laboratory reports showing abnormal complement levels and C1q antibody
Evidence of organ dysfunction, especially eye, renal, cardiac, and respiratory systems (for example, pulmonary function tests)
Suggested Listings for Evaluation:
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.