HYPOCOMPLEMENTEMIC URTICARIAL VASCULITIS SYNDROME

Hypocomplementemic 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 is often associated with systemic diseases such as COPD, systemic lupus, and Sjögren syndrome.

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.

Physical findings: HUVS usually involves the skin with painful urticaria (hives), and may cause inflammatory changes in joints, kidneys, gastrointestinal tract, lungs, heart, and eyes.

ICD-9: 279.4

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:

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  Claimant’s medical source(s) records of clinical history and physical findings;

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  Skin and/or tissue biopsy report;

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  Laboratory reports showing abnormal complement levels and C1q antibody; or

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  Evidence of organ dysfunction, especially eye, renal, cardiac, and respiratory systems (for example, pulmonary function tests).

Suggested Listings for Evaluation:

REMARKS