Granulomatosis With Polyangiitis: Symptoms, Causes, Treatment

Severity ranges from localized symptoms to organ-threatening disease. Early symptoms can resemble persistent sinusitis, but renal or pulmonary findings call for broader assessment.

Related reading: Another form of small-vessel vasculitis.

Nasal congestion, crusting or bleeding, sinus pain and hearing problems may accompany fatigue, rash or cough. Kidney involvement may remain silent until urine or blood testing detects it.

Immune dysfunction causes vessel inflammation and granulomatous inflammatory clusters. The cause remains uncertain; environmental and genetic influences may contribute. It is not contagious or an inevitable consequence of a cold.

The disease usually develops in adulthood but can affect children. Associated antibodies are not a sufficient general screening test for diagnosing otherwise healthy people.

Assessment combines urine and kidney tests, antibody studies, imaging and sometimes tissue biopsy. A positive antibody test alone is insufficient, and a negative result does not always exclude disease.

Treatment depends on severity. Severe disease may need rituximab or cyclophosphamide with corticosteroids, followed by remission-maintenance therapy. Less intensive options are selected for appropriate nonsevere cases.

Hearing or vision loss, kidney damage, pulmonary bleeding and nerve injury can occur. Relapse is possible, while treatment-related infection risk needs ongoing monitoring and a clear care plan.

There is no established prevention. Regular urine and kidney monitoring and infection-prevention planning are important during treatment. Do not stop medicines solely because symptoms improve.