Description
A type of vasculitis that involves medium sized arteries. The name has been changed to granulomatosis with polyangiitis. Causes inflammation and narrowing of the blood vessels. It most often affects and damages the kidneys, lungs, and upper respiratory tract. The cause of the disease is not known. Left untreated the disorder is life threatening due to severe lung injury and /or kidney failure. This disease is more often seen in adults greater than 35 years old.
Symptoms
Persistent sinus and nasal inflammation, fever, night sweats, weight loss, bloody urine, cough, coughing up blood, chest pain, shortness of breath.
Tests
Common tests used for diagnosis and treatment
Workup:
A history and physical exam will be performed. A blood test for antineutrophil cytoplasmic antibodies (ANCA) is almost always positive in people with the disease. Other tests include chest x-ray, chest CT scan, urine analysis, blood tests for kidney function. A biopsy is usually needed to confirm the diagnosis.
Other Specific Tests: Antineutrophil cytoplasmic antibodies (ANCA), open lung biopsy, upper airway biopsy, nasal mucosal biopsy, bronchoscopy with transtracheal biopsy
Specialists:
Internal Medicine, Rheumatology, Pulmonology, Nephrology
Workup:
A history and physical exam will be performed. A blood test for antineutrophil cytoplasmic antibodies (ANCA) is almost always positive in people with the disease. Other tests include chest x-ray, chest CT scan, urine analysis, blood tests for kidney function. A biopsy is usually needed to confirm the diagnosis.
Other Specific Tests: Antineutrophil cytoplasmic antibodies (ANCA), open lung biopsy, upper airway biopsy, nasal mucosal biopsy, bronchoscopy with transtracheal biopsy
Specialists:
Internal Medicine, Rheumatology, Pulmonology, Nephrology
Treatment
Initial treatment usually includes corticosteroids combined with cyclophosphamide/Cytoxan or rituximab (Rituxan). Once the disease is in remission, doses of the drugs are slowly lowered.