What it is
Granulomatosis with polyangiitis (GPA) is a rare disease in which the immune system inflames and damages small to medium-sized blood vessels. Because those vessels run throughout the body, GPA can affect several organs at once, most often the sinuses and airways, the lungs, and the kidneys.
Signs and symptoms
Pulmonary nodule
GPA can produce nodules (rounded spots) in the lungs, sometimes with a hollow center, which is why a chest scan is often part of the work-up.
Hemoptysis
Coughing up blood (hemoptysis) can occur when GPA inflames the lungs' blood vessels. It is a symptom to report to a clinician promptly.
Cytoplasmic antineutrophil antibody positivity
Most people with GPA carry a specific autoantibody in their blood, c-ANCA, which a lab test can detect. A positive result helps point to the diagnosis.
Anti-proteinase 3 antibody positivity
The c-ANCA in GPA is usually aimed at a protein called proteinase-3 (PR3). A blood test showing anti-PR3 antibodies strengthens the case for GPA.
Hematuria
Blood in the urine (hematuria), often together with protein, can be an early sign that GPA is affecting the kidneys, even before symptoms are obvious.
Glomerulonephritis
GPA often inflames the kidney's tiny filters (glomerulonephritis), which can show up as blood or protein in the urine and, if unchecked, can reduce kidney function.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
Cyclophosphamide
Cyclophosphamide is a long-established immune-suppressing medicine used to bring active GPA under control (remission induction). It is one of the standard options a specialist may use for serious disease.
Used to help with: Granulomatosis with polyangiitis.
“Cyclophosphamide remains a cornerstone of remission induction therapy in granulomatosis with polyangiitis…”
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How to read the evidence labels
Where this comes from
This guide is built from 3 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.