What's granulomatosis with polyangiitis?
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.
| Also indexed as | ORPHA:900, MONDO:0012105 |
|---|---|
| Features mapped | 15 |
| Treatments mapped | 3 |
| Published sources | 9 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Vasculitis
Granulomatosis with polyangiitis is characterised by vasculitis, meaning inflammation of small and medium-sized blood vessels. This inflammation is what damages the organs the disease affects.
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.
Subglottic stenosis
GPA can scar and narrow the windpipe just below the vocal cords (subglottic stenosis). This narrowing can cause a hoarse voice, noisy breathing, and shortness of breath, and sometimes needs procedures to keep the airway open.
Hemoptysis
Coughing up blood (hemoptysis) can occur when GPA inflames the lungs' blood vessels. It is a symptom to report to a clinician promptly.
Epistaxis
Nosebleeds, along with crusting and a blocked or runny nose, are common early signs of GPA as the lining of the nose becomes inflamed and ulcerated.
Granulomatosis
In granulomatosis with polyangiitis, the immune system forms granulomas, which are small clusters of inflammatory cells. In this condition they typically develop in the respiratory tract.
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.
Fever
Granulomatosis with polyangiitis can cause a prolonged fever as part of its general, whole-body symptoms, and it is an uncommon but recognised cause of fever without an obvious source.
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.
Otitis media
GPA frequently affects the ears, causing fluid build-up behind the eardrum (otitis media with effusion). This can lead to ear fullness, pain, and hearing loss, and is sometimes the first symptom before other organs are involved.
Sinusitis
Persistent sinus inflammation is one of the most common early features of GPA and is often mistaken for ordinary chronic sinus infection, which can delay the diagnosis.
Abnormality of the nose
In granulomatosis with polyangiitis, the nose is one of the most commonly affected areas. Nasal crusting, nosebleeds, and damage to the nasal septum are among the most frequent findings.
Saddle-nose deformity
GPA inflammation can destroy the cartilage that supports the bridge of the nose, causing it to collapse into a saddle shape. This saddle-nose deformity is one of the more recognizable signs of long-standing or severe disease.
How it is diagnosed
Granulomatosis with polyangiitis
Diagnosed using: ANCA testing and tissue biopsy.
“Early biopsy, ANCA testing, and multidisciplinary management improved diagnostic accuracy and prevented organ damage.”
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…”
rituximab
Rituximab is a medicine that targets certain immune (B) cells. In granulomatosis with polyangiitis it is now a central option both for bringing active disease under control (remission induction) and for keeping it controlled afterwards (maintenance).
Used to help with: Granulomatosis with polyangiitis.
“Rituximab is now central to remission induction and maintenance, while avacopan in GPA (granulomatosis with polyangiitis) and MPA (microscopic polyangiitis) and interleukin (IL)-5 blockade in EGPA (eosinophilic granulomatosis with polyangiitis) further reduce glucocorticoid exposure and toxicity.”
glucocorticoids
Glucocorticoids (steroids) are an established part of treatment for granulomatosis with polyangiitis. Newer treatment approaches aim to use them in lower amounts to reduce their side effects.
Used to help with: Granulomatosis with polyangiitis.
“Rituximab is now central to remission induction and maintenance, while avacopan in GPA (granulomatosis with polyangiitis) and MPA (microscopic polyangiitis) and interleukin (IL)-5 blockade in EGPA (eosinophilic granulomatosis with polyangiitis) further reduce glucocorticoid exposure and toxicity.”
What changes how it shows up
The diagnosis is not the whole story. The factors and open questions below are described in the research mapped here as shaping whether, or how strongly, the condition shows up, or as points the field has not yet settled. They are not, on their own, its cause or its cure.
PR3-ANCA positivity
Most people with granulomatosis with polyangiitis test positive for PR3-ANCA, a specific type of ANCA antibody. Across studies, the great majority of cases are PR3-ANCA positive.
Described as modulating: Granulomatosis with polyangiitis.
“PR3-ANCA positivity ranged from 21 to 100%, with most studies reporting rates above 85%.”
How to read the evidence labels
Where this comes from
This guide is built from 9 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.