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Blood and immune system diseases

Granulomatosis with polyangiitis (GPA, Wegener's)

A rare disease with inflammation of small and medium-sized blood vessels. The nose, sinuses, lungs and kidneys are most often affected.

Not part of the panel of Türkiye's National Newborn Screening Programme (heel-prick test). Source

Prepare an emergency card

This page is translated from the Turkish original.

This disease is followed through the “Blood and immune system diseases” group.

Understanding the disease

This section has not yet been reviewed by a physician. It summarises patient information from sources such as MedlinePlus, Orphanet and the NHS in plain language. The disease can differ from person to person; talk to your doctor about diagnosis and treatment.

What happens in this disease?

The immune system attacks the body's own blood vessels. An antibody called ANCA is usually found in the blood. Inflammation of the vessels reduces the blood going to the organs. Small lumps of inflammation called granulomas form in the tissue.

Which symptoms can occur?

Symptoms can be mild or severe. They may include:

  • Blocked nose, crusting and nosebleeds (common)
  • Sinusitis and ear infections
  • A cough that does not go away, coughing up blood and shortness of breath
  • Fever, night sweats, weakness and weight loss
  • Blood in the urine and kidney problems
  • Red eyes, joint pain and skin sores

How is it diagnosed?

The blood is tested for the ANCA antibody. A biopsy, which is a small tissue sample, is taken from the affected tissue. Imaging of the chest and sinuses and a urine test are done.

Treatment and care

Treatment has two stages. First the disease is brought under control, then flare-ups are prevented. Cortisone and medicines that suppress the immune system are used. In severe cases, biological medicines or a chemotherapy medicine may be given. There are approved biological treatments for this disease. If the kidneys are badly affected, dialysis or a transplant may be needed. The doctor who follows the disease decides which treatment is suitable.

For the family: inheritance and genetic counselling

GPA is not considered an inherited disease. Most people have no other affected family members. Some genes may increase the risk. Genes and the environment play a role together.

Source: MedlinePlus Genetics: Granulomatosis with polyangiitis · NHS: Granulomatosis with polyangiitis (GPA) · MedlinePlus: Granulomatosis with polyangiitis

Key facts

How common is it?
New cases per year: 1-9 per 1 000 000 (Europe) · Prevalence: 1-9 per 100 000 (Europe)
How is it inherited?
Not inherited
When does it usually start?
Adolescence, Adulthood, Childhood, Older age
Gene(s) involved
PRTN3, CTLA4, PTPN22, HLA-DPB1, HLA-DPA1

These facts are taken from Orphanet and MedlinePlus records (last checked: October 7, 2026). Frequency values are approximate ranges.

You are not alone: patient associations in Türkiye

Non-profit patient and family organizations in Türkiye working on this disease or disease group. Links are for information; Doktorclub is not affiliated with these organizations and does not endorse them.

We could not find a patient association specific to this disease.

For all rare diseases: Nadir Hastalıklar Federasyonu

Support and help

Doktorclub does not diagnose, recommend treatments or refer you to hospitals or doctors. These pages are meant to help you talk with your doctor.

News about GPA (Wegener's)

Clinical trials (ClinicalTrials.gov)

A clinical trial is a regulated study that tests whether a new treatment is safe and whether it works, in patients who volunteer. Most trials look for a certain age, genetic change and health condition. Taking part is voluntary.

This list is not a treatment recommendation. Treatments tested in phase 1–3 trials are investigational (experimental): their efficacy and safety have not yet been proven and they are not approved treatments. Taking part depends on each trial’s criteria and a physician’s assessment; the first step is to talk to the doctor who manages your care.

Questions you can ask your doctor

  • Could this trial be suitable for us?
  • If we take part, how would current treatment and check-ups be affected?
  • Where does the trial take place, and how are travel and accommodation handled?
  • What happens if we want to leave the trial?

ClinicalTrials.gov lists 7 active interventional studies for this disease. 6 are recruiting now; none has a site in Türkiye. Last checked: October 7, 2026.

Selected records (studies with a site in Türkiye first):

See all studies on ClinicalTrials.gov · Joining clinical trials

Blood and immune system diseases: other news

Medical codes (for talking with your doctor and finding sources)

These codes identify the disease in international classifications and databases. They can help when you talk with your doctor or look for reliable sources.

Orphanet code: ORPHA:900 · ICD-11 4A44.A1

Codes checked against WHO ICD-11 and Orphanet on October 7, 2026.

This page provides general information and does not replace diagnosis or treatment. Talk to your doctor about symptoms, diagnosis and treatment.

The information on these pages is for general information only and does not replace medical advice. Talk to your doctor about diagnosis and treatment decisions. In an emergency in Türkiye, call 112.

Every update is based on an open source and labeled with its level of evidence. No advertising and no drug brand names. Our scientific principles

This page was translated from Turkish by the Doktorclub editorial team.