Granulomatosis with Polyangiitis – Symptoms and Causes
Overview
Granulomatosis with polyangiitis (GPA) is a rare condition. It’s characterized by inflammation of small blood vessels. This disease primarily affects vessels in the respiratory system and kidneys.
However, it can impact any organ in the body. GPA belongs to a group of blood vessel disorders known as vasculitis.
The disease was previously known as Wegener granulomatosis. When this condition slows blood flow, inflamed areas called granulomas can form in affected tissues. These granulomas may cause organ damage and impair normal function.
GPA symptoms vary widely among patients. Initial symptoms often resemble a common cold, with additional symptoms developing based on which organs become affected.
While the exact cause remains unclear, the immune system likely plays a significant role in the disease process.
With proper treatment, patients can maintain a good quality of life. However, without intervention, the disease may cause serious organ damage that can be life-threatening.
Signs and Symptoms
Ear, Nose and Throat Issues
When this condition affects the head area, you might notice several problems.
Your nose may drain thick fluid with crusts. You might get sores inside your nose or mouth. Many people feel stuffiness or have frequent sinus infections.
Nosebleeds can happen often. Some people experience ear pain or drainage from their ears. The cartilage in your ears or the bridge of your nose might become inflamed and change shape over time.
Breathing and Chest Problems
This condition can affect your lungs and breathing passages. You might cough frequently, sometimes bringing up blood. Many people feel short of breath or wheeze when breathing.
Some develop a high-pitched sound (stridor) when they breathe. Your voice may become hoarse or raspy. For some people, only the lungs are affected. Without proper treatment, this can lead to bleeding in the lungs and tissue scarring.
Kidney Changes
When the condition affects your kidneys, you might not notice any symptoms at first. As it progresses, you might develop high blood pressure.
Some people notice swelling in their legs. Blood may appear in your urine, which can look pink, red, or brown.
Vision and Eye Effects
If your eyes are affected, they might become red and irritated. You may feel burning or pain in your eyes. Some people describe a sensation like having something stuck in their eye.
Your vision might become blurry, or you might see double. In some cases, the eyes can bulge outward, or the eyelids may become swollen.
Additional Body Effects
This condition can cause other symptoms throughout your body:
- Nerve problems causing numbness, tingling, or deep aching in your limbs or fingers
- Skin rashes that may appear as purple patches or spots
- Digestive problems including stomach pain, diarrhea, and blood in your stool
When To Contact a Doctor
The symptoms of this condition can resemble other illnesses. Early signs might feel like a cold or flu that doesn’t go away for weeks.
You should see a healthcare provider if you have a persistent runny nose that cold medicines don’t help. It’s especially important to get medical attention if you have a runny nose along with:
- Nosebleeds with thick discharge
- Coughing up blood
- Any combination of the symptoms mentioned above
Without treatment, this condition can worsen rapidly and lead to serious complications.
Causes
The root cause of granulomatosis with polyangiitis (GPA) remains unclear. Scientists believe the immune system plays a key role in this condition. In people with GPA, certain white blood cells may function abnormally.
The body produces special proteins called antibodies to fight harmful germs. However, in GPA, specific antibodies mistakenly attack healthy cells instead. These misguided attacks damage the body’s own tissues.
GPA creates two main problems in the body:
- Inflamed, narrowed blood vessels – reducing blood flow to organs.
- Granulomas – inflammatory tissue masses that can destroy healthy tissue.
It’s important to understand that GPA is not contagious. You cannot catch it from someone else. Research also suggests that GPA is not typically inherited from parents, meaning genes likely don’t play a major role in its development.
Risk Factors
Age plays a key role in who gets granulomatosis with polyangiitis. People between 40 and 65 years old have a higher chance of developing this condition. However, it’s important to note that this disease can affect someone at any age.
Health Problems
GPA can cause several health issues throughout the body. These problems vary based on which organs are affected by the disease.
Some people experience hearing loss or a sunken bridge of the nose due to weakened cartilage. Skin may develop sores or scars.
Kidney damage can occur, sometimes leading to kidney failure. The lungs might bleed or develop scarring.
Other possible complications include:
- Deep vein blood clots (usually in the legs)
- Heart inflammation
- Stroke (rare)
- Vision loss (rare)
