Wegener’s Granulomatosis: Granulomatosis With Polyangiitis

Wegener’s granulomatosis, or known now as Granulomatosis with polyangiitis is a rare disorder that causes inflammation of blood vessels.  This inflammation can occur anywhere but is most common in occurring in the nose, sinuses, throat, lungs, and kidneys.  This is defined under a blood vessel disorder called vasculitis. 

This disorder is when the tissues in the body become inflamed, slowing the blood flow from your organs.  These inflamed tissues are called granulomas.  Granulomas are what affect how the organs that are affected work

What causes this disease is unknown.  Without treatment this disease can be fatal.  Though with early treatment most people have a full recovery.  Granulomatosis with polyangiitis can affect anyone at any age, though it shows to be more common in ages between 40-65.   

Symptoms

Granulomatosis with polyangiitis can occur anywhere, most common areas are ear, eye, nose, sinuses, trachea, lungs, kidneys, skin, joints, and nerves.  Symptoms can occur suddenly or gradually over time.  The first signs present in the sinuses, throat, or lungs.  Sometimes the condition will progress quickly.  Symptoms are dependent on what organs are affected.  You will want to see a doctor immediately if you develop pus like drainage from your nose, or have stuffiness, nosebleeds, or continuous sinus infections. 

Other symptoms are cough with phlegm, shortness or breath with wheezing, fever, fatigue, joint pain, numbness in limbs, fingers or toes, weight loss, blood in your urine, skin sores, bruising, rashes, eye redness, vision problems, ear inflammation, or hearing problems. 

Symptoms are dependent on the person and the case.  Not everyone will experience the same symptoms. 

Diagnosis

This disease can be difficult to diagnose.  Though not being treated can cause some serious complications.  Some complications are kidney or lung failure, hearing loss, skin scarring, kidney damage, weakened cartilage in nose or blood clots.  Early diagnosis is key. 

If your doctor suspects you have Granulomatosis with polyangiitis they will want to run some blood tests.  The blood test can indicate signs of inflammation, anemia, or kidney functions.  After the blood test your doctor may want an urine sample as well to test kidney functions. 

Imaging tests such as chest X-ray, CT, or MRI can show if any granulomas are growing and what organs they are affecting.  If granulomas are present your doctor will want to biopsy the affected tissue. 

Treatment

After diagnosis, treatment is straightforward once you know what organs are involved.  Achieving remission is the goal of treatment.  Taking long term medications can help prevent relapse.  Medications that may be prescribed to you are immunosuppressants which will help your body suppress your immune system to help reduce the inflammation of the blood vessels.  Corticosteroids are another medication that can help.  Plasma exchanges can help when the kidneys have been damaged and need to recover.  

Outcomes

You will have to be continuously supervised if you are diagnosed with Granulomatosis with polyangiitis.  Relapses can occur with either the same symptoms or with completely different ones, which is why making sure you go in for regular check ups and scans can be a crucial part of staying on top of treatment.  Going without treatment can be fatal.  So staying on top of this disease is crucial to making a full recovery. 

Most people who receive treatment have a full life with minor setbacks.  Though this wasn’t always the case, before the 1970’s patients with Granulomatosis with polyangiitis didn’t live past five months after diagnosis.  Now about 80% of people who are diagnosed in time, are now surviving eight years and longer past diagnosis.

Prepare for your doctor’s visit with a list of ongoing symptoms you have been having, and how long they have lasted for so your doctor can help diagnose you as quickly and effectively as possible.  Early treatment is key to beating Granulomatosis with polyangiitis.