A rare autoimmune disease, granulomatosis with polyangiitis (GPA) causes inflammation of blood vessels in the nose, sinuses, throat, lungs, and kidneys. The causes, symptoms, diagnosis, treatment, and prevention of GPA will be discussed in this blog post. If left untreated, GPA can cause serious complications, such as kidney failure, lung damage, and blindness.
A genetic component may play a role in triggering GPA, which is believed to be caused by an abnormal immune response that attacks the body’s own tissues. The exact cause of GPA is unknown, but it may be triggered by an abnormal immune response. Some people may be more susceptible to GPA due to a genetic component, since it is neither contagious nor inherited.
The symptoms of GPA vary depending on which organs are affected by the inflammation. The most common symptoms include:
Nosebleeds, nasal congestion, crusting, and discharge
Sinus pain and infections
Coughing, wheezing, shortness of breath, and coughing up blood
Fever, fatigue, weight loss, and night sweats
Joint pain and swelling
Skin rashes, ulcers, and bruises
Eye redness, pain, and vision problems
Ear infections and hearing loss
Numbness, tingling, and weakness in the limbs
Blood in the urine and kidney problems
The symptoms of GPA can develop suddenly or gradually, and they can range from mild to severe. Some people may have only one or a few organs affected, while others may have multiple organs involved.
There is no single test that can confirm a GPA diagnosis, making it difficult to diagnose. To check for signs of inflammation and organ damage, the doctor usually performs a physical examination, asks about the patient's medical history, and orders blood tests and imaging tests, such as X-rays, CT scans, or MRI scans. The doctor may also take a small sample of tissue (biopsy) from the affected organ, such as the nose, lung, or kidney, and examine it under a microscope for evidence of granulomas and vasculitis. Inflammation of the blood vessels causes granulomas, which are clusters of inflammatory cells.
Anti-neutrophil cytoplasmic antibody (ANCA) testing, which detects antibodies targeting certain proteins in white blood cells, is one of the blood tests that can help diagnose GPA. There are about 90% of people with these antibodies, but they can also appear in other conditions, such as lupus, rheumatoid arthritis, or infections. A positive ANCA test alone cannot diagnose GPA, and it must be confirmed by other tests and clinical findings.
Inflammation is stopped, organ damage is prevented, and relapse is reduced with treatment of GPA. Corticosteroids, cyclophosphamide, rituximab, azathioprine, methotrexate, or leflunomide are some of the medications available for treating this condition. Doctors need to closely monitor these medications because they can have serious side effects, such as infections, bone loss, diabetes, or cancer. Treatment dosage and duration may need to be adjusted over time based on the severity and extent of the disease.
Occasionally, plasma exchange is used to remove harmful antibodies from the blood and replace them with fresh plasma. This can improve symptoms and outcomes for people with severe kidney or lung damage.
The infections, pain, or breathing problems caused by GPA may require supportive care, such as antibiotics, painkillers, nasal sprays, or oxygen therapy, in addition to medications. Surgery may also be required to repair damage to the nose, sinuses, ears, or eyes, or to perform a kidney transplant if the kidneys fail.
There is no known way to prevent GPA, as the cause is unknown. However, some general measures that can help reduce the risk of complications and improve the quality of life of people with GPA are:
Taking the medications as prescribed and following up with the doctor regularly
Avoiding smoking and exposure to secondhand smoke, as they can worsen the lung damage
Getting vaccinated against common infections, such as influenza and pneumonia, as they can trigger a flare-up of GPA
Eating a balanced diet and drinking plenty of fluids to maintain good nutrition and hydration
Exercising moderately and resting adequately to boost the immune system and reduce stress
Seeking medical attention promptly if any new or worsening symptoms occur
In addition to affecting any organ in the body, GPA can also affect the nose, sinuses, throat, lungs, and kidneys. Symptoms include nosebleeds, coughing up blood, kidney problems, and vision loss. A combination of blood tests, imaging tests, and biopsies can be used to diagnose it, and medication that suppresses the immune system, plasma exchanges, and supportive care can be used to treat it. A timely diagnosis and treatment are essential to prevent organ damage and improve the prognosis of GPA. People with GPA need to follow their treatment plan and take care of their health to avoid complications.
