Summary about Disease
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disorder that causes inflammation of the blood vessels (vasculitis). This inflammation can restrict blood flow to various organs, most commonly the sinuses, lungs, and kidneys. GPA can be life-threatening if left untreated.
Symptoms
Symptoms vary depending on which organs are affected. Common symptoms include:
Nasal and Sinus: Chronic runny nose (often with pus or crusting), sinus pain and infections, nosebleeds, nasal cartilage collapse (saddle nose deformity).
Lungs: Cough, shortness of breath, coughing up blood, chest pain.
Kidneys: Blood in the urine, protein in the urine, kidney failure (can be asymptomatic in early stages).
Other: Fatigue, fever, weight loss, joint pain, skin rashes, eye inflammation (redness, pain, vision changes), hearing loss.
Causes
The exact cause of GPA is unknown. It is an autoimmune disease, meaning the body's immune system mistakenly attacks its own tissues. Genetic predisposition and environmental triggers may play a role.
Medicine Used
Treatment typically involves medications to suppress the immune system. Common medications include:
Corticosteroids (e.g., prednisone): To quickly reduce inflammation.
Immunosuppressants (e.g., cyclophosphamide, methotrexate, rituximab): To maintain remission and prevent relapses.
Trimethoprim-sulfamethoxazole (Bactrim): May be used to prevent certain infections and might have a role in preventing relapses.
Is Communicable
No, Granulomatosis with polyangiitis (GPA) is not communicable. It is not an infectious disease and cannot be spread from person to person.
Precautions
Since GPA itself is not communicable, precautions focus on managing the disease and minimizing risks associated with treatment:
Medication Adherence: Strictly follow prescribed medication regimens.
Regular Monitoring: Attend all scheduled appointments and undergo regular blood and urine tests to monitor disease activity and medication side effects.
Infection Prevention: Immunosuppressant medications increase the risk of infections. Practice good hygiene (handwashing), avoid contact with sick individuals, and get recommended vaccinations (pneumonia, flu). Discuss live vaccines with your doctor.
Lifestyle Modifications: Maintain a healthy lifestyle with a balanced diet, regular exercise (as tolerated), and adequate sleep.
Early Symptom Recognition: Be aware of potential symptoms of relapse and report them to your doctor promptly.
How long does an outbreak last?
GPA is a chronic condition that can have periods of active disease ("outbreaks" or "flares") and periods of remission. An untreated flare can worsen rapidly, leading to organ damage. With treatment, flares can be controlled, and remission can be achieved. The duration of a flare varies depending on the severity of the disease and the effectiveness of treatment. Without treatment it can be deadly.
How is it diagnosed?
Diagnosis typically involves a combination of:
Medical History and Physical Exam: Evaluation of symptoms and overall health.
Blood Tests: To detect elevated inflammatory markers (e.g., ESR, CRP), ANCA (anti-neutrophil cytoplasmic antibodies), and assess kidney function.
Urine Tests: To check for blood and protein in the urine.
Imaging Studies: Chest X-rays or CT scans to evaluate lung involvement. Sinus CT scans for sinus involvement.
Biopsy: A tissue sample from an affected organ (e.g., nose, lung, kidney) is examined under a microscope to confirm the presence of granulomatous inflammation and vasculitis.
Timeline of Symptoms
The timeline of symptoms varies significantly from person to person. Some individuals may experience a gradual onset of symptoms over months, while others may develop symptoms more rapidly. The initial symptoms are often nonspecific (fatigue, fever), which can delay diagnosis.
Early Stage: Nasal and sinus symptoms (runny nose, sinus pain) are common initial symptoms.
Progression: If untreated, the disease can progress to involve the lungs, kidneys, and other organs.
Advanced Stage: Severe organ damage can occur, leading to complications such as kidney failure or respiratory failure.
Important Considerations
Early Diagnosis and Treatment: Early diagnosis and treatment are crucial to prevent organ damage and improve outcomes.
Long-Term Management: GPA is a chronic condition that requires long-term management, including regular monitoring and medication adjustments.
Relapses: Relapses are common, even with treatment. Be vigilant for symptoms and report them to your doctor promptly.
Medication Side Effects: Immunosuppressant medications can have significant side effects. Discuss potential side effects with your doctor and be aware of signs of infection.
Multidisciplinary Care: Management of GPA often requires a multidisciplinary team of specialists, including rheumatologists, pulmonologists, nephrologists, and ENT specialists.
Pregnancy: Women with GPA should discuss pregnancy plans with their doctor, as some medications can be harmful to the fetus.