Granulomatosis With Polyangiitis

Overview

Granulomatosis with polyangiitis is an uncommon condition in which blood vessels become inflamed. It usually affects the ears, sinuses, nose, lungs and kidneys. This condition slows blood flow to the organs and can be fatal if left untreated.

Signs and Symptoms

Fatigue, Fever, Joint pain, Weight loss, Stuffy nose, Crusts around the nostrils, Nosebleeds, Sinusitis, Hearing problems, Earache, Cough, Wheezing, Shortness of breath, Chest pain, Skin rashes, Eye redness or pain, Double vision, Hematuria, High blood pressure

Common Causes

Cause of granulomatosis is unknown, May be due to an immune system problem leading to inflammation, Bacterial and viral infection can be an initiating factor of the autoimmunity, Inflammation in various tissues but primarily the kidneys, blood vessles and respiratory tracts,

Risk Factors

People between the ages of 40 and 65, Bacterial infection, Viral infection, White ethnicity, Genetic predisposition

Investigation Techniques

Medical history, Physical examination, Blood tests including full blood count, kidney function tests, C-reactive protein, erythrocyte sedimentation rate, and anti neutrophil cytoplasmic antibodies, Urinalysis, Chest x-ray, Computed tomography (CT) scan of the chest, Magnetic resonance imaging (MRI) scan, Biopsy

Treatment and Prevention

Oral or injectable medications such as prednisone, cyclophosphamide, methotrexate, azathioprine, mycophenolate and rituximab, Plasmapheresis, Nasal irrigation, Nasal corticosteroids, Antibiotics, Surgical repair for nose deformity or perforation of nasal septum, Kidney transplant, Pneumocystis Pneumonia prophylaxis