Granulamatosis with polyangiitis, a rare disorder

Granulomatosis with polyangiitis or GPA, formerly known as Wegener’s granulomatosis–WG, is a systemic disorder involving both granulomatosis and ployangiitis, a form of vasculitis or inflammation of blood vessels, affecting small and medium size vessels in various organs.

Granulomatosis with polyangiitis is a rare disorder in which the walls of the blood vessels tend to get inflamed or swollen and leaky leading to poor blood flow to the issues in the body. It is a serious ailment which needs to be treated and could be fatal if not treated in time.

It can cause a wide range of symptoms like frequent sinusitis, persistent fever, fatigue and weakness, loss of appetite and weight loss, joint pain and soreness, persistent ear infections and problem in hearing, coughing out blood or blood in the phlegm and in urine, chest pain, cough or shortness of breath, recurrent nosebleeds or sores around the nose, general feeling of sickness and night sweats.

Vasculitis or inflammation of the Blood Vessels

Some may be at a risk of having blood clots in the leg or deep vein thrombosis or in the lung, pulmonary embolism.

Vasculitis or the inflammation of the blood vessels caused by granulomatosis with polyangiitis affects mainly the nose, sinuses, lungs, ears and kidney though other organs could also be affected in due course including the brain, nerves, heart, bowels and the skin and could also lead to tissue damage and organ failure eventually.

Symptoms may start any age even in childhood though the condition is usually seen in middle age between the age of forty and fifty.

Autoimmune Disease

The cause of GPA is not known but it is presumed to be an autoimmune disease meaning that the body’s immune system can mistakenly attack its own tissues. Just in the case of other autoimmune conditions, it could be that people with GPA have a gene which makes them more likely to be the victim of this ailment and could be triggered by some other factor such as infection with virus or bacteria. The genes alone cannot be responsible for this condition and it is very unlikely for two people in the same family to suffer from GPA.

Anti-Neutrophil-Cytoplasmic Antibodies

GPA is usually traced when a person has some unexplained symptoms for a long period of time and determination of Anti neutrophil-cytoplasmic antibodies (ANCAs)which are antibodies that the immune system of people with GPA use to attack the body’s own blood vessels, can assist in the diagnosis.

Blood test can be done to check the levels of ANCA though these testsat times may show signs of negative in people with GPA and hence it cannot be relied upon in diagnosing the condition. Besides this, a tissue sample of the affected area may also be conducted and viewed under the microscope for signs of inflammation wherein in GPA; the white blood cells tend to clump together forming tiny lumps known as granulomas.

 Urine test may also be done to check for blood or protein in the urine with indication that GPA may have affected the kidneys. Other test may also include an x-ray of the chest to check if the lungs have been affected, a Computed tomography CT scan of the sinuses, nose and ears and an MRI – magnetic resonance imaging scan of the affected organ.