Wegener’s Granulomatosis is a type of vasculitis, inflammation (redness, heat and swelling) of blood vessels. Vasculitis is an autoimmune disorder where the immune system attacks its own blood vessels causing inflammation. This results in narrowing or closing of the blood vessels. It may sometimes lead to stretching and bulging of the blood vessels (aneurysm) which may burst and bleed and can lead to death. Vasculitis can occur at any age and causes serious complications depending on which organ systems are involved.
Symptoms
Depending on the part of the body that is affected, symptoms will vary. The areas affected with vasculitis are listed below with their respective symptoms:
- Skin – various kinds of spots and bruises
- Joints – Arthritis
- Lungs – shortness of breath and sometimes blood with coughing (haemoptysis)
- Gastrointestinal tract – mouth sores, stomach pain and intestinal wall bleeding; in severe cases it can be fatal
- Nose and ear – sinus or nose ulcers and middle ear infection or hearing loss in some cases
- Eyes – redness, blurred vision, sensitivity to light and even blindness in rare cases
- Brain – headache, stroke-like symptoms and even paralysis in severe cases
- Nerves – Numbness in different parts of the body and sharp severe pain in the hands and legs
Other general symptoms can include fever, weight loss, aches and pain. The condition may suddenly go in remission or flare up in certain cases.
Risks
Vasculitis is mostly linked to other auto-immune disorders and may occur in patients suffering already with lupus, scleroderma or rheumatoid arthritis. It may also co-occur with leukaemia and lymphoma. Smoking and hepatitis B and C infection also increases the risk for developing vasculitis.
Diagnosis
Diagnosis is done by the doctor based on the medical history, physical examination, symptoms and result of various diagnostic tests. Tests that the doctor orders to confirm vasculitis may include:
- Blood test for Erythrocyte sedimentation rate, C-reactive protein, haemoglobin and haematocrit and antineutrophil cytoplasmic antibodies
- Biopsy of the affected blood vessel or organization
- Blood pressure
- Urine-analysis
- ECG
- Echocardiography
- Chest X-ray
- Lung function test
- Abdominal ultrasound
- Angiography of affected blood vessels
Treatment
There is no cure for vasculitis therefore treatment is aimed at reducing pain and inflammation by prescribing non-steroidal anti-inflammatory drugs and corticosteroids. In severe cases cytotoxic drugs are given to kill the immune cells involved in inflammation. Rarely surgery is required to repair aneurysms that can occur.
Support of family and friends is equally important for the patient to deal with this chronic disease.
Changes in BMI Associated with Improvements in Disease Activity & Glucocorticoid Treatment
Patients with antineutrophil cytoplasmic antibody–associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), are often prescribed high doses of glucocorticoids and immunosuppressive medication, such as rituximab. Glucocorticoid treatment is commonly associated with weight gain. Despite the well-established link between glucocorticoid use and cardiovascular risk, weight gain is not always an adverse event due to the risks of weight loss associated with increased catabolic activity experienced by patients with these inflammatory conditions.
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