Granulomatosis polyangiitis, (Wegener Granulomatosis) A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

This book describes Granulomatosis with polyangiitis (Wegener’s Granulomatosis), Diagnosis and Treatment and Related Diseases
Granulomatosis with polyangiitis (Wegener's granulomatosis) is a disease that causes the small blood vessels (arterioles and capillaries) in the body to become inflamed.
Granulomatosis with polyangiitis (GPA) can lead to damage in major organs of the body.
It was formerly known as Wegener's granulomatosis.
Particularly, lungs, throat, nose, sinuses and kidneys become affected.
In the beginning, the symptoms are often vague, such as runny nose or prolonged otitis media that do not recover with standard treatment.
Symptoms from the lungs later develop with cough and breathing difficulty.
Mild fever, fatigue and general malaise are common.
Some people get joint pain, swelling around an eye or skin rash. ‘
Disease of the kidney is normally present late in the course of the disease.
The disease can be serious and life threatening and prompt treatment is important.
GPA is most frequent in middle-aged adults of northern European race.
It is infrequent in children, but has been observed in infants as young as 3 months old.
Causes
The exact cause is not known but it is an autoimmune disorder.
Granulomatosis with polyangiitis manifests itself as a so-called autoimmune disease, meaning that the immune system mistakenly attacks the body's own tissues.
Granulomatosis with polyangiitis is a disease that can become life threatening if not treated.
With the treatment methods available today, most patients recover.
Some will relapse and must be treated again.
Symptoms
Frequent sinusitis and bloody nose are the most frequent symptoms.
Other early symptoms are:
1. A fever that has no clear cause,
2. Night sweats,
3. Fatigue, and
4. A general ill feeling (malaise).
Other frequent symptoms may be:
1. Chronic ear infections
2. Pain, and sores around the opening of the nose
3. Cough with or without blood in the sputum
4. Chest pain and shortness of breath as the disease progresses
5. Loss of appetite and weight loss
6. Skin changes such as bruises and ulcers of the skin
7. Kidney problems
8. Bloody urine
9. Eye problems ranging from mild conjunctivitis to severe swelling of the eye
Diagnosis
When the doctor suspects the disease, it may be because of medical examination of the body showed presence of signs of the disease.
It will have blood tests and various imaging studies.
Detection of specific types of antibodies (cANCA), high blood sedimentation rate, or renal impairment strengthens the suspicion of the diagnosis.
A chest x-ray will be done to look for signs of lung disease.
Urinalysis is done to look for signs of kidney disease such as protein and blood in the urine.
Blood tests are done to rule out other illnesses:
1. Antinuclear antibodies
2. Anti-glomerular basement membrane (anti-GBM) antibodies
3. C3 and C4, cryoglobulins, hepatitis serologies, HIV
Tissue samples can help to confirm the diagnosis.
Treatment
It is essential to medically treat the patient for him or her to become healthy.
The treatment is lengthy, often lasting 2-3 years.
The patient will probably be treated with glucocorticoids
These are given along with other immunosuppresants that reduce down the immune response.
Medicines used with prednisone to treat GPA are:
1. Cyclophosphamide with high-dose glucocorticoids (criterion standard)
2. Rituximab
3. Methotrexate (oral or subcutaneous)
Plasma exchange may be indicated in patients with rapidly progressive renal disease
For the next two years the doctors normally used a maintenance chemotherapy, methotrexate.
Folic acid tablets are given to prevent deficiency.

TABLE OF CONTENT
Introduction
Chapter 1 Granulomatosis Polyangiitis (Wegener Granulomatosis)
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Vasculitis
Chapter 8 Microscopic Polyangiitis
Epilogue