The Wegener's granulomatosis is a form of inflammation that usually affects the lungs, kidneys and other body organs. People having this infection get their end organs damaged. Sometimes it becomes even more serious requiring long term immune suppression. The syndrome is named after Dr. Friedrich Wegener who described the disease in 1936.
Actually the Wegener's granulomatosis is part of the vasculitic syndromes. It also features the presence of circulating antibody termed as ANCAs. The syndrome usually affects the small and medium sized blood vessels. Other than the Wegener's, this category also comprise the Churg-Strauss syndrome and microscopic polyangiitis.
Symptoms of Wegener's granulomatosis:
The initial symptoms of Wegener's granulomatosis are protein resulting the diagnosis getting severely delayed because of non-specific nature of the symptoms. The first and the foremost symptom of the disease is Rhinitis. Many people have the same signs and symptoms including the following:
- Upper airway, ear and eye disease
- Pain and stuffiness in nose. It also causes rhinitis, nosebleeds, crusting and saddle-nose
- Some patients also report of partial hearing loss due to Eustachian tube dysfunction sensor neural hearing loss
- The eyes develop scleritis, pseudo tumours, uveitis, conjunctivitis and episcleritis
The inhalation is also disturbed causing following disturbances:
- Trachea: subglottal stenosis
- Lungs: Pulmonary nodules, infiltrates, cavitary lesions, pulmonary hemorrhage causing bronchial stenosis.
Patients having Wegener's granulomatosis get progressive segmental necrotising glomerulonephritis in Kidney that causes chronic renal failure. Some patients also report of having arthritis pain or swelling which in initial diagnosis is recognized as rheumatoid arthritis. The skin also develops with the nodules on the elbow, purpura and other parts. Some complain of having sensory neuropathy and mononeuritis multiplex. Disturbances are also noticed in heart, gastrointestinal tract, brain other organs.The Wegener's granulomatosis is usually traced when a patient is having some symptoms for a long period of time.
|
|
If the patient develops renal failure or cutaneous vasculitis the organs obtain the biopsy form. The granulomas are the primary cause for the
appellation of "Wegener's granulomatosis.
Treatment of Wegener's granulomatosis
Before the steroids were available for treating Wegener's granulomatosis, the death rate, in one year was common in 90% of cases. The introduction of cyclophosphamide in the 1970s was a major breakthrough. The initial treatment generally goes with corticosteroids and oral cyclophosphamide.
Some patients having Wegener's granulomatosis severe subglottic stenosis, tracheotomy is required to continue an airway.
To know more about Wegener's granulomatosis you can log on to our web service arthritisxl.com.
|
