Rare Diseases: Wegener’s granulomatosis, what is it?
The first case of this condition was discovered and described by a German medical student, named Heinz Klinger in 1931.
After several years, another German doctor, the pathologist Friedrich Wegener, discovered and scored another 3 cases and recognized that this new disorder was a different form to vasculitis. This disease is also known as the “Wegener’s disease”.
It was described that this disease causes vasculitis and necrosis in the affected organs, which are frequently the kidneys, the lower respiratory tract and the upper respiratory tract.
What happens during the course of this disease?
This disease affects any part of the body, but there is a predisposition to affect certain organs. Generally the most affected organs are the trachea, nose, paranasal sinuses, ears, kidneys and lungs.
What are the populations at risk?
This disease occurs almost equally in both men and women, with only a slight predominance in the male population.
It usually begins during middle age, but there may be cases in people of all ages. It is very rare to occur during childhood, as it is also usual for a patient between 70 and 80 years to be diagnosed with this condition.
This condition affects several organs at the same time as described, it can even affect in many cases the urinary tract, the protective layers and brain tissues, as well as the spinal cord. The most visible symptoms are weight loss, fever and generalized state of fatigue.
The causes for which this disease affects people are still unknown, and it has even been shown that the genetic and hereditary factor does not play a very important role in this disease.
It is believed that this disease can be caused by infections of some type of fungus, virus or bacteria, but to date they have not been identified.
How is Wegener’s granulomatosis diagnosed?
The first way to diagnose this condition is through biopsy of the affected organ, looking for similarities in the characteristics of the disease of the organs with this disease, always looking for common points to detect it under the microscope.
It is important to be very certain when diagnosing this disease because there are some diseases that can mimic the same symptoms, and this is necessary before starting a treatment, all to ensure improvements in the quality of life of patients.
Although the most affected organs are the nose, paranasal sinuses, trachea and ears, and being the biopsy of these organs a non-invasive procedure, generally the biopsies in these organs do not give accurate results, so doctors must resort to other organs where Biopsies are more invasive.
Is there really a treatment?
This disease was the cause of death until the 70s. However, research has shown that the use of steroids such as prednisone is a great relief and help in patients’ lives over a period of time.
Since the 1960s, the diagnosis of this disease has ceased to be fatal, in order to find alternatives that would extend the life of patients, proving that an average of 90% of patients responded positively to treatments, and a total of 75% of patients the patients were able to achieve remission.
However, this condition has a large number of frequent relapses in patients, even when they continue their treatment.
Even patients who have achieved remission of this disease, suffer temporarily from relapses, which even under treatment, continue to generate high sensitivity in patients, and this leads to intensify treatment, placing more powerful drugs.