Wegener’s granulomatosis: Definition, populations at risk and treatment

Rare Diseases: Wegener’s granulomatosis, what is it?

A look.

The first case of this condition was discovered and described by a German medical student 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 different from 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 the kidneys, the lower respiratory tract, and the upper respiratory tract.

What happens during this disease?

This disease affects any part of the body, but there is a predisposition to affect specific 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 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 scarce 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, brain tissues, and the spinal cord. The most visible symptoms are weight loss, fever, and a 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 vital role in this disease.

It is believed that this disease can be caused by infections of some 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, and always looking for common points to detect it under the microscope.

It is essential to be very specific when diagnosing this disease because some diseases can mimic the same symptoms. Before starting treatment, this is necessary to ensure improvements in patients’ quality of life.

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 a treatment?

This disease was the cause of death until the 70s. However, research has shown that steroids such as prednisone are a great relief and helps in patients’ lives.

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 frequent relapses in patients, even when they continue treatment.

Even patients who have remission of this disease suffer temporarily from relapses, which, even under treatment, continue to generate high sensitivity in patients, leading to intensifying treatment and placing more potent drugs.