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Wegener’s disease Symptoms and Treatment

What is it ?

Wegener’s disease, or Wegener’s granulomatosis is a rare disease affecting the blood vessels. It is an infection of the wall of these vessels (vasculitis).

This vasculitis causes difficulties in the blood circulation and the feeding of the various organs and tissues with oxygen and nutrients. This can lead to the development of damage to tissues and depleted organs.
Late management or failure to treat the disease may lead to the death of the patient.
However, effective treatments exist in the treatment of Wegener’s disease, allowing subjects suffering from this pathology to live in “normal” living conditions.

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The phenomenon of inflammation is characterized by an accumulation of cells at the site of the inflamed, which results in a cell cluster. These cell clusters are also called “granulomas”. These granulomas are all the more observable in the nose and lungs. In this sense, Wegener’s granulomatosis mainly affects the respiratory tract and the kidneys. Nevertheless, other organs can suffer a deficiency in their blood supply, this is the case for the eyes, the heart, the skin, the joints and the nervous system.

This pathology therefore associates inflammation of the walls of the blood vessels and granulomatosis. The main symptoms associated with this are ENT (otolaryngology), pulmonary and renal diseases.

Wegener’s disease is also known as granulomatosis with polyangiitis (GPA).

The prevalence (number of subjects affected by this disease) is estimated at 3/100 000.
The disease affects both boys and girls (with a slight male predominance according to some studies and is growing on average around 45 years old. However, clinical forms differ according to age.

Symptoms of Wegener’s disease

Wegener’s disease, or Wegener’s granulomatosis, is defined by inflammation of the blood vessels. This phenomenon of inflammation is the cause of a thickening of the walls of these vessels. The circulation is thus slowed down by a narrowing of the circulation area. Oxygen and nutrient inputs are therefore reduced for some tissues and / or organs.
As a result, ischemia (decrease or even arrest of arterial circulation in an organ or tissue) is observable.
The presence of granulomas (cell clusters) in the initially inflamed part contributes to the increase of this inflammation.

Although many organs and / or tissues may be affected by this pathology (the nervous system, the skin, the heart and the intestines), the inflammation of the blood vessels particularly concern the nose, sinuses, ears, lungs and the kidneys.

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The first clinical signs observed are similar to a cold or sinusitis: sinus pain, nasal discharge, congestions of the ears, fatigue, added to respiratory symptoms such as coughing, shortness of breath, and others.
If these different symptoms are not alleviated by cold treatments, these can be a source of Wegener’s disease.

Other symptoms are characteristic of granulomatosis:
– persistent fever;
– night sweats;
– intense fatigue resulting in general weakness;
– loss of appetite resulting in weight loss;
– joint pain and body aches;
– ear infections reducing hearing;
– frequent sinusitis;
– recurrent nasal bleeding;
– chest pain;
– coughing and breathing difficulties;
– blood coughs and the presence of blood in the phlegm;
– hematuria (presence of blood in the urine).
Some people with this pathology may develop blood clots, especially in the legs (risk of venous thrombosis, phlebitis, etc.) or in the lungs (risk of pulmonary embolism).

The origins of the disease

The origins of Wegener’s disease are, so far, still unknown.
The hypothesis currently put forward is that it would be an autoimmune disease, the aggression of the body by its own immune system. It is also likely that there are certain genes that make the person more susceptible to the development of the disease.

Wegener’s granulomatosis can also be triggered by other factors, including bacterial or viral infections.

The disease is not hereditary. Indeed, no gene taken independently would be responsible for the development of the disease. The development of the disease in many people within a family is rare.
In addition, some very exceptional cases of people with the disease within the same siblings have been identified. Certain genes predispose to the development of the disease. However, they are still unknown today.

Risk factors for Wegener’s disease

Since the origins of Wegener’s disease are not clearly identified, the risk factors for a person to develop the disease remain difficult to identify.

Indeed, even if in very rare cases, several people can be reached within the same family, it is not a hereditary disease. In this sense, genetic transmission does not make it a risk factor.

Some studies have shown that infectious factors may be at the origin of the development of this pathology. Either a microbial infection could be a risk factor for developing the disease. However, this represents only a minimal probability.

Current research on Wegener’s granulomatosis therefore focuses on a better understanding of the origins of the disease, risk factors and a further understanding of the mechanisms of action related to the disease.

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Prevention and treatment of Wegener’s disease

The diagnosis of Wegener’s disease can be made following a blood test.
The presence in the blood of certain antibodies: cytoplasmatic antineutrophilic antibodies (ANCA) are significant for the disease.
Nevertheless, the blood test of some patients with granulomatosis does not demonstrate the presence of this type of antibody. Additional examinations are therefore necessary.

Since pulmonary involvement is a significant sign of the disease, a chest x-ray is often prescribed in parallel with the blood test.

The presence of blood in the urine is also a symptom related to Wegener’s disease, a urine test may also be prescribed in this context. Usually the collection of urine is done over 24 hours to determine any renal abnormalities.

A biopsy can be performed as part of a confirmation of the diagnosis: lung biopsy (preceded by bronchoscopy), kidney biopsy, mucosal biopsy of the nose, skin biopsy or biopsy of the upper airways.

Finally, a sinus or chest CT scan can be performed to diagnose the disease.

The diagnosis of the disease must be made at the earliest to treat the symptoms as soon as possible and thus avoid lethality.

To date, no cure for the disease as a whole exists. Nevertheless, in order to limit the inflammatory reaction of the blood vessels as well as to treat the main symptoms and to limit complications, drugs may be prescribed. It is mainly a combination of anti-inflammatories (corticosteroids) and immunosuppressants.

This symptomatic treatment and to avoid the repercussions is generally taken over a long period (a few years). Their administrations must be supervised by a general practitioner or specialist.

In severe disease, long-term antibiotic therapy may be prescribed to prevent microbial infections.
Plasmapheresis (transfusion of healthy plasma) may be recommended for major renal impairment or renal transplantation.

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