Wegener's granulomatosis: what is it, causes, symptoms (photo), treatment

Introduction

Wegener's granulomatosis (now also called polyangiitis granulomatosis) is a rare inflammatory disease that affects medium to small blood vessels. In other words, it is vasculitis.

As a result of the disease, the blood flow through the blood vessels affected by inflammation decreases; the consequences are taken by the organs in the patient's body, which no longer receive the proper amount of blood.

In the early stages of the disease, symptoms of Wegener's granulomatosis appear first in the upper and lower lungs and respiratory tract. Later, especially if not properly treated, the disease can spread to the kidneys, heart, ears and skin.

For proper treatment, corticosteroids and immunosuppressants are essential.

What is Wegener's Granulomatosis?

Wegner's granulomatosis - an inflammatory disease that alters the walls of medium and small blood vessels.

With a decrease in the integrity of the vessel wall, blood flow decreases, and the organs of the body (directly connected to the vessels) are no longer properly supplied with blood.

Usually, the areas of the body most susceptible to Wegener's granulomatosis are the kidneys, lungs, upper and lower respiratory tract, but it is possible that it can occur in other places as well.

Without adequate therapeutic treatment or in the case of late diagnosis, Wegener's granulomatosis can also be fatal, especially when it comes to the kidneys.

Wegener's granulomatosis is a vasculitis

When it comes to vasculitis, we mean inflammation of the blood vessels, both arterial and venous.

Wegener's granulomatosis should be considered as vasculitis, since it affects the arterial and venous vessels of medium and small caliber, as well as some capillary vessels.

Why is the disease called Wegener's granulomatosis?

The term granulomatosis comes from the fact that the disease is characterized by the formation granulomas. Granulomas are proliferations of connective tissue, limited and nodular in shape, their origin may be different: with Wegener's granulomatosis, the presence of granulomas is undoubtedly associated with inflammatory condition.

The term Wegener comes from the first doctor who described the disease: Frederick Wegener.

The disease, however, is also known as granulomatosis with polyangiitis, where the term polyangiitis indicates an inflammatory process involving several blood or lymph structures of the vessels.

Epidemiology

According to British statistics, about 500 people suffer from Wegener's granulomatosis every year.

The disease does not favor a particular gender, although it appears to occur for a currently unknown reason in people with fair skin.

Wegener's granulomatosis can develop at any age, but, according to some epidemiological data, it is more common in adults aged 40 to 60 years.

Causes

Currently, the exact cause of Wegener's granulomatosis remains unknown.

However, given the characteristics of the disease, it is believed that it may have autoimmune origin. Autoimmune diseases - these are all those diseases that are characterized by an exaggerated and abnormal response of the immune system; while the immune system in a healthy person is the body's protective barrier against external aggressions (bacteria, viruses, etc.), in people with autoimmune diseases the immune system mistakenly attacks the healthy tissues of the body.

Genetics and environment

According to some theories (but so far without any evidence), it seems that in order to provoke an exaggerated autoimmune response, the simultaneous presence of two factors is necessary, the first is genetic and the other is ecological.

The genetic factor means the patient's predisposition to the development of Wegener's granulomatosis.

An environmental factor means an infection caused by a viral or bacterial agent (in particular, the etiological agent is still unknown).

In the absence of any of these factors, the disease does not occur.

Symptoms and complications

Symptoms and signs of Wegener's granulomatosis may appear suddenly or take several months before manifesting themselves. Therefore, each patient is a separate case.

The first organs and areas of the body that will be responsible for the effects of inflammation at the level of blood vessels are upper respiratory tract (nose, mouth, paranasal sinuses and ears), lower (larynx, trachea and bronchi) and lungs (cm. Photo).

As soon as it appears in these places, the disease can spread and affect other vessels, especially those that supply kidneys.

A patient with Wegener's granulomatosis may complain of the following symptoms:

• persistent nasal discharge with pus;
• nosebleeds (nosebleeds);
• sinusitis;
• ear infections;
• coughing with or without bleeding (hemoptysis);
• chest pain;
• dyspnea;
• general malaise
• weight loss for no reason (more precisely, the cause and may be granulomatosis with polyangiitis);
• joint pain and swelling;
• blood in urine (hematuria);
• skin irritation, ulcers (see. photo above);
• redness, burning, and pain in the eyes;
• fever.

When to see a doctor?

The hallmarks of Wegener's granulomatosis are permanent runny nose (even after medication), epistaxis and hemoptysis. Therefore, if such symptoms occur, it is advisable to see a doctor immediately.

Carelessness and neglect of health can cause great complications.

Complications

Complications occur when the inflammatory condition caused by Wegener's granulomatosis spreads to other parts of the body. Possible targets: kidneys, eyes, ears, spinal cord, heart, and skin.

• Loss of hearing. The inflammation of the blood vessels reaches the middle ear, causing more or less permanent hearing loss.
• Cutaneous vasculitis and cutaneous manifestations. Cutaneous vasculitis is characterized by red spots on the body; other signs, on the contrary, may appear, for example, in nodules at the level of the elbows.
• Heart attack (myocardial infarction). This occurs when inflammation reaches the coronary arteries (arteries of the heart). In fact, under such circumstances, the blood supply drops and the heart cells (myocardium) die, resulting in decreased heart activity. The main symptoms are chest pain, shortness of breath, sweating, heartburn, etc.
• Kidney damage and subsequent renal failure. The less blood flows to the kidneys, the more irreversibly damage the kidneys begin to damage and stop working properly. This causes the accumulation of those substances that are toxic to the body, which are usually filtered and removed by the renal system. The final phase of this very slow process - and, unfortunately, despicable (due to symptoms that are not always obvious initially) - is renal failure, characterized by severe uremia (that is, a high level of nitrogenous substances in the blood). Renal failure is the leading cause of death in patients with Wegener's granulomatosis.
• Anemia. This disease can develop over time. Many patients with Wegener's granulomatosis have anemia.
• Deep vein thrombosis and pulmonary embolism. They can appear as early pulmonary disorders.

Diagnostics

A definite diagnosis of Wegener's granulomatosis is established only after a long series of precise studies.

Doctors begin, as is often the case, with objective examination, during which the symptoms and signs manifested by the patient are analyzed and his medical history.

Doctors then move on to more thorough and specific checks, such as blood and urine tests, chest x-ray.

If there is still doubt at the end of this diagnostic process, the doctor may biopsy the organs or tissues involved; the presence of granulomas and signs of vasculitis speak of only one thing: Wegener's granulomatosis.

Blood test

A blood sample taken from a patient can be subjected to the following tests:

• Antineutrophil Cytoplasmic Antibody (ANCA) Assay. ANCA is present in the blood of many patients with Wegener's granulomatosis, but not all. This makes this analysis not 100% reliable.
• Analysis erythrocyte sedimentation rate (ESR). This study helps to assess the rate at which red blood cells settle to the bottom of the tube. The faster they settle, the easier the inflammation proceeds. It is not 100% reliable, as many other inflammatory diseases cause the same reactions.
• Anemia test. It is also not a 100% reliable test because not all patients with Wegener's granulomatosis are anemic.
• Level analysis blood creatinine. Used to assess kidney function. This is an effective method of research if the disease has affected the kidneys; otherwise, erroneous conclusions may be drawn.

Analysis of urine

V urinoanalysis contains information related to kidney health. The presence of blood and proteins may indicate a kidney disorder.

X-ray

A chest X-ray shows the condition of the lungs. Some of their abnormalities can be recognized, but it is not possible to establish exactly whether this is Wegener's granulomatosis or another pulmonary disease.

Biopsy

A biopsy is the safest and most informative clinical test.

It is performed under local anesthesia and involves the removal of a piece of tissue from the affected organ for observation under a microscope. At the same time, the presence of granulomas or signs of vasculitis in the tissue under study is beyond doubt.

Treatment of Wegener's granulomatosis

Thanks to early diagnosis and appropriate treatment, the acute phase of Wegener's granulomatosis passes after a few months.

After this first period has been passed, it is necessary supportive therapy, which lasts about 18-24 months, since a complete cure takes a long time. Neglecting this aspect means exposing yourself to relapses.

Drug treatment

The most commonly used drugs are corticosteroids, immunosuppressants, and a monoclonal antibody called rituximab.

Corticosteroids powerful anti-inflammatories that are actually used to reduce inflammation. These drugs can cause numerous side effects, which explains why they are prescribed at the lowest effective dose.

In patients with Wegener's granulomatosis, the most widely used corticosteroid is Prednisone.

Immunosuppressive drugs reduce inflammation by acting on the immune system, which, as mentioned, in the case of Wegener's granulomatosis, begins to attack the body. Cyclophosphamide, Azathioprine or Methotrexate belong to this category of drugs. Taking immunosuppressants puts the patient at a greater risk of contracting infections.

Rituximab — monoclonal antibody, which reduces the activity of group B lymphocytes, cells of the immune system that cause inflammation. Its use in cases of Wegener's granulomatosis has also been approved by the FDA (United States Food and Drug Administration).

What to do with drug side effects?

To prevent the side effects of the aforementioned drugs, your doctor will prescribe or recommend:

• Sulfamethoxazole, in combination with Trimethoprimto prevent lung infections.
• Drugs bisphosphonates, calcium and on vitamin D based supplementsto reduce or prevent osteoporosis.
• Folic acid, to prevent the consequences of treatment with methotrexate.

Other therapies

Certain circumstances, such as late diagnosis of the disease or carelessness in treatment, may require plasmapheresis and surgery.

Plasmapheresis is a rather complex procedure that is performed with the patient's blood (more precisely, the procedure for taking blood, cleaning and returning it or some part of it back into the bloodstream). This is practiced not only in the case of Wegener's granulomatosis, but also in the presence of other autoimmune diseases such as systemic lupus erythematosus.

In turn, surgery may be required for kidney transplantif the kidneys are irreparably damaged, or to correct airway complications or problems with the middle ear.

Forecast

For those with Wegener's granulomatosis, the prognosis will be good under the following conditions:

• early diagnosis of the disease;
• proper care of the acute phase of the disease;
• maintenance therapy of the desired duration.

If these three basic conditions are met, duration and the quality of life guaranteed. It is true that some irregularities and side effects can be caused as a result of prolonged use of medication or periodic blood tests (tests) (to see how inflammation), but this is nothing compared to the complications (renal failure, severe respiratory disease, heart attack, etc.) of advanced or poorly treated granulomatosis Wegener.