Full review of Wegener's granulomatosis disease: causes, types, treatment

In our article today we will talk about an extremely rare and severe disease like Wegener's granulomatosis. Its diverse clinical manifestations, the difficulties of diagnosis and treatment.

Contents of the article:

• Reasons
• Forms and symptoms of the disease
• Diagnosis
• Treatment methods
• Forecast

Wegener's granulomatosis is called the most complex autoimmune disease in which small and medium-sized vessels are affected. Fortunately, the disease is rare. The average incidence in the world is about 3-4 episodes per 100 000 population, but these figures may be higher due to the difficulties of setting a specific diagnosis. Many patients are already posthumously diagnosed with this diagnosis, and during the course of their life the symptoms of the disease are mistaken for very different diseases. Representatives of both sexes are equally sick, and for the first time the disease usually appears at the age of 30-50 years. We will try to understand in more detail what is happening with this disease.

Wegener's granulomatosis refers to the so-called autoimmune vasculitis - inflammatory vascular disease. It is believed that inflammation occurs against the background of the invasion of pathogenic viruses or bacteria. In the case of autoimmune diseases, the body itself becomes the cause of inflammation, cells of the immune system of which, for as yet obscure reasons, "declare a hunt" for their own organs and tissues. In the case of Wegener's granulomatosis, the walls of small vessels become the target of immune cells: arterioles, venules, capillaries. Inflammation in the walls of these vessels is accompanied by the formation of specific nodules - granulomas, which as the process progresses die and degenerate into connective tissue - there are the processes of fibrosis and sclerosis. Of course, the vessels in this case quickly lose their function, and the organ fed by them begins to suffer and die.

The disease is characterized by a sudden onset, rapid and even lightning development and extremely bad consequences. Without proper treatment, the patient dies within 6-12 months. On the background of therapy the patient can live 5-10 years and more.

As a rule, a rheumatologist deals with the treatment of autoimmune diseases, but on the way to it the patient faces a huge number of doctors of related specialties: otorhinolaryngologists, nephrologists, urologists, pulmonologists, phthisiatricians and even oncologists. Symptoms of this pathology are so multifaceted that they seize many organs and simulate a huge number of diseases. About the clinical manifestations and forms of the disease, we'll talk just below.

Causes of pathology

Strictly speaking, the causes of all autoimmune diseases are for certain unknown. However, scientists around the world have identified a certain relationship between the aggression of their own immune system to their native cells( these are common causes for all autoimmune diseases):

Infectious mononucleosis is one of the possible causes of the development of Wegener's granulomatosis

Forms and symptoms of

It is very important to mention that Wegener's granulomatosis has some favored localizations in which several forms or localizations of the disease are created:

The first symptoms of Wegener's granulomatosis are not very specific. Patients may complain of general weakness, fatigue, a slight increase in temperature, pain in the muscles and joints, poor appetite and weight loss. Similar symptoms are typical for any cold or chronic fatigue syndrome, so most patients do not attach much importance to them. Specific manifestations of the disease appear somewhat later.

Most often, the disease begins with an otorhinolaryngological form or eye injury, and after a while - for several months - develops one or another generalized form, which becomes lethal. Let's go through each of the clinical forms of the disease.

Otorhinolaryngological form

The most typical inflammation of ENT organs is inflammation and subsequent necrosis of the walls and partitions of the nose. Initial symptoms can proceed according to the type of inflammation of the nose - rhinitis, inflammation of the paranasal sinuses - sinusitis.

Ear lesions occur less often, which are manifested by chronic and not responding to conventional otitis media. A prolonged course of the disease leads to the development of deafness.

Ophthalmic form

Eye damage often becomes the first typical sign of Wegener's granulomatosis. The eye shape is characterized by the appearance of inflammation of the sclera of the eye - scleritis and the choroid of the eye - uveitis. The disease is characterized by a marked reddening of the eye, eyelid edema, pain and burning sensation. Against the backdrop of these processes, there is a decrease in vision up to complete blindness.

Skin form

Skin manifestations of autoimmune vasculitis are called purpura. Purple is characterized by the appearance of dense red or crimson spots of various sizes, slightly rising above the surface of the skin. In fact, these spots are the result of hemorrhages from damaged vessels under the skin.

Eruptions are painless, can merge with each other, sometimes - spontaneously disappear. In rare cases, against the background of secondary infection, the sites can be subjected to inflammation and necrosis. Sometimes ulcers appear on the skin.

Such skin lesions should be distinguished from a huge number of other vasculitides - kA autoimmune, and related to the blood coagulation system and the pathology of the vascular wall.

Purpura is a cutaneous manifestation of the pathology

Pulmonary form

The onset of the disease can proceed according to the type of bronchitis or tracheitis, accompanied by a dry, low-productivity cough.

In pulmonary tissue, the same granulomas or nodules are formed, which eventually decompose. When the bacterial infection is attached, the granulomas are transformed into cavities of purulent decay, sometimes their opening is accompanied by pulmonary hemorrhage. Complaints of patients are joined by a cough with blood or pus, shortness of breath, chest pain.

In some patients, such complaints may be absent, so a diagnosis can be assumed based on an X-ray of the lungs. Unfortunately, the primary diagnosis is most often tuberculosis or lung cancer. Death can come from acute respiratory failure or pulmonary hemorrhage.

Renal form of

This is perhaps the most serious and dangerous form of Wegener's syndrome, leading to mortality and severe disability of patients. Kidneys, in fact, are a kind of ball of small vessels surrounded by parenchymal tissue.

These very vessels are very often affected by the disease, causing a specific lesion of the kidneys - glomerulonephritis. Glomerulonephritis can be caused by various factors, including streptococcus toxins, poisons, drugs, but in the case of Wegener's syndrome, the disease proceeds with lightning speed, with rapid onset of renal failure.

Most of the patients present the following complaints:

Unfortunately, very often kidney disease is detected already at the stage of development of chronic kidney failure, with damage to the heart and other internal organs by the products of the decomposition of one's own organism, which dying kidneys are unable to excrete in the urine.

Neuropathic form

The disease mainly affects the peripheral nerve endings - there is polyneuropathy. This condition is characterized by the following symptoms:

This form of the disease is distinguished from many other neuropathies: from banal vitamins B to multiple sclerosis.

Diagnosis

As we have already understood, the diagnosis of Wegener's granulomatosis is an extremely difficult task. It must be distinguished from malignant tumors, kidney disease, tuberculosis, sarcoidosis, blood diseases, a variety of complex forms of otitis, sinusitis, uveitis, as well as the large number of other autoimmune diseases such as lupus, hemorrhagic vasculitis, Gudpatchera syndrome.

For the initial stages of diagnosis, the following studies are needed:

• Clinical blood and urine tests can indicate the presence of an inflammatory process, anemia, detect traces of protein and blood in the urine, a change in its relative density.
• Biochemical blood test will indicate the signs of kidney and liver damage.
• X-ray and computer tomography studies will indicate the presence of granulomas and foci of disintegration in the lungs and bronchi.
• Ultrasound examination of the abdominal organs and kidneys will indicate the damage to the kidneys, a decrease in their blood flow.
• Additional urological tests such as excretory urography, scintigraphy will indicate the degree of decrease in kidney function.
• Consultations of related specialists: ENT doctor, ophthalmologist, dermatologist, urologist, nephrologist, pulmonologist will help to exclude other similar diseases.

Scintigraphy is optional, but one of the additional methods for diagnosing Wegener's granulomatosis. In scintigraphy in the organism is introduced a small kollichestvo radioactive substance, which then, emitting radiation, produces an image on a gamma imager

main specific test that with the highest degree of probability indicate exactly on Wegener's granulomatosis is a blood test for the presence of classical anti-neutrophil cytoplasmic antibodies( Kanz).These are special antibodies that attack cells of the immune system - neutrophils. Such antibodies are detected with several autoimmune vasculitides, including Wegener's disease.

Treatment Methods

Given that the disease has an autoimmune nature, the main group of drugs for the treatment of Wegener's disease are immunosuppressants - drugs to suppress immune defense.

Currently, there are two main groups of immunosuppressants:

These drugs affect the "raging" immunity, suppressing its reaction. Unfortunately, in addition to this link, drugs reduce the general resistance of the body to infections, the growth of skin cells and mucous membranes, disrupt the metabolism.

The earlier the disease is detected and confirmed, the earlier the therapy is started, the better the predictions of the patients. Patients should receive treatment consistently. Doses and the mode of administration of drugs are selected by a rheumatologist. Some patients are well suited to the so-called pulse therapy - the introduction of drugs in large doses once or twice a week.

Particularly complex are patients with renal forms of the disease and developed renal failure. For their treatment, higher doses of drugs are used. Many of the patients are shown lifelong hemodialysis - purification of blood with the aid of the "artificial kidney" apparatus.

To such groups of patients are absolutely contraindicated any means for stimulating immunity, even plant origin, alcohol, many groups of antibiotics and cardiac drugs.

Prognosis for

As a whole, the disease is not favorable for Wegener's granulomatosis. Without proper treatment, patients die within the first year of the disease. About 90% of patients against the background of timely treatment begun, the average life expectancy is 5 years or more.

The greatest complexity for treatment is pulmonary and especially renal forms, with a total survival of less than 4 years.