Wegener's granulomatosis: causes of the disease, symptoms, treatment

Causes and course of the disease

Currently, the causes of this disease are not fully understood, but most researchers are of the opinion that it is an autoimmune disease, in the occurrence of which viruses such as cytomegalovirus and herpes virus play an important role.
An equally important factor is the genetic peculiarity of the immune system, especially since with this disease in the blood of patients there is an increased content of the HLA-A8 antigen, which is responsible for the genetic predisposition to the development of autoimmune diseases. In the pathogenesis of Wegener's granulomatosis, immunological disorders such as deposition of immune complexes in the walls of blood vessels and impaired cellular immunity are of great importance. Patients experience necrotizing vasculitis, which affects medium- and small-caliber arteries and is accompanied by the formation of polymorphic cell granulomas that contain giant cells.

Prevention

We can only talk about secondary prevention, i.e., prevention of relapses of the disease. It consists of preventing intercurrent diseases, exposure to harmful factors (physical, mental fatigue, excessive insolation, etc.), clinical observation, and continuation of maintenance therapy with prednisone.

Bibliography:

Ageev A.K. Necrotizing granulomatosis of the upper respiratory tract (Wegener's granulomatosis), Arkh. pathol., t. 28, no. 2, p. 81, 1966; Gorlina A. A. and Sapronov V. G. Wegener's disease, Vestn, otorhinol., No. 2, p. 118, 1972; Wegener's granulomatosis, ed. A. I. Titova, Yaroslavl, 1964; Dranitsky Yu. S. and Denisyuk N. I. Wegener's granulomatosis, Arch. pathol., t. 30, no. 3, p. 75, 1968; Kurmaeva M. E. and Potekhina R. N. Wegener's granulomatosis, Sov. med., no. 10, p. 85, 1972; Fotin A.F., Yudov N.N. and Kogan R.P. About malignant nonspecific granulomas of the nose, Vestn, otorhinol., No. 6, p. 43, 1961; Yarygin N. E. and Gornak K. A. Periarteritis nodosa, Wegener's granulomatosis, combined forms of systemic vasculitis, M., 1970; Braun-Faleo O. u. Lukas I. Zur Klinik und Behandlung des Granuloma gangraenescens nasi, Hautarzt, S. 7, 1969; Burston HH Lethal midline granuloma, Laryngoscope (St Louis), v. 69, p. 1, 1959, bibliogr.; Godman G. S. a. Churg J. Wegener's granulomatosis, Arch. Path., v. 58, p. 533, 1954, bibliogr.; Klinger H. Grenzformen der Periarteriitis nodosa, Frankfurt. Z. Path., Bd 42, S. 455, 1931; Wegener, tiber generalisierte, septische Gefasserkrankungen, Verh. dtsch. path. Ges., Bd 29, S. 202, 1937; aka, tiber eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren, Beitr. path. Anat., Bd 102, S. 36, 1939; Woods R. Malignant granuloma of the nose, Brit. med. J., v. 2, p. 65, 1921; Yarington S. T., Abbott J. a. Raines D. Wegener's granulomatosis, Laryngoscope (St Louis), v. 75, p. 259, 1965.

M. E. Kurmaeva; A. V. Fotin (ENT)

Clinical picture

Clinical manifestations of Wegener's granulomatosis can be divided into the following stages:

• Damage to the upper respiratory tract, and in some cases the eyes and ears;
• Generalization of the disease with damage to internal organs. The lungs and kidneys are most often affected;
• The terminal stage in which pulmonary, renal and heart failure develops.

The duration of the first period is 1-2 years. Most patients with signs of early Wegener's granulomatosis have manifestations of acute sinusitis and acute rhinitis. At the initial stage of the disease, patients complain of nasal congestion, dryness and scanty mucous discharge, which soon becomes purulent, and then an admixture of blood appears. Some patients with granulations in the nasal cavity and destruction of the nasal septum experience nosebleeds. One of the characteristic symptoms of Wegener's granulomatosis is the formation of purulent-bloody crusts of a brown-brown color. They are removed in the form of casts, while the mucous membrane becomes thinner and acquires a bluish-red color, and in some places necrotization (death) of tissue is observed. With the development of the inflammatory process, the number of crusts increases, and they acquire an unpleasant, putrid odor.

In some cases, granulation tissue is observed in the nasal passages, which has a bright red color. Most often it is located on the turbinates, as well as in the upper cartilaginous parts of the nasal septum; somewhat less frequently, the posterior part of the nasal septum becomes its location. If you touch it, it bleeds, and because of this it is often mistaken for a tumor of the nasopharynx.

Wegener's granulomatosis is characterized by ulceration of the mucous membrane in the anterior parts of the nasal septum. At the beginning of the disease, the ulcer is on the surface, but gradually deepens and reaches the cartilage. With further progression of the disease, the ulcer necrotizes the cartilage and a perforation (hole) of the nasal septum is formed, at the edges of which granulation tissue is located. With further development of the process, necrotization of the bony part of the nasal septum occurs and the external nose, having lost its support frame, becomes saddle-shaped deformed.

With this disease, in the first 1-3 years other organs may not be involved in the process, but very often after 3-4 months intoxication appears and the process generalizes with the involvement of other organs and systems. Most often, one sinus is involved in the process and occurs against the background of ulcerative necrotic rhinitis. In case of exacerbation of the process, a deterioration in the general condition is observed. With further development of the disease, the outer (lateral) wall of the nose, which is the wall of the maxillary sinus, is gradually involved in the ulcerative-necrotic process. In case of its necrosis, a single communication is obtained between the nasal cavity and the sinus. The maxillary sinus is most often affected, and the frontal and ethmoid sinuses are somewhat less common. Quite often, despite the bright manifestations of sinusitis, no purulent discharge is detected during sinus puncture. Quite rarely, simultaneous destruction of the sphenoid sinus and nasal septum occurs.

In the later stages of the development of Wegener's granulomatosis, a necrotic mucous membrane is observed in the nasal cavity, covered with a large number of crusts, which are difficult to remove in the form of an impression. Near the bone walls of the nasal sinuses there are specific granulomas that affect the muperiosteum and disrupt the nutrition of the bone. Before the bone begins to break down, a process of demineralization occurs.

Friends! Timely and correct treatment will ensure you a speedy recovery!

With Wegener's granulomatosis, the most striking and logical primary manifestation of the disease is damage to the upper respiratory tract. A third of patients have ear lesions, but otitis media is only rarely the first sign of the disease. In some cases, complications arise such as paresis of the facial nerve, spread of the pathological process to the labyrinth, with the development of labyrinthitis.

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Systemic damage in Wegener's granulomatosis quite often manifests itself as a combination of rhinological and ophthalmological symptoms, manifested as keratitis (inflammation of the cornea of ​​the eye). If granulomatous infiltrates are located deep in the cornea, they can ulcerate, leading to the formation of deep ulcers that have undermined, raised edges. The sclera may also be involved in the pathological process. If the process affects the superficial layers of the sclera, then episcleritis occurs, and if the deep layers become inflamed, then scleritis develops. In some cases, a more severe disease develops - uveitis (inflammation of the choroid of the eyeball). With the development of keratoscleritis and keratosclerouveitis, swelling of the conjunctiva of the eye occurs, and patients complain of pain in the eye and blurred vision, the appearance of lacrimation and photophobia, and the occurrence of blepharospasm (persistent spasmodic closing of the eyelids). If such symptoms occur, you should consult an ophthalmologist.

The pathological process in the eye area is most often one-sided. In the later stages of Wegener's granulomatosis, exophthalmos (bulging eyes) or enophthalmos (recession of the eyeball) often develops. Exophthalmos often occurs if there is granulomatous tissue in the orbit and may recur. Enophthalmos appears as a late symptom of Wegener's granulomatosis. It occurs due to scar formation in the orbital tissues and optic nerve atrophy.

Ulcerative-necrotic changes in the larynx, pharynx and trachea are much less common.

In this case, the mucous membrane is hyperemic (pronounced reddened), and tubercles appear on the palatine arches, tonsils, soft palate and posterior wall of the pharynx, which quickly ulcerate. The eroded surface is covered with plaque, which is gray-yellow in color and difficult to remove, and the surface underneath bleeds. Patients complain of hoarseness, sore throat, stridorous (noisy, wheezing) breathing. Then the pain intensifies and profuse salivation (salivation) is observed. As the symptoms increase, weakness, headache, fatigue occur, and the temperature rises.

At stages 2 and 3 of the development of Wegener's granulomatosis, the most common clinical manifestation is lung damage. This causes a cough, which is sometimes accompanied by shortness of breath and hemoptysis. In the lungs you can find rounded infiltrates, which can be single or multiple. When they disintegrate, cavities with thin walls are formed, which are sometimes filled with liquid.

The classic sign of stages 2 and 3 of Wegener's granulomatosis is kidney damage. Characteristic pathologies are proteinuria (protein in the urine), microhemoturia (red blood cells in the urine), and progressive renal failure.

In Wegener's granulomatosis, joint damage and the development of ulcerative skin lesions are rare. Much more common are general symptoms such as weakness, fever, and weight loss.

The disease can occur in both acute and chronic forms. But the more acute the onset of the disease, the more severe its further course and the faster the generalization of the pathological process occurs.

Diagnostics

Diagnosis of the disease is very difficult. The diagnosis is made based on clinical symptoms. With malignant granuloma of the nose, some patients, before establishing a true diagnosis, are treated for other diseases - syphilis, malignant neoplasms, chronic paranasal sinusitis, etc.; with predominant damage to internal organs, they are treated for acute infiltrative-destructive pulmonary tuberculosis, abscess pneumonia, neoplasms in the lungs, systemic lupus erythematosus. All these diseases have some common features, as well as specific features.

Forecast

adverse. Sometimes, with early diagnosis of damage to internal organs and comprehensive treatment, one can hope for remission.

Types of granulomatosis

In addition to the pathological changes that characterize the described forms of the disease, the progression of Wegener's syndrome can be divided into four stages:

• granulomatous necotic vasculitis;
• pulmonary parenchyma;
• generalized inflammation of the entire respiratory and cardiovascular systems, as well as the kidneys and gastrointestinal tract;
• pulmonary-cardiac and renal failure.

The first stage passes rapidly, with the formation of purulent and ulcerative rhinosinusitis, laryngitis or otitis. Infections have a destructive effect on the cartilaginous tissue of the nasal septum and orbit. The pathological process continues with the lung parenchyma and moves to the next stage, in which inflammation of the lungs, blood vessels and kidneys progresses. The final terminal stage is characterized by the development of heart and kidney failure, which without proper treatment leads to the death of the patient.

The initial mechanisms and causes of granulomas are not fully known to scientists. The pathology can be traced in people who have previously suffered from various respiratory infections. It is based on the synthesis of protein antibodies that fight harmful microorganisms. Subsequently, they tend to settle on the walls of blood vessels, which leads to the release of chemical active substances.

There is chronic Wegener's granulomatosis, which is described by the inability of leukocytes to produce reactive oxygen species and the inability to phagocytose microorganisms. Manifests itself in different ways:

• in the form of recurrent infections;
• noticeable changes in the lungs, liver, kidneys, lymph nodes and other organs;
• abscesses;
• lymphadenitis;
• anemia.

In half of the cases, the occurrence of Wegener's chronic granulomatosis is due to heredity. This method of infection is achieved through an autosomal recessive type or a linked molecule to the X chromosome. This type of granulomatosis is characterized by defective white blood cells that have ceased to produce oxygen peroxide and superoxide. This leads to disruption of the phagocytosis of microorganisms by cells that are unable to completely destroy pathogens and fungi.

References

1. Adasheva, T.V., Nesterenko, O.I., Zadionchenko, V.S. and others. Clinical case of Churg-Strauss syndrome: diagnostic difficulties, therapeutic tactics. Archives of Internal Medicine, 2021. - No. 5. — P. 63-69.
2. Khodosh, E.M., Krutko, V.S., Efremova, O.A. Granulomatosis with polyangiitis (Wegener's) and systemic vasculitis: a multifaceted view of the problem (literature review). Current problems of medicine, 2021. - No. 19(268).
3. Clinical laboratory diagnostics: national guidelines. In 2 volumes / ed. V.V. Dolgova, V.V. Menshikov. - M.: GEOTAR-Media, 2012. - T.2 - P.106-108.

Forecast for the development of the disease

The transition of granulomatosis from the initial stage to the terminal stage occurs over a short period.

Moreover, in almost all cases, the infected organism is prone to complications. Among the large number of patients at a late stage there are:

• destruction of facial cartilage and bones;

• 
  deafness and blindness;

• hemoptysis;
• formation of granulomas;
• gangrene of the foot;
• additional infections;
• renal failure.

Lack of therapy in 97% of cases has an unfavorable prognosis: patients die within a period of six months to two years.

The course of the local form of the disease is benign. Immunosuppressive therapy helps improve the overall state of remission in 80% of patients. In half of the patients, a new exacerbation occurs after a long period.