Wegener's Granulomatosis - Symptoms.

Patients usually consult an otolaryngologist at the initial stage of Wegener's granulomatosis, when the rhino- and pharyngoscopic picture is assessed as a banal inflammatory process (atrophic, catarrhal rhinitis, pharyngitis), due to which precious time for the beginning of treatment is lost. It is important to promptly recognize the characteristic signs of damage to the nose and paranasal sinuses in Wegener's granulomatosis.

Nasal lesions in Wegener's granulomatosis

The first complaints of patients who consult an otolaryngologist usually boil down to nasal congestion (usually one-sided), dryness, scanty mucous discharge, which soon becomes purulent, and then bloody-purulent. Some patients complain of bleeding caused by the development of granulation in the nasal cavity or the destruction of the nasal septum. However, nosebleeds are not a pathognomonic symptom, since they occur relatively rarely. A more constant symptom of damage to the mucous membrane of the nasal cavity, which occurs in the earliest stages of Wegener's granulomatosis, is the formation of purulent-bloody crusts.

During anterior rhinoscopy, crusts of a characteristic brownish-brown color are revealed, which are removed in the form of casts. After removal of the picks, the mucous membrane is thinned, has a bluish-red color, in places (more often in the area of the lower and middle turbinates) necrotic. As the disease develops, especially if rational therapy is not carried out, the number of crusts increases, they become more massive, and a putrid odor appears. In terms of massiveness, the crusts resemble ozenous crusts, but differ from azenous crusts in color (in Wegener's granulomatosis, they are gray-brown with an admixture of blood, in ozena, they are green). In addition, the putrid odor emanating from them does not resemble the characteristic odor from the nasal cavity in patients with ozena. Doctors should also be alerted by unilateral damage to the nasal cavity.

Sometimes, the nasal passages contain a bumpy, bright red granulation tissue, most often located on the conchae and in the upper sections of the cartilaginous part of the nasal septum. Less often, granulation tissue is localized in the posterior sections of the nasal septum, covering the choana. When probing this area, even with a very light touch, bleeding is observed, which is why the process is often mistaken for a tumor.

One of the features of Wegener's granulomatosis is the presence of ulcerated mucous membrane in the area of the anterior nasal septum. In the initial stages of the disease, the ulcer is located superficially, but gradually deepens and can reach the cartilage. As the process progresses, cartilage necrosis develops and a perforation of the nasal septum is formed. Usually, there is also granulation tissue at the edges of the perforation. At first, the perforation occupies mainly the anterior sections of the septum (cartilaginous section), and as the process develops, it also affects the bone sections, due to which the external nose loses support and acquires a saddle-shaped form. In addition to a visual examination, an X-ray examination of the nasal cavity is necessary to detect changes in the nasal septum.

In some cases of chronic Wegener's granulomatosis, damage to the nose and paranasal sinuses may occur without general signs of intoxication (increased body temperature, weight loss, general weakness).

Other organs may not be involved in the process for 2-3 years. However, sipi, "harmless" course of ulcerative-necrotic rhinitis and sinusitis in Wegener's granulomatosis is extremely rare. More often, after 3-4 months, intoxication phenomena develop and the process generalizes with symptoms of damage to other organs. If atrophic phenomena of the mucous membrane are detected in the presence of poor health of the patient, subfebrile body temperature, protein in the urine, it is necessary to conduct a comprehensive examination of the patient to exclude Wegener's granulomatosis.

Along with inflammatory changes in the nasal cavity, pathology in the paranasal sinuses may also occur. Most often, one of the maxillary sinuses is affected, usually on the side of pronounced changes in the nasal cavity. Unilateral sinusitis usually occurs against the background of ulcerative-necrotic rhinitis and, when the process worsens, is accompanied by a deterioration in the general condition, a temperature reaction, and swelling of the cheek on the affected side. Over time, the ulcerative-necrotic process affects the mucous membrane of the nasal cavity, which is also the medial wall of the maxillary sinus. Gradually, the wall becomes necrotic, and a single cavity with the nasal cavity is created. Less often, simultaneous destruction of the nasal septum and the anterior wall of the sphenoid sinus is observed. In advanced stages of Wegener's granulomatosis, the nasal cavity and sinuses are lined with necrotic mucous membrane with a large number of dry crusts that are difficult to remove in the form of a massive cast.

Bone tissue changes in Wegener's granulomatosis are caused by the presence of a specific granuloma of soft tissues located directly at the bone walls of the paranasal sinuses. In this case, the mucoperiosteum is affected, as a result of which the nutrition of the bone is disrupted. Bone decay occurs due to perivasculitis in the bone tissue itself and in the peripherally located vessels. Bone walls are destroyed as a result of inflammation and osteoclastic process: the bone is replaced first by granulation tissue, and then by scar tissue; sometimes necrosis occurs in it. Demineralization precedes bone destruction.

Normalization of the bone pattern of the nasal cavity and paranasal sinuses after treatment for Wegener's granulomatosis is not observed, which is associated with a pronounced disruption of osteoblastic processes in bone tissue and reparative processes in the mucous membrane.

The systemic nature of the lesion in Wegener's granulomatosis sometimes manifests itself already in the early stages of the disease, when ophthalmological symptoms are detected along with rhinological symptoms. Apparently, this is explained by the common blood supply of the nose and eyes, due to which vasculitis can develop in them simultaneously. When the mucous membrane of the nose, paranasal sinuses and eyes are affected in combination, rhinological symptoms appear first in most cases.

Eye lesions in Wegener's granulomatosis

One of the most frequent and early symptoms of damage to the organ of vision in Wegener's granulomatosis is keratitis - inflammation of the cornea. In some cases, keratitis may be the result of toxic effects, but specific granulomatous lesions of the cornea are more common. With a deep location of granulomatous infiltrates in the cornea, they can ulcerate and lead to the development of a deep ulcer with undermined raised edges. Keratitis begins with infiltration of the corneal tissue from the marginal loop network and scleral vessels, infiltrates are formed along the edge of the membrane, and the developing ulcers are marginal. The objective picture (hyperemia of the tissues surrounding the infiltrate and ulcers) depends on which vessels vascularize the infiltrate (conjunctival or scleral). In severe forms of keratitis, pericorneal injection of vessels appears, which surrounds the entire cornea of the eye in a wide circle.

The sclera may also be involved in the process. Depending on the depth of the lesion, episcleritis (inflammation of the superficial layers of the sclera) or scleritis (inflammation of the deep layers) are distinguished. A severe process in the sclera may lead to uveitis (inflammation of the vascular membrane of the eyeball). With keratoscleritis and keratosclerouveitis, conjunctival edema of the eye is observed. Complaints of patients depend on the severity of the process; pain in the eyeball, deterioration of vision, photophobia and lacrimation, and the development of blepharospasm are possible. If these complaints are present, a patient with Wegener's granulomatosis should be carefully examined by an ophthalmologist.

The process in the eye area is often unilateral (on the side of the affected nose), much less often - bilateral. Sometimes the corneal ulcer reaches the posterior border plate (Descemet's membrane), and the eye is perforated, its anterior chamber is emptied.

In the late stages of Wegener's granulomatosis, patients may develop exo- or eophthalmos. Exophthalmos (forward displacement of the eyeball) may be recurrent. It can be assumed that exophthalmos develops due to the appearance of granulomatous tissue in the orbit, its manifestations increase with an exacerbation of the process and decrease with a decrease in the activity of the process under the influence of therapy. Enophthalmos is an even later symptom of Wegener's granulomatosis, with its development, the mobility of the eyeball is impaired up to complete ophthalmoplegia. Enophthalmos is obviously based on gross cicatricial changes in the tissues of the orbit. Ophthalmological manifestations of Wegener's granulomatosis in the late period may include dacryocystitis, which is a result of nonspecific changes, and the addition of a secondary infection. Treatment of ophthalmological and rhinological manifestations of Wegener's granulomatosis, as well as damage to other organs in this disease, is systemic.

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Pharyngeal and laryngeal lesions in Wegener's granulomatosis

Ulcer-necrotic changes in the pharynx in the larynx as a primary manifestation of Wegener's granulomatosis occur in about 10% of cases. Isolated lesion of the larynx is very rare, more often it is combined with lesions of the oropharynx and laryngopharynx. In these cases, a feeling of pain or discomfort in the throat (scratching, awkwardness when swallowing) occurs, later the pain in the throat intensifies, profuse salivation appears. The dominant symptom is spontaneous pain, which sharply intensifies when swallowing. In the initial stages of the disease, the general condition may not be disturbed, but as symptoms increase, signs of intoxication appear - malaise, weakness, fatigue. As a rule, patients associate them with malnutrition due to fear of sore throat when eating. However, in the absence of rational treatment, headache and subfebrile temperature soon appear. Often, the temperature is septic from the very beginning.

The process may be limited to the pharynx, but in some cases the mucous membrane of the oral cavity and larynx may be changed. The mucous membrane is hyperemic, small tubercles appear on the anterior arches of the tonsils, soft palate and posterior wall of the pharynx. The tubercles quickly ulcerate, and the ulcerated surface becomes covered with a grayish-yellow coating. The coating is removed with great difficulty, and a bleeding surface is found underneath. Gradually, necrosis of the mucous membrane intensifies, and the changes take on the character of a deep ulcer. At first, individual aphthae scar, forming stellate scars. Superficial aphthae form a delicate scar and do not lead to deformation of the underlying and surrounding tissue. As the process progresses, ulcerations quickly merge, forming an extensive ulcer occupying the entire posterior wall of the pharynx, the area of the tonsils, soft palate and capturing the area of the epiglottis. Depending on the localization of the process, scars tighten the soft palate, the larynx, and the epiglottis. With cicatricial deformation of the soft palate, open nasal speech and food reflux into the nasopharynx are observed. Scarring of the epiglottis limits its mobility, changes its shape, which contributes to choking due to food getting into the larynx. The earlier treatment is started, the less the function of the organ is disturbed.

When the pharynx and larynx are affected by Wegener's granulomatosis, despite extensive changes, the regional lymph nodes are either not enlarged or are slightly enlarged and painless.

There are no reports in the literature of combined lesions of the nasal cavity and pharynx, or the nasal cavity and larynx. In the pharynx, the process is usually combined with changes in the larynx. This is obviously explained by the common blood supply of the pharynx and the external parts of the larynx and the different blood supply of the pharynx and nasal cavity.

Ear lesions in Wegener's granulomatosis

Lesions of the middle and inner ear are not specific, but occur in one third of patients with Wegener's granulomatosis. These include: hearing loss due to impaired sound conduction and perception, adhesive otitis, sensorineural hearing loss. Acute purulent otitis that does not respond to conventional anti-inflammatory treatment requires special attention. At the height of the activity of the underlying disease, purulent otitis media is often complicated by facial nerve paresis. There are reports in the literature that granulation tissue removed from the ear is morphologically characterized as granulation tissue of nonspecific inflammation and necrotic vasculitis.

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The course of Wegener's granulomatosis

There are several variants of the course of Wegener's granulomatosis. Currently, the idea of Wegener's granulomatosis as a rare (casuistic) disease is becoming a thing of the past. More and more reports on various manifestations of this polysymptomatic disease appear in the literature. Thanks to such works, an idea of the clinical picture of the disease as a whole is formed. However, there are almost no publications on the general patterns of development of Wegener's granulomatosis. This gap in the study of this disease is apparently explained by the fact that studies of this kind require long-term observation of large groups of patients.

As a result of an in-depth clinical and laboratory study of patients with Wegener's granulomatosis, the clinical and pathological characteristics of various variants of the disease were clarified.

The severity of the development of the pathological process (suddenly or gradually) is determined by the body's response to damage and can determine both the onset and the further course of Wegener's granulomatosis. This classification of course variants is based on the characteristics of the onset and further course of the disease, its activity, the presence or absence of remissions, their duration, as well as the life expectancy of patients.

• In the acute phase, the pathological process is most active; the features of immunological homeostasis are significantly expressed, causing rapid progression and generalization of the process (development of lung, kidney, and skin lesions). At the same time, the general condition of patients is severe - high temperature (sometimes hectic), weight loss, general weakness, arthralgia. In the clinical blood test, a rapid increase in ESR to 40-80 mm / h, a decrease in the hemoglobin level, leukocytosis, lymphopenia, a shift in the blood formula to the right are noted, hypergammaglobulinemia appears. The C-reactive protein test is sharply positive. In the general urine analysis - pronounced hematuria, albuminuria, cylindruria. Despite active therapy, these patients fail to achieve stable remission of the disease, and they die in the first year and a half. The average life expectancy is about 8 months.
• In the subacute course of the disease, the onset of the process is not as rapid as in the acute course. Generalization is much slower. In the early stages, small spontaneous remissions are possible, and with adequate treatment (treatment-induced remissions) can last up to 1-2 years. In some cases, maintenance therapy corresponding to the activity of the process is necessary. At the onset of the disease, general symptoms (weakness, weight loss, anemia, temperature reaction) may occur, but they disappear or decrease under the influence of therapy. Hematological changes are less pronounced. An increase in ESR, leukocytosis are observed only in the initial period of the disease or during its exacerbation. The subacute course of Wegener's granulomatosis is difficult to diagnose, since the symptoms of the disease increase slowly. However, timely recognition and adequate therapy are extremely important for the prognosis of the disease as a whole. Life expectancy with this form varies from 2 to 5 years, depending on the timing of diagnosis and the beginning of treatment.
• In the chronic variant of the disease, the disease develops slowly and may remain monosymptomatic for several years. At the onset of the disease, spontaneous remissions are possible, which are easily achieved later with drug therapy. Generalization of the process with the appearance of general symptoms and changes in hematological parameters may develop 3-4 years after the onset of the disease. Exacerbation of the disease and earlier generalization may be facilitated by cooling, acute respiratory diseases, injuries and various secondary infections. The life expectancy of these patients is on average 7 years. It should be noted that the primary localization of the process (nose, pharynx) does not determine the further course of the disease.