Wegener's granulomatosis: symptoms

To the otorhinolaryngologist, patients usually turn to the initial stage of Wegener's granulomatosis disease, when the rhino and pharyngoscopic picture is regarded as a banal inflammatory process (atrophic, catarrhal rhinitis, pharyngitis), which is why precious time is wasted to start treatment. It is important to recognize in a timely manner the characteristic signs of nasal and paranasal sinus lesions with Wegener's granulomatosis.

The defeat of the nose with Wegener's granulomatosis

The first complaints of patients who turn to the otorhinolaryngologist are usually reduced to nasal congestion (usually one-sided), dryness, poor mucous secretions, which soon become purulent, and then blood-purulent. Some patients complain of bleeding due to the development of granulations in the nasal cavity or destruction of the septum of the nose. However, nosebleeds are not a pathognomonic symptom, since they are relatively rare. A more permanent symptom of the defeat of the nasal mucosa, which occurs in the earliest stages of Wegener's granulomatosis, is the formation of purulent-bloody crusts.

With anterior rhinoscopy, crusts of characteristic brown-brown color are revealed, which are removed in the form of casts. After removal of the picks, the mucosa is thinned, has a cyanotic red color, in some places (more often in the area of the lower and middle shell) is 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 smell appears. Massiveness of the crust resembles ozonous, but they differ from azogenous in color (with Wegener's granulomatosis they are gray-brown with an admixture of blood, while green is green). In addition, the putrid smell emanating from them does not resemble the characteristic odor from the nasal cavity in patients with ozona. Doctors should be alerted to unilateral damage to the nasal cavity.

Sometimes in the nasal passages there is a bumpy, bright red granulation tissue, most often it is located on the shells and in the upper parts of the cartilaginous part of the nasal septum. Less often the granulation tissue is localized in the posterior parts of the nasal septum, closing the choana. During the probing of 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 an ulcerated mucous membrane in the region of the anterior part of the septum of the nose. In the initial stages of the disease, the ulcer is superficial, but gradually deepens and can reach the cartilage. With the progress of the process, necrosis of cartilage develops and a perforation of the septum of the nose is formed. Typically, at the edges of the perforation there is also a granulation tissue. At first, the perforation occupies mainly the anterior part of the septum (the cartilaginous part), and as the process develops, it also seizes the osseous parts, so that the outer nose loses the support and acquires a saddle shape. In addition to visual inspection, an x-ray examination of the nasal cavity is necessary to detect changes in the nasal septum.

In some cases of chronic course of Wegener's granulomatosis, the defeat of the nose and paranasal sinuses can occur without common intoxication phenomena (fever, weight loss, general weakness).

Other bodies may not be involved in the process for 2-3 years. However, sipi, the "harmless" course of ulcerative necrotic rhinitis and sinusitis with Wegener's granulomatosis is extremely rare. More often after 3-4 months the phenomena of intoxication develop and generalization of the process begins with the symptoms of defeat of other organs. At detection of atrophic phenomena of the mucous membrane 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 while excluding 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 maxillary sinusitis usually occurs against the background of ulcerative necrotic rhinitis and when the process worsens, the general condition worsens, the temperature reaction, and the cheeks swell on the side of the lesion. Over time, the ulcerative-necrotic process captures 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 a nasal cavity is created. Less often observed simultaneous destruction of the septum of the nose and anterior wall of the sphenoid sinus. In the far-advanced stages of Wegener's granulomatosis, the nasal cavity and sinus are lined with necrotized mucosa with a large number of dry crusts, which are difficult to remove in the form of a massive impression.

Changes in bone tissue with Wegener's granulomatosis are due to the presence of a specific granuloma of the soft tissues located directly at the bone walls of the paranasal sinuses. At the same time, the muckeroper is affected, as a result of which the nutrition of the bone is disturbed. Decay of the bone occurs due to perivascular in the bone tissue itself, and in peripherally-located vessels. Bone walls are destroyed as a result of inflammation and osteoclastic process: the bone is replaced first by granulation and then by scar tissue; sometimes necrosis occurs in it. The destruction of the bone is preceded by its demineralization.

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

Systemic lesions in Wegener's granulomatosis are sometimes manifested already in the early stages of the disease, when ophthalmic symptoms are found along with rhinological symptoms. Apparently, this is due to the generality of the blood supply to the nose and eyes, in connection with which they can simultaneously develop vasculitis. When combined defeat of the nasal mucosa, paranasal sinuses and eyes, the first in most cases appear rinological symptoms.

Eye damage in Wegener's granulomatosis

One of the most frequent and early-onset symptoms of damage to the eye in Wegener's granulomatosis is keratitis, an inflammation of the cornea of the eye. In some cases, keratitis may be the result of toxic effects, but more specific granulomatous lesions of the cornea are observed. With a deep arrangement of granulomatous infiltrates in the area of the cornea, they can ulcerate and lead to the development of deep ulcers with undercut elevated margins. Keratitis begins with infiltration of the tissue of the cornea from the marginal loop network and scleral vessels, infiltrates are formed along the edge of the shell, the developing ulcers are marginal. The objective picture (hyperemia of the tissues surrounding the infiltrate and ulcers) depends on which vessels the vascularization of the infiltrate (conjunctival or scleral) occurs. In severe forms of keratitis, a pericorneal injection of vessels appears, which surrounds the entire cornea of the eye with a wide aureole.

A sclera can be involved in the process. Depending on the depth of the lesions, epicleritis (inflammation of the surface layers of the sclera) or scleritis (inflammation of the deep layers) is distinguished. A severe process in the sclera can lead to uveitis (inflammation of the choroid of the eyeball). With keratoscleritis and keratosclerovitis, edema of the conjunctiva of the eye is observed. Complaints of patients depend on the severity of the process, there may be pain in the eyeball area, visual impairment, the appearance of photophobia and lacrimation, the development of blepharospasm. In the presence of these complaints, the patient with granulomatosis of Wegener should be carefully examined by an ophthalmologist.

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

In the late stages of Wegener's granulomatosis, exo- or eiophthalmos may develop in patients. Exophthalmos (shifting the eyeball forward) may have a recurring character. It can be assumed that exophthalmus develops in connection with the appearance of granulomatous tissue in the orbit, its manifestations intensify with exacerbation of the process and decrease with a decrease in the activity of the process under the influence of therapy. Enofthalm is an even later symptom of Wegener's granulomatosis, with its development the mobility of the eyeball is disturbed right up to full ophthalmoplegia. At the heart of enophthalmos, obviously, there are gross cicatricial changes in the tissues of the orbit. To ophthalmological manifestations of Wegener's granulomatosis in the late period can be attributed dacryocystitis, which is the result of nonspecific changes, and the attachment of a secondary infection. Treatment of ophthalmological, rhinological manifestations of Wegener's granulomatosis, as well as the defeat of other organs in this disease is systemic.

[1], [2], [3]

Lesion of the pharynx and larynx with Wegener's granulomatosis

Ulcerative necrotic changes in the pharynx in the larynx as the primary manifestation of Wegener's granulomatosis occur in about 10% of the observation. Isolated lesion of the larynx is very rare, more often it is combined with damage to the mouth and throat. In these cases, there is a feeling of pain or discomfort in the throat (perspiration, awkwardness when swallowing), later the pain in the throat increases, there is abundant salivation. The dominant symptom are pains of a spontaneous nature, sharply increasing when swallowed. In the initial stages of the disease, the general condition may not be disturbed, but with the increase in symptoms there are signs of intoxication malaise weakness, weakness. As a rule, patients associate them with malnutrition due to fear of pain in the throat when receiving a baby. However, in the absence of rational treatment, headache and subfebrile temperature soon appear. Often the temperature from the outset is septic in nature.

The process can be limited only to the pharynx, but in a number of cases the mucous membrane of the oral cavity and larynx can be changed. Mucous membrane is hyperemic, on the front arches of the tonsils, soft palate and back wall of the pharynx appear small tubercles. The tubercles rapidly ulcerate, and the surface is covered with a greyish-yellow coating. Removed plaque with great difficulty, under it is found bleeding surface. Gradually, the necrosis of the mucosa is amplified, and the change is characterized by a deep ulcer. At first, separate aphytes are scarred, forming zebra scars. Surface aphyons form a gentle scar and do not lead to deformation of the underlying and surrounding tissue. When the process of ulceration progresses, they quickly merge, forming an extensive ulcer that occupies the entire posterior wall of the pharynx, the area of the tonsils, the soft palate, and seize the region of the epiglottis. Depending on the location of the process, scars are contracted by the soft palate, larynx region, epiglottis. When cicatricial deformity of the soft palate is observed open nasal and throwing food into the nasopharynx. Scarring of the epiglottis limits its mobility, changes its shape, which promotes tearing due to ingestion of food into the larynx. The earlier the treatment is started, the less the function of the organ is affected.

With the defeat of the pharynx and larynx with Wegener's granulomatosis, despite extensive changes, regional lymph nodes are either not enlarged or enlarged insignificantly and painlessly.

In the literature there are no reports of a combined lesion of the nasal cavity and pharynx, or nasal and laryngeal cavities. In the throat, the process, as a rule, is combined with changes in the larynx. This is obviously explained by the generality of the blood supply to the pharynx and the outer parts of the larynx and various blood supply to the pharynx and the nasal cavity.

Ear damage with Wegener's granulomatosis

The lesions of the middle and inner ear are not specific, but occur in a third of patients with Wegener's granulomatosis. These include: hearing loss due to disturbance of sound and sound perception, adhesive otitis, sensorineural hearing loss. Special attention is required for acute purulent otitis, which is not amenable to the usual anti-inflammatory treatment. At the height of the activity of the underlying disease, purulent otitis media is often complicated by paresis of the facial nerve. There are reports in the literature that granulations removed from the ear are morphologically characterized as granulation tissue of nonspecific inflammation and necrotic vasculitis.

[4], [5], [6], [7], [8], [9]

The course of Wegener's granulomatosis

There are several variants of the course of Wegener's granulomatosis. Now we are losing sight of the Wegener's granulomatosis as a rare (casuistic) disease. In the literature there are more and more reports about the various manifestations of this polysymptomatic disease. Thanks to such works, there is an idea of the clinical picture of the disease as a whole. 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 probably due to the fact that studies of this kind require a long-term observation of large groups of patients.

As a result of the advanced clinical and laboratory study of patients with Wegener's granulomatosis, the clinical and pathological characteristics of various variants of the course of the disease have been refined.

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

• In acute course, the pathological process is most active; the features of the immunological homeostasis, which determine the rapid progression and generalization of the process (development of lung, kidney, and skin lesions) are markedly expressed. In this case, the general condition of patients is severe - high temperature (sometimes hectic), weight loss, general weakness, arthralgia. In the clinical analysis of blood there is a rapid increase in ESR to 40-80 mm / h, a decrease in hemoglobin, leukocytosis, lymphopenia, a shift of the blood formula to the right, hypergammaglobulinemia appears. The sample for C-reactive protein is sharply positive. In the general analysis of urine - pronounced hematuria, albuminuria, cylindruria. Despite active therapy, these patients can not achieve a 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 turbulent as when acute. Generalization is much slower. In the early stages, small spontaneous remissions are possible, and with adequate treatment (remission-induced remissions) can reach 1-2 years. In some cases, supportive therapy is necessary, corresponding to the activity of the process. 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. Hematologic changes are less pronounced. Increased ESR, leukocytosis are noted only in the initial period of the disease or in its aggravation. A subacute variant of the course of Wegener's granulomatosis is complicated for diagnosis, since the symptoms of the disease grow slowly. However, timely recognition and adequate therapy are extremely important for the prediction 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 start of treatment.
• With a chronic variant of the disease, the disease develops slowly, it can remain monosymptomatic for a number of years. At the onset of the disease, spontaneous remissions are possible, which are easily achieved in the future in drug therapy. The generalization of the process with the appearance of general symptoms and the change in hematological parameters can develop 3-4 years after the onset of the disease. Aggravation of the disease and earlier generalization can contribute to cooling, acute respiratory infections, injuries and various secondary infections. The average life expectancy of these patients is 7 years. It should be noted that the primary localization of the process (nose, pharynx) does not determine the further course of the disease.