Symptoms of Wegener's granulomatosis
It often develops in adults, but it can also occur in children. There are generalized, borderline and localized (malignant granuloma of the face) forms. Some authors consider the localized form an independent disease. In generalized form, in addition to necrotic lesions of the nasal mucous membranes, sinuses, upper airways, leading to extensive destruction, changes in internal organs (lungs, kidneys, intestines) due to lesions of small arteries and veins are observed. In the lungs there are calcified foci, in the kidneys - focal or diffuse glomerulonephritis. With the borderline form, severe pulmonary and extrapulmonary (including skin) changes occur, but without marked renal involvement. With a localized form, skin changes predominate with a pronounced destruction of the facial tissues.
The main clinical symptoms of Wegener's granulomatosis include ulcerative necrotic changes in the upper respiratory tract (ulcerative necrotic rhinitis, laryngitis, sinusitis), trachea and bronchi, lung and kidney damage.
For a long time it was believed that with Wegener's granulomatosis pathological changes from the heart are rare enough and they do not affect the prognosis. This is due to the low or asymptomatic course of heart disease. Thus, Wegener's granulomatosis is characterized by the asymptomatic course of the coronary artery; described cases of the development of painless myocardial infarction. However, it is shown that the lesion of the coronary arteries occurs in 50% of patients (submitted series of autopsies). A more typical result of coronary artery disease may be dilated cardiomyopathy (DCM). Occasionally, granulomatous myocarditis, valvular defects and pericarditis are noted, which can be explained by the involvement of small artery valves and pericardium. AH is noted in patients with a generalized variant of the disease when involved in the pathological process of the kidneys.
The skin is involved in the process again approximately in 50% of patients. There are extensive ulcerative necrotic lesions of the central part of the face as a consequence of the spread of the process from the nasal cavity; ulcerous lesions of the oral mucosa; mainly in the late stages of the process may be polymorphic eruptions: petechiae, ecchymosis, erythematoma-papular, nodular necrotic elements, ulcerative necrotic lesions such as gangrenous pyoderma on the trunk and distal limbs. The outlook is unfavorable. The development against the background of immunosuppressive therapy of the disease of generalized tumor in the form of malignant histiocytosis is described.
A similar skin lesion, but usually without a hemorrhagic component, can be observed with a so-called granulomatous lymphoma that differs from Wegener's granulomatosis with a predominant lung injury without changes in the upper respiratory tract and, possibly, the kidneys, an increased risk of developing lymphomas. Presence of atypical lymphocytes in polymorphic infiltrates.