Wegener's granulomatosis - necrotizing granulomatous respiratory tract, the orbit or the lungs, often combined with systemic vasculitis and glomerulonephritis.
The etiology of Wegener's granulomatosis is not known. Among the possible causes of Wegener's granulomatosis Gr more likely to find a microbial or viral factor. The disease often occurs after a viral respiratory infections, hypothermia, vaccination, antibiotic therapy, but can occur in healthy children.
In the pathogenesis of Wegener's granulomatosis is very important violation of the humoral and cellular immunity, the formation and fixation of the affected organs of immune complexes and activation of blood clotting.
Children produce 2 forms of Wegener's granulomatosis: localized generalized.
In the localized form developed necrotizing lesion of upper respiratory tract, granulomatous ocular orbit or a combination of both processes.
In generalized form, along with granulomatosis of respiratory tract or the eyes, watching pulmonary, cardiac and cutaneous syndromes, all patients with glomerulonephritis.
Wegener's granulomatosis begins acutely or gradually: there is a low-grade fever of the body, worsening appetite after a few days the body temperature reaches 38-39C, 10 days - 3 months joined the characteristic inflammatory and ulcerative necrotic changes in the facial skeleton or the lungs.
Depending on Wegener's granulomatosis first complaints are complaints of headache, pain in the ears and mouth, epistaxis, hemoptysis, edema of the eyelids, rhinitis. If it affects the upper respiratory tract appear persistent runny nose with sanious discharge, sinusitis pattern, necrotising mucosa of nasal passages, soft palate, tonsils. Beyond that there is destruction of the walls, sinks with perforation of the soft and hard palate.
In a generalized form of Wegener's granulomatosis in a few weeks or months on the background of progressive necrotizing process in the primary focus of cutaneous symptoms appear - polymorphic, nodular, hemorrhagic and ulcerous-necrotic elements, in some patients, myocarditis, pericarditis, bronchitis.
However, the most formidable prognostic is joining in this stage of glomerulonephritis. It occurs without arterial hypertension, with the rapid development of renal failure.
During Wegener's granulomatosis is an acute and subacute. In the acute course of the clinical picture is clearly expressed, the generalization of the process occurs within a few weeks or months, is rapidly developing renal failure. Subacute characterized by slow development of clinical symptoms.
Treatment of Wegener's granulomatosis spend cytostatics. Until recently, the prognosis was poor, life expectancy of patients was 6-12 months, the forecast has improved by treatment with cytostatics.