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AB0471 Granulomatosis with polyangiitis (gpa, wegener’s): complications and causes of death
  1. O. Zimba1
  1. 1Internal Medicine No2, Danylo Halytsky Lviv National Medical University, Lviv, Ukraine

Abstract

Background GPA is a necrotizing vasculitis with predominant involvement of the upper airways, pulmonary parenchyma and kidneys. In the absence of treatment prognosis for life is unfavorable.

Objectives To determine the complications and causes of death in patients with GPA.

Methods Specific causes of death of GPA were revealed based on clinical and pathomorphological data assessment of 11 fatal cases (8 men and 3 women, aged 16-74).

Results Combined pulmonary and renal failure holds leading position among causes of death in patients with GPA (observed in six cases (54,5%). Destructive and productive panvasculitis, affecting small and medium sized vessels (capillaries, venules, arterioles, and arteries), were revealed in bronchial and pulmonary vessels. In the affected vessels thrombosis was found, causing hemorrhagic infarctions of lungs with frequent abscess formation. Histologically, the most common lesions of lungs were extravascular polymorphic necrotizing granular tissue in the form of pulmonary nodules with frequent cavitation. Disturbed circulation in lungs creates favorable conditions for the emergence of infectious complications - pneumonia, lung abscess, pleural empyema were observed in half of the cases. These changes significantly worsen thecourse of disease, increasing the risk of death. Analysis of pathological changes in kidneys showed that the most common lesions were diffuse necrotizing glomerulonephritis (63,7%), ischemic infarction (18,2%) and abscess (18,2%). Common cause of death from GPA was profuse hemorrhage from the eroded vessels of the upper respiratory tract, lungs and intestine. Our data showed that in four cases (36,4%) the disease was complicated by lethal bleeding. In two cases, the source of hemorrhage was duodenal ulcer. In other patients the cause of death was bleeding from eroded vessels of lung and trachea. Death from pulmonary hemorrhage occurred due to upper airway obstruction induced by asphyxia. The central nervous system pathology plays an important role in thanatogenesis of GPA. In 6 cases (54,5%) edema of brain tissue was observed, wherever in one case, the bulbar syndromewas dominating, characterized by dysarthriaanddysphagia due tothe weakness of soft palate, tongue and pharynx. The autopsy results showed edema of the brain, foci of demyelination and necrosis with cyst formation in the medulla oblongata.

Conclusions The complications and causes of death in patients with GPA are: combined pulmonary and renal failure; profuse hemorrhage from the eroded vessels of the upper respiratory tract, lungs, intestine; infectious complications; central nervous system disorders.

Disclosure of Interest None Declared

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