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Diffuse alveolar hemorrhage in microscopic polyangiitis


Authors: M. Brůžková 1;  J. Vítová 1;  P. Cihlářová 2;  J. Novák 3
Authors‘ workplace: Interní oddělení Nemocnice České Budějovice, a. s. 1;  Anesteziologicko-resuscitační oddělení Nemocnice České Budějovice, a. s. 2;  Centrum klinické imunologie Nemocnice České Budějovice, a. s. 3
Published in: Čes. Revmatol., 32, 2024, No. 1, p. 33-37.
Category: Case Report

Overview

Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis characterized by the presence of anti-myeloperoxidase (anti-MPO) antibodies. It is a small vessel vasculitis with focally necrotizing glomerulonephritis without granuloma formation in the airways. Constitutional symptoms are present at the onset of the disease. These include arthralgia, arthritis, and myalgia.

Diffuse alveolar hemorrhage (DAH) is a clinical syndrome characterized by acute onset of alveolar infiltrates and hypoxemia leading to progressive diffuse alveolar hemorrhage requiring immediate treatment.

This case report describes a patient with newly diagnosed microscopic polyangiitis whose first manifestation was diffuse alveolar hemorrhage. The underlying disease was further complicated by severe hypoxic respiratory failure with the need for extracorporeal membrane oxygenation (V-V ECMO), difficult weaning (disconnection from artificial pulmonary ventilation), and recurrent infections including multidrug-resistant strains.

The patient was treated with high-dose pulses of glucocorticoids, reduced-dose cyclophosphamide, and immunoglobulin administration. After eight months of successful treatment, she was discharged home and her condition remains satisfactory.

Keywords:

diffuse alveolar hemorrhage – microscopic polyangiitis


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