Successful Treatment of a Rare Complication of HenochSchoenlein Purpura in Advanced Age: Pulmonary Hemorrhage

Abstract

Henoch-Shönlein purpura (HSP) is a form of systemic vasculitis involving

An adult patient with Kabuki syndrome presenting with Henoch-Schönlein purpura complicated with pulmonary hemorrhage

“…It is an immunemediated vasculitis associated with IgA deposition [1,2]. Although the underlying causes of HSP remain unclear, a recent history of upper respiratory infection and the [5][6][7][8][9][10]. Of 29 HSP patients with pulmonary hemorrhage, 48% were over 18 years old [5][6][7][8][9][10].…”

“…Although the underlying causes of HSP remain unclear, a recent history of upper respiratory infection and the [5][6][7][8][9][10]. Of 29 HSP patients with pulmonary hemorrhage, 48% were over 18 years old [5][6][7][8][9][10]. The mortality of HSP with pulmonary hemorrhage was 50% in adults and 20% in pediatric patients [5][6][7][8][9][10].…”

“…Of 29 HSP patients with pulmonary hemorrhage, 48% were over 18 years old [5][6][7][8][9][10]. The mortality of HSP with pulmonary hemorrhage was 50% in adults and 20% in pediatric patients [5][6][7][8][9][10]. The principal cause of pulmonary hemorrhage in HSP is vasculitis in the small pulmonary blood vessels.…”

Successful Treatment of Diffuse Alveolar Hemorrhage with Recombinant Factor VIIa in a Child with Henoch-Schönlein Purpura

“…Pulmonary hemorrhage with fatal respiratory failure has seldom been reported. [2][3][4][5][6] In life-threatening bleeding situations, even without pre-existing coagulopathies, recombinant factor VIIa (rFVIIa) seems to be a possible choice for treatment. The primary mode of action used by rFVIIa to stop the bleeding focuses on the tissue factor (TF)-dependent coagulation activity, which also affects the activated platelet surface.…”

“…When we reviewed the English literature, we came across only a few case reports concerning DAH in HSP. [2][3][4][5][6] Therapy with steroids alone is usually not enough to control this kind of severe bleeding; however, a treatment which combines pulse steroids and cyclophosphamide seems to be effective. Despite these kinds of efficient drugs, some extra time is required to observe their effects.…”