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Akutes Nierenversagen bei pulmorenalem Syndrom

Acute renal injury in patients with pulmonary renal syndrome

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Der Nephrologe Aims and scope

Zusammenfassung

Das akute Nierenversagen (ANV) beim pulmorenalen Syndrom ist ein lebensbedrohliches Krankheitsbild, bedingt durch eine Glomerulonephritis bei diffuser alveolärer Hämorrhagie. Auslöser sind meist systemische Autoimmunerkrankungen, zu etwa 60% ANCA-assoziierte Vaskulitiden, zu 20% ein Goodpasture-Syndrom (Anti-Basalmembran-Erkrankung), seltener andere Vaskulitiden/Kollagenosen (z. B. SLE). Die Lungenbeteiligung kann subklinisch oder auch fulminant verlaufen. Sie lässt sich in der Thorax-CT darstellen und mittels bronchoalveolärer Lavage belegen. Die Nierenbiopsie zeigt eine extrakapillär proliferative Glomerulonephritis. Immunhistologisch finden sich je nach Entität nur lineare, granuläre oder spärliche Immunglobulinablagerungen. Die Therapie basiert auf einer Immunsuppression mit Cyclophosphamid und hochdosierten Kortikosteroiden, beim Goodpasture-Syndrom und den ANCA-assoziierten Vaskulitiden ergänzt durch die Plasmapherese. Während die renale Prognose bei Goodpasture-Syndrom mit Dialysepflichtigkeit ungünstig ist (die Therapie stabilisiert lediglich die pulmonale Situation), lässt sich bei den Vaskulitiden ein terminales Nierenversagen auch bei initialer Dialysepflichtigkeit häufig vermeiden.

Abstract

Acute renal injury in patients with pulmonary renal syndrome is life-threatening due to glomerulonephritis and diffuse alveolar hemorrhage. Usually, a systemic autoimmune disorder is the underlying disease – in 60% of cases, vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA); in 20%, Goodpasture’s syndrome (antiglomerular basement membrane disease); and rarely, other vasculitides or collagen vascular disorders such as systemic lupus erythematosus. Pulmonary involvement may range from subclinical to fulminant; it can be demonstrated by computed tomography and proven by bronchoalveolar lavage. Renal biopsy will show an extracapillary proliferative glomerulonephritis, and depending on the underlying disease, immunohistochemistry will show linear, granular, or only sparse immunoglobulin deposits. Therapy is based on immunosuppression with cyclophosphamide and corticosteroids, and in the case of Goodpasture’s syndrome and the ANCA-associated vasculitides is supplemented by plasma exchange. In dialysis-dependent patients with Goodpasture’s syndrome, the renal prognosis is poor, and treatment only stabilizes the pulmonary situation. In contrast, vasculitis patients may regain independent kidney function even if they had initially been dialysis dependent.

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  1. Nierenzentrum Weinheim, Röntgenstr 1, 69469, Weinheim, Deutschland

    W.H. Schmitt

  2. Zentrum für Nephrologie und Dialyse, Lindau, Deutschland

    R. Nowack

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  1. W.H. Schmitt
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  2. R. Nowack
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Correspondence to W.H. Schmitt.

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Schmitt, W., Nowack, R. Akutes Nierenversagen bei pulmorenalem Syndrom. Nephrologe 4, 107–117 (2009). https://doi.org/10.1007/s11560-008-0240-1

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