Granulomatose mit Polyangiitis

Anja Kerstein; Konstanze Holl-Ulrich; Antje Müller; Gabriela Riemekasten; Peter Lamprecht

doi:10.1055/s-0042-111610

Granulomatose mit Polyangiitis

Translated title of the contribution: Granulomatosis with polyangiitis

Anja Kerstein, Konstanze Holl-Ulrich, Antje Müller, Gabriela Riemekasten, Peter Lamprecht^*

^*Corresponding author for this work

Clinic of Rheumatology

2 Citations (Scopus)

Abstract

Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.

Translated title of the contribution	Granulomatosis with polyangiitis
Original language	German
Journal	Deutsche Medizinische Wochenschrift
Volume	142
Issue number	1
Pages (from-to)	24-31
Number of pages	8
ISSN	0012-0472
DOIs	https://doi.org/10.1055/s-0042-111610
Publication status	Published - 05.01.2017

Research Areas and Centers

Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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This output contributes to the following UN Sustainable Development Goals (SDGs)

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title = "Granulomatose mit Polyangiitis",

abstract = "Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.",

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AU - Kerstein, Anja

AU - Holl-Ulrich, Konstanze

AU - Müller, Antje

AU - Riemekasten, Gabriela

AU - Lamprecht, Peter

PY - 2017/1/5

Y1 - 2017/1/5

N2 - Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.

AB - Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.

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DO - 10.1055/s-0042-111610

M3 - Zeitschriftenaufsätze

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AN - SCOPUS:85008873327

SN - 0012-0472

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JO - Deutsche Medizinische Wochenschrift

JF - Deutsche Medizinische Wochenschrift

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Granulomatose mit Polyangiitis

Abstract

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