open access

Vol 82, No 1 (2014)
EDUKACJA. Zmniejszmy ciężar przewlekłej obturacyjnej choroby płuc (cz. II)
Submitted: 2013-12-20
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Granulomatosis with polyangiitis — clinical picture and review of current treatment options

Marta Lembicz, Halina Batura- Gabryel, Agata Nowicka
DOI: 10.5603/PiAP.2014.0010
·
Pneumonol Alergol Pol 2014;82(1):61-73.

open access

Vol 82, No 1 (2014)
EDUKACJA. Zmniejszmy ciężar przewlekłej obturacyjnej choroby płuc (cz. II)
Submitted: 2013-12-20

Abstract

Granulomatosis with polyangiitis (GPA) is a rare disease of unknown aetiology, characterized by necrotizing inflammation of small and medium-sized vessels and formation of granulomas. The disease typically involves the upper and lower respiratory tracts and the kidneys, but it can occur in many other locations, often giving a surprising clinical picture. The majority of patients have autoantibodies against cytoplasm of neutrophils (ANCA), of which a pathogenetic role is postulated. The disease affects mostly middle-aged and elderly people, with a predominance of Caucasians. Before the era of immunosuppressive therapy GPA was associated with high early mortality. Nowadays, we have a treatment that gives remission in the majority of patients. The decision about the method of treatment should be made depending on the clinical form of the disease (local or generalized, refractory). Standard remission induction therapy in the generalized disease is cyclophosphamide in combination with steroids. This therapy, however effective, is associated with a number of side effects. In addition, some patients are resistant to standard therapy, and half of them experience relapses. Therefore, there is need for further research for alternative treatment and maintenance therapy. Currently, hopes are raised by biological agents. Among them, the best studied so far is rituximab, which found a place in the current recommendations of EULAR as an alternative drug for patients with refractory disease. The article discusses the clinical picture of GPA, diagnosis and currently recommended treatment.

Abstract

Granulomatosis with polyangiitis (GPA) is a rare disease of unknown aetiology, characterized by necrotizing inflammation of small and medium-sized vessels and formation of granulomas. The disease typically involves the upper and lower respiratory tracts and the kidneys, but it can occur in many other locations, often giving a surprising clinical picture. The majority of patients have autoantibodies against cytoplasm of neutrophils (ANCA), of which a pathogenetic role is postulated. The disease affects mostly middle-aged and elderly people, with a predominance of Caucasians. Before the era of immunosuppressive therapy GPA was associated with high early mortality. Nowadays, we have a treatment that gives remission in the majority of patients. The decision about the method of treatment should be made depending on the clinical form of the disease (local or generalized, refractory). Standard remission induction therapy in the generalized disease is cyclophosphamide in combination with steroids. This therapy, however effective, is associated with a number of side effects. In addition, some patients are resistant to standard therapy, and half of them experience relapses. Therefore, there is need for further research for alternative treatment and maintenance therapy. Currently, hopes are raised by biological agents. Among them, the best studied so far is rituximab, which found a place in the current recommendations of EULAR as an alternative drug for patients with refractory disease. The article discusses the clinical picture of GPA, diagnosis and currently recommended treatment.

Get Citation

Keywords

granulomatosis with polyangiitis, ANCA, cyclophosphamide, rituximab, Wegener granulomatosis

About this article
Title

Granulomatosis with polyangiitis — clinical picture and review of current treatment options

Journal

Advances in Respiratory Medicine

Issue

Vol 82, No 1 (2014)

Pages

61-73

DOI

10.5603/PiAP.2014.0010

Bibliographic record

Pneumonol Alergol Pol 2014;82(1):61-73.

Keywords

granulomatosis with polyangiitis
ANCA
cyclophosphamide
rituximab
Wegener granulomatosis

Authors

Marta Lembicz
Halina Batura- Gabryel
Agata Nowicka

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