Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3

Relle, Manfred; Föhr, Bernd; Fasola, Federica; Schwarting, Andreas

doi:10.1016/j.mcp.2016.08.009

Cited by 26 publications

(22 citation statements)

References 96 publications

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“…32 No other autoimmune diseases were assessed. The extrahepatic autoimmune diseases were categorised as those believed to share HLA associations (ie HLA DR3, DR4 or DR7) with AIH, 4,[33][34][35][36][37][38] and those with other HLA associations 4,[39][40][41][42][43][44][45] or unknown associations [46][47][48][49][50] (Table 1).…”

Section: Extrahepatic Autoimmune Diseasesmentioning

confidence: 99%

Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Grønbæk

Vilstrup

Pedersen

et al. 2018

Liver International

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Section: Extrahepatic Autoimmune Diseasesmentioning

confidence: 99%

Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Grønbæk

Vilstrup

Pedersen

et al. 2018

Liver International

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“…The typical clinical manifestations of GPA are the consequence of the presence of granulomas in the upper and lower respiratory tract (ear, nose, and throat lesions), consisting in sinusitis, chronic otitis media crusting rhinitis, saddle‐nose deformity, or nasal septum perforation. Pulmonary involvement is quite frequently observed with parenchymal nodules and/or alveolar hemorrhage, and represents the most dangerous presentation of the disease together with the rapidly progressive glomerulonephritis .…”

mentioning

confidence: 99%

Th17 and Treg lymphocytes as cellular biomarkers of disease activity in Granulomatosis with Polyangiitis

Cosmi

2017

Eur J Immunol

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“…It has been proposed that infections, genetic and environmental factors may contribute to development of the disease. Genome‐wide association studies (GWAS), candidate gene analysis, familial association studies have provided insight into the genetic etiology of GPA . However, very sparse data is available from Asian countries regarding the various etiological factors in GPA.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Is granulomatosis with polyangiitis in Asia different from the West?

et al. 2018

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The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries.The annual incidence of GPA among Asians varies between 0.37/million to 2.1/million population. The prevalence of GPA has been estimated to be 1.94/100 000 in a Chinese population. Polymorphisms in class II major histocompatibility genes and ETS1 proto-oncogene has been shown in Asian patients with GPA. There is a difference in mean age at onset and proteinase 3 (PR3) or myeloperoxidase (MPO) positivity in GPA patients from different Asian countries. Those from India had mean age of 40 years and those from Japan had mean age of 65 years. Sixty percent of GPA patients from China and Japan were MPO ANCA positive while the majority of patients from India and Korea were PR3 positive. Geographical variation with lower frequency of renal involvement in Indian studies and higher frequency in Chinese patients has also been noted. Treatment outcomes have been similar to those reported from other parts of the world. Remission was achieved in about two-thirds of patients while relapses were noted in one-third to half of the patients. Apart from minor differences in the organ systems involved, MPO-ANCA GPA and PR3-ANCA GPA had similar rates of remission and relapses. K E Y W O R D S Asia, granulomatosis with polyangiitis, vasculitisWe searched PubMed using the search terms "Granulomatosis with polyangiitis", "GPA" and "Wegener's granulomatosis". We screened for articles published from Asian countries and describing the epidemiology, pathogenesis, clinical features and outcomes of GPA patients. Case reports and small case series of less than 5 patients were not included. The basis of diagnosis of GPA in different studies was either histopathology suggestive of GPA or fulfilling the American College of Rheumatology (ACR) criteria for GPA or classified according to European Medicines Evaluation Agency classification. We also included large case series on GPA from Europe and USA to compare them with Asian studies. | EPID EMIOLOGYThe incidence and prevalence of GPA shows a regional and ethnical difference with higher incidence of GPA in northern Europe and lower incidence in Asian countries. 1 The precise estimates of incidence and prevalence of AAV and GPA are not available from Asian countries. In the epidemiological study by Fujimoto et al, 1 the annual incidence of GPA in Japan was calculated to be 2.1/million population, which was much lower than that noted in the UK (annual incidence of 14.3/million). The annual incidence of MPA was higher in Japan than the UK and the overall incidence of all AAV was similar between Japan and the UK. Liu et al 7 used DIS-MOD II software to estimate prevalence based on the inpatient data of GPA patients in 100 hospitals across China. They estimated the prevalence of GPA to be 1...

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Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3

Cited by 26 publications

References 96 publications

Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Th17 and Treg lymphocytes as cellular biomarkers of disease activity in Granulomatosis with Polyangiitis

Is granulomatosis with polyangiitis in Asia different from the West?

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