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5/2014 vol. 52
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REVIEW PAPER
Clinical forms of granulomatosis with polyangiitis
Marta Madej
,
 
Agnieszka Matuszewska
,
 
Katarzyna Białowąs
,
 
Piotr Wiland
 
 
 
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Submission date: 2014-01-12
 
 
Final revision date: 2014-08-19
 
 
Acceptance date: 2014-09-25
 
 
Online publication date: 2014-11-17
 
 
Publication date: 2014-10-31
 
 
Reumatologia 2014;52(5):332-338
DOI: https://doi.org/10.5114/reum.2014.46672
 
 
Article (PDF)
References (36)
 
KEYWORDS
granulomatosis with polyangiitis
ANCA antibody
pulmonary-renal syndrome
 
TOPICS
Vasculitis - aetiology, pathogenesis, clinical features and treatment
 
ABSTRACT
Granulomatosis with polyangitis (GPA) is one of systemic connective tissue diseases. Necrotizing vasculitis affects small- and medium-sized vessels. Antineutrophil cytoplasmic antibodies directed against the neutrophil serine protease proteinase-3 (cANCA, PR3-ANCA) have been identified in approximately 80–95% of patients with GPA. In typical cases upper and lower respiratory tract and kidneys are involved. However GPA produce a broad spectrum of clinical symptomes, that includes: ocular and ear involvement, peripheral and central nervous system manifestations, heart and digestive tract involvement. The diffuse alveolar hemorrhage and pulmonary-renal syndrome may also occur. The diagnosis of granulomatosis with polyangiitis may cause many difficulties, specially in cases with limited organ involvement, with untypical spectrum of clinical features and in ANCA-negative patients. We attempt to present differential diagnosis of GPA in this review article.
 
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Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
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