Clinical Features and Outcomes of 37 Argentinean Patients With Severe Granulomatosis With Polyangiitis (Wegener Granulomatosis)

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a ’pure’ small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

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Rheumatoid Arthritis and Granulomatosis With Polyangiitis (Wegener’s Granulomatosis): A Rare Association

Bangladesh Journal of Medicine ◽

10.3329/bjm.v31i2.48542 ◽

2020 ◽

Vol 31 (2) ◽

pp. 114-119

Author(s):

Wafia Najifa ◽

Mohiuddin Sharif ◽

Rajib Roy ◽

Mahfuzul Haque ◽

Md Robed Amin

Keyword(s):

Rheumatoid Arthritis ◽

Clinical Practice ◽

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Wegener's Granulomatosis ◽

Overlap Syndrome ◽

Wegener Granulomatosis ◽

Overlap Syndromes ◽

Rare Association

The presentation of Rheumatoid arthritis (RA) combined with a second rheumatological disorder thatis, different RA overlap syndromes are frequently encountered in clinical practice. But RA-vasculitisoverlaps are relatively rare. This paper presents a case of Rheumatoid arthritis and Granulomatosiswith polyangiitis (Wegener granulomatosis) overlap syndrome which is first of its kind reported from Bangladesh. Bangladesh J Medicine July 2020; 31(2) : 114-119

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Granulomatosis with polyangiitis (Wegener granulomatosis): A proteinase-3 driven disease?

Joint Bone Spine ◽

10.1016/j.jbspin.2017.05.004 ◽

2018 ◽

Vol 85 (2) ◽

pp. 185-189 ◽

Cited By ~ 6

Author(s):

Véronique Witko-Sarsat ◽

Nathalie Thieblemont

Keyword(s):

Granulomatosis With Polyangiitis ◽

Proteinase 3 ◽

Wegener Granulomatosis

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Clinical features of ANCA-associated vasculitis

10.1093/med/9780199642489.003.0131 ◽

2013 ◽

Author(s):

Wolfgang L. Gross ◽

Julia U. Holle

Keyword(s):

Respiratory Tract ◽

Clinical Features ◽

Lower Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Systemic Disease ◽

Alveolar Haemorrhage ◽

Medium Size ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Necrotizing Glomerulonephritis ◽

Granulomatous Lesions

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a 'pure' small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

Download Full-text