Clinical features of ANCA-associated vasculitis

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.

Clinical features of ANCA-associated vasculitis

2013 ◽  
pp. 1090-1102
Author(s):  
Wolfgang L. Gross ◽  
Julia U. Holle
Keyword(s):  
Respiratory Tract ◽  
Clinical Features ◽  
Lower Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Clinical Signs ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Granulomatous Lesions ◽  
Organ Manifestations

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.

scholarly journals An extraordinary pulmonary artery involvement in granulomatosis with polyangiitis

2021 ◽  
Vol 8 (10) ◽  
pp. 1587
Author(s):  
Ahmad A. Alkhdairi ◽  
Ayman A. Alharbi
Keyword(s):  
Pulmonary Artery ◽  
Immunosuppressive Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Disease ◽  
Pulmonary Arteries ◽  
Unusual Presentation ◽  
Granulomatous Lesions ◽  
Large Vessels ◽  
Immune Vasculitis

Granulomatosis with polyangiitis (GPA) is an autoimmune mediated systemic disease and characterized by pauci-immune vasculitis mainly of small and medium vessels with typical necrotizing granulomatous lesions in the affected tissues. A 44 year old lady who was known to have GPA presented with unusual presentation of its category with extraordinary large vessels involvement including pulmonary arteries and her condition improved with immunosuppressive therapy. We reported this case, to improve the awareness about other overlapping categories of vessels vasculitis that may involve large vessels and main pulmonary arteries and to avoid misdiagnosing these patients with category of classical large vessels vasculitis.

scholarly journals 062. UPPER AND LOWER RESPIRATORY TRACT INVOLVEMENT IS SIMILAR IN MPO-ANCA AND PR3-ANCA GRANULOMATOSIS WITH POLYANGIITIS

Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_2) ◽  
Author(s):  
Marco Antonio Alba Garibay ◽  
Susan Hogan ◽  
Ashley Henderson ◽  
Caroline Poulton ◽  
Yichun Hu ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Lower Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Tract Involvement

scholarly journals Upper respiratory tract manifestations in patients with ANCA-associated vasculitides and their association with the presence and type of ANCA

2021 ◽  
Vol 59 (5) ◽  
pp. 555-562
Author(s):  
I. G. Smirnova ◽  
N. M. Bulanov ◽  
P. I. Novikov ◽  
I. A. Osipova ◽  
S. V. Moiseev
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Respiratory Tract ◽  
X Ray ◽  
Saddle Nose Deformity ◽  
Radiological Patterns ◽  
Upper Respiratory ◽  
Eosinophilic Granulomatosis

Aim of the work – to compare the frequency of upper respiratory tract (URT) involvement in patients with ANCAassociated vasculitides (AAV), to reveal its main clinical and radiological patterns and to estimate their association with the serological profile (ANCA presence and type).Material and methods. This retrospective study evaluated 369 patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The enrolled patients were diagnosed with AAV according to the ACR criteria, CHCC classification (2012) and EMA algorithm. Patients with URT manifestations underwent standard ENT assessment and X-ray/CT. Serum ANCA levels were measured by ELISA.Results. URT involvement was diagnosed in 280 (75.9%) patients with AAV. It was significantly more common amongthe patients with GPA (86.4%) and EGPA (85.5%) compared with the MPA group (29.2%) (p<0.001).URT manifestations mainly appeared as sinusitis (77.2% – GPA; 33.3% – MPA; 70.8% – EGPA) and rhinitis with crusting (87.8%, 72.2% and 16.9% respectively).Proteinase 3 ANCA positive patients had a significantly higher incidence of bone destructive URT lesions, including sinuses wall destruction (p<0.001) and saddle nose deformity (p=0.016), compared with myeloperoxidase-ANCA-positive patients. Similar results were obtained in the GPA group separately.Localized disease with isolated URT involvement was observed in 41.3% cases of ANCA negative GPA (p<0.001).Conclusion. The frequency and patterns of upper respiratory tract manifestations depend both on the nosologic form of AAV and type of ANCA. Localized forms of URT involvement can be observed in patients with GPA and are closely associated with absence of ANCA, which determines the need for especially high suspicion level.

Recurrent gastrointestinal bleeding due to jejunal artery vasculitis as debut presentation of granulomatosis with polyangiitis

2021 ◽  
Vol 14 (1) ◽  
pp. e237876
Author(s):  
Saddam Yasin ◽  
Kanwal Mehmood
Keyword(s):  
Mortality Rate ◽  
Respiratory Tract ◽  
Gastrointestinal Bleeding ◽  
Lower Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Surgical Excision ◽  
Gastrointestinal Involvement ◽  
Small Arteries ◽  
Arteries And Veins ◽  
Late Manifestation

Granulomatosis with polyangiitis (GPA) is characterised by systemic necrotising vasculitis of small arteries and veins with multitude of organ involvement, with the most common being the upper and lower respiratory tract and renal system. Gastrointestinal involvement is a rare late manifestation with a high mortality rate and usually results in intestinal perforation. Our patient presented with gastrointestinal bleeding secondary to jejunal artery vasculitis. Gastrointestinal bleeding as initial presentation of GPA is very rarely documented. CT mesenteric angiogram is helpful for the localisation of bleed in these cases. In case of refractory bleeding, surgical excision is required.

scholarly journals Concurrent outbreaks of influenza A and influenza B

1985 ◽  
Vol 94 (3) ◽  
pp. 331-339 ◽  
Author(s):  
D. W. Spelman ◽  
C. J. McHardy
Keyword(s):  
Young Adults ◽  
Tract Infection ◽  
Respiratory Tract ◽  
Clinical Features ◽  
Older Patients ◽  
Lower Respiratory Tract ◽  
Influenza A ◽  
Clinical Syndrome ◽  
Influenza B ◽  
Significant Difference

SUMMARYDuring the winter of 1982 concurrent outbreaks of influenza A and influenza B occurred. The epidemiology and clinical features of 151 cases referred during this time are described, and patients are discussed according to age and presenting clinical syndrome: croup was the commonest presentation in young children, a typical influenza syndrome predominated in young adults, while older patients were more likely to have lower respiratory tract infection. There was no significant difference between the clinical features of influenza A and influenza B. Unusual clinical features include rash, exudative tonsillitis and the need for myringotomy during the course of influenza.

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