Vasculitis—classification and diagnosis

Author(s):  
Richard A. Watts ◽  
David G. I. Scott

The vasculitides are a group of conditions characterized by inflammation and necrosis of blood vessels; they are generally of unknown aetiology. The classification of vasculitides is based on the size of vessel involved and whether there is a known cause (secondary) or not (primary). This approach has stood the test of time. The American College of Rheumatology (ACR) in 1990 produced classification criteria for the major types of vasculitis and in 1994 definitions were promulgated by the Chapel Hill Consensus Conference. These did not include anti-neutrophil cytoplasm antibodies (ANCA) and the ACR scheme did not include microscopic polyangiitis. The definitions have recently been updated to include modern concepts of pathogenesis including ANCA. No validated diagnostic criteria are available for routine clinical practice. The diagnosis of vasculitis requires a high index of suspicion, especially in the systemically unwell patient with multiorgan involvement. The key to diagnosis is a detailed and systematic approach to patient assessment involving all potentially involved organs. In a patient with suspected vasculitis immediate urinalysis is mandatory as the severity of renal involvement at presentation is a major determinant of outcome. Each potentially involved organ should be comprehensively evaluated. Tissue biopsy should be obtained whenever possible, as treatment is potentially toxic using glucocorticoids combined with cytotoxic agents. Biopsy should not, however, delay initiation of treatment. Potential alternative diagnosis should be considered, especially infection and malignancy, and excluded whenever possible.

Author(s):  
Richard A. Watts ◽  
David G. I. Scott

The vasculitides are a group of conditions characterized by inflammation and necrosis of blood vessels; they are generally of unknown aetiology. The classification of vasculitides is based on the size of vessel involved and whether there is a known cause (secondary) or not (primary). This approach has stood the test of time. The American College of Rheumatology (ACR) in 1990 produced classification criteria for the major types of vasculitis and in 1994 definitions were promulgated by the Chapel Hill Consensus Conference. These did not include anti-neutrophil cytoplasm antibodies (ANCA) and the ACR scheme did not include microscopic polyangiitis. The definitions have recently been updated to include modern concepts of pathogenesis including ANCA. No validated diagnostic criteria are available for routine clinical practice. The diagnosis of vasculitis requires a high index of suspicion, especially in the systemically unwell patient with multiorgan involvement. The key to diagnosis is a detailed and systematic approach to patient assessment involving all potentially involved organs. In a patient with suspected vasculitis immediate urinalysis is mandatory as the severity of renal involvement at presentation is a major determinant of outcome. Each potentially involved organ should be comprehensively evaluated. Tissue biopsy should be obtained whenever possible, as treatment is potentially toxic using glucocorticoids combined with cytotoxic agents. Biopsy should not, however, delay initiation of treatment. Potential alternative diagnosis should be considered, especially infection and malignancy, and excluded whenever possible.


2018 ◽  
Vol 90 (6) ◽  
pp. 55-58
Author(s):  
E M Shchegoleva ◽  
N M Bulanov ◽  
P I Novikov ◽  
S V Moiseev

Aim. To evaluate clinical features and outcomes of renal involvement in patients with microscopic polyangiitis (MPA). Materials and methods: We enrolled 99 patients with MPA, diagnosed in accordance with the algorithm of the European Medicines Evaluation Agency (EMEA) and the Chapel Hill consensus conference definition (2012). Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and proteinuria were estimated. Frequency of rapidly progressive renal failure (a twofold increase in the sCr level in ≤3 months) was regarded as the clinical equivalent of rapidly progressive glomerulonephritis (RPGN). Results and discussion. Renal involvement was present in 92 (92.9%) patients. RPGN developed in 51 (55,4%) patients. The most common features of kidney involvement were hematuria and subnephrotic proteinuria. Arterial hypertension was revealed in 32 (34.7%) patients and was associated with RPGN (p


2020 ◽  
Author(s):  
Nestor Oliva-Damaso ◽  
Andrew S Bomback

Abstract The nomenclature for antineutrophil cytoplasmic antibody (ANCA)-associated kidney disease has evolved from honorific eponyms to a descriptive-based classification scheme (Chapel Hill Consensus Conference 2012). Microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis do not correlate with presentation, response rates and relapse rates as when comparing myeloperoxidase versus leukocyte proteinase 3. Here we discuss the limitations of the currently used classification and propose an alternative, simple classification according to (i) ANCA type and (ii) organ involvement, which provides important clinical information of prognosis and outcomes.


Author(s):  
Elena Lopez-Francos ◽  
Sammy Place ◽  
Daphnee Delplace ◽  
Frédéric Vandergheynst

We describe a 66-year old patient with a recurrent ulcer on her right ankle. Biopsy revealed medium-vessel vasculitis consistent with cutaneous polyarteritis nodosa. There were no signs or symptoms suggestive of systemic vasculitis, but a 18FDG-PET scan showed areas of increased uptake around the large arteries and the pelvic and shoulder girdles. These findings suggested polymyalgia rheumatica in the setting of large-vessel vasculitis. This case thus supports the statement from the Chapel-Hill consensus conference that classification of systemic vasculitis by vessel size is based on the vessels predominantly involved, but vessels of other sizes may also be affected.


2018 ◽  
Vol 90 (6) ◽  
pp. 15-21 ◽  
Author(s):  
N M Bulanov ◽  
E A Makarov ◽  
E M Shchegoleva ◽  
A S Zykova ◽  
E S Vinogradova ◽  
...  

Objective. To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA). Materials and methods. In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016). Results. Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p


2020 ◽  
Vol 96 (1) ◽  
pp. 18-27
Author(s):  
Vladislav R. Khairutdinov ◽  
Irena E. Belousova ◽  
Aleksey V. Samtsov

Cutaneous vasculitis is a heterogeneous group of diseases caused by inflammation of the blood vessel wall of the skin. The variety of cutaneous vasculitis, their clinical similarity, lack of clear diagnostic criteria and common terminology cause an extremely complex process of diagnosis of this group of diseases. A dermatologist is often the first specialist to diagnose vasculitis. Currently there is no single "monodisciplinary" classification of v ascular damage, there is no generally accepted terminology and classification of cutaneous vasculitis. In domestic dermatovenerology, vasculitis is classified according to the morphological elements of the skin rash, and not by etiology and pathogenesis. This makes it difficult for different specialists to understand the diagnosis and maintain continuity in the management of patients. The article presents the nomenclature of vasculitis of the 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides, supplemented by organ-specific cutaneous vasculitis, describes the pathomorphological and clinical manifestations of this group of dermatoses, summarizes information about modern treatment tactics.


2013 ◽  
Vol 154 (40) ◽  
pp. 1571-1578
Author(s):  
Margit Zeher

Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future. Orv. Hetil., 2013, 154, 1571–1578.


2012 ◽  
Vol 65 (1) ◽  
pp. 1-11 ◽  
Author(s):  
J. C. Jennette ◽  
R. J. Falk ◽  
P. A. Bacon ◽  
N. Basu ◽  
M. C. Cid ◽  
...  

2017 ◽  
Vol 18 (5) ◽  
pp. 786 ◽  
Author(s):  
Jee Hye Hur ◽  
Eun Ju Chun ◽  
Hyon Joo Kwag ◽  
Jin Young Yoo ◽  
Hae Young Kim ◽  
...  

2020 ◽  
pp. 4573-4579
Author(s):  
Richard A. Watts

Small vessel vasculitis is vasculitis affecting predominately small intraparenchymal arteries, arterioles, capillaries, and venules. There are two main types: antineutrophil cytoplasmic antibody associated and immune complex mediated. The ANCA associated vasculitides are discussed in chapter 19.3 IgA vasculitis (IgAV) was formerly known as Henoch Schönlein purpura. The revised nomenclature reflects the importance of IgA vasculitis in pathogenesis. The Chapel Hill Consensus Conference defined IgA vasculitis as ‘vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)’. IgA vasculitis often involves skin and gut, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Its aetiology is unknown, but it frequently occurs after an infection several days to weeks before. The most frequently isolated organism is beta-haemolytic streptococcus. Drugs such as a penicillin, ampicillin, erythromycin, and non-steroidal anti-inflammatory drugs have been reported as precipitating agents. There is an association with HLA-DRB1*01 in Caucasians and there appears to be a familial association.


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