Do vasculitis categorization systems really matter?

Jennette, J. Charles; Rj, Falk

doi:10.1007/s11926-000-0044-4

Cited by 32 publications

(10 citation statements)

References 32 publications

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“…14,15 For instance, hepatitis C infection can be found in association with several different types of vasculitis such as polyarteritis nodosa (PAN), cryoglobulinemic vasculitis (CV), and hypersensitivity/cutaneous leukocytoclastic angiitis (CLA). 14,15 For instance, hepatitis C infection can be found in association with several different types of vasculitis such as polyarteritis nodosa (PAN), cryoglobulinemic vasculitis (CV), and hypersensitivity/cutaneous leukocytoclastic angiitis (CLA).…”

Section: Diagnosis and Classification Of Vasculitismentioning

confidence: 99%

Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and Prognosis

Carlson

Ng²,

Chen³

2005

The American Journal of Dermatopathology

204

238

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Vasculitis, inflammation of the vessel wall, can result in mural destruction with hemorrhage, aneurysm formation, and infarction, or intimal-medial hyperplasia and subsequent stenosis leading to tissue ischemia. The skin, in part due to its large vascular bed, exposure to cold temperatures, and frequent presence of stasis, is involved in many distinct as well as un-named vasculitic syndromes that vary from localized and self-limited to generalized and life-threatening with multi-organ disease. To exclude mimics of vasculitis, diagnosis of cutaneous vasculitis requires biopsy confirmation where its acute signs (fibrinoid necrosis), chronic signs (endarteritis obliterans), or past signs (acellular scar of healed arteritis) must be recognized and presence of extravascular findings such as patterned fibrosis or collagenolytic granulomas noted. Although vasculitis can be classified by etiology, many cases have no identifiable cause, and a single etiologic agent can elicit several distinct clinicopathologic expressions of vasculitis. Therefore, the classification of cutaneous vasculitis is best approached morphologically by determining vessel size and principal inflammatory response. These histologic patterns roughly correlate with pathogenic mechanisms that, when coupled with direct immunofluorescent examination, anti-neutrophil cytoplasmic antibody (ANCA) status, and findings from work-up for systemic disease, allow for specific diagnosis, and ultimately, more effective therapy. Herein, we review cutaneous vasculitis focusing on diagnostic criteria, classification, epidemiology, etiology, pathogenesis, and evaluation of the cutaneous vasculitis patient.

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Section: Diagnosis and Classification Of Vasculitismentioning

confidence: 99%

Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and Prognosis

Carlson

Ng²,

Chen³

2005

The American Journal of Dermatopathology

204

238

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“…3 The major advances in the classification of vasculitis have been in the recognition of dominant blood vessel size involved, the distinction between primary and secondary vasculitis, and the incorporation of pathophysiologic markers such as direct immunofluorescent (DIF) examination for vascular and/or basement membrane zone immune-complexes, and antineutrophil cytoplasmic antibodies (ANCA). 3,5,6 In clinical practice, the goal for a working classification of vasculitis affecting the skin should incorporate clinical relevance to management, and address clinical, histologic and laboratory features, and underlying causes. Specifically, classification of cutaneous vasculitis should be based on the clinical pattern (distribution, type of primary lesion, and resultant secondary changes, including ulceration or bullae formation), laboratory findings (eg, DIF and ANCA results), and histologic characteristics identified in a primary lesion.…”

Section: Skin Biopsy and The Histologic Classification Of Vasculitismentioning

confidence: 99%

Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

Carlson

Kr²

2006

The American Journal of Dermatopathology

183

151

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A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis.

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“…These classification criteria were primarily developed to facilitate and improve research efforts by defining consistent and homogeneous patient populations for clinical protocols and outcome studies [2]. They were not developed to diagnose disease in individuals.…”

Section: Classification and Epidemiology Of Childhood Vasculitismentioning

confidence: 99%

ANCA-associated vasculitides—lessons from the adult literature

2010

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Anti-neutrophil cytoplasmic antigen antibody (ANCA)-associated disease is a rare manifestation of primary systemic vasculitis in paediatric patients but one that carries significant morbidity, potential long-term disability and early mortality. It therefore requires a high index of suspicion, targeted investigation, prompt treatment and long-term follow-up with specialist input at every stage. The well-recognised diversity and overlap in clinical, laboratory and histopathological features of the ANCA-associated systemic vasculitides continue to hamper accurate diagnosis, confounding epidemiological data and necessitating a blanket approach to treatment, which is largely extrapolated from studies in adult patients and carries significant side-effects. Herein we summarise current knowledge of the epidemiology, pathogenesis, principal manifestations, investigation and evidence-based management, extrapolated from adult studies, of these disorders. We also discuss recent efforts towards classification of the childhood vasculitides that emphasise the value of histological diagnosis. Progress in our understanding of the pathophysiology underlying ANCA-associated disease should lead to targeted, safer and more effective therapies for these conditions. Nonetheless, many questions remain outstanding, and academic paediatricians face real challenges in identifying and collating the few cases they encounter into study cohorts. Meeting this challenge will require international collaboration, not only among paediatricians but also with the specialists taking over care of these patients as they reach adulthood.

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Do vasculitis categorization systems really matter?

Cited by 32 publications

References 32 publications

Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and Prognosis

Cutaneous Vasculitis Update: Diagnostic Criteria, Classification, Epidemiology, Etiology, Pathogenesis, Evaluation and Prognosis

Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

ANCA-associated vasculitides—lessons from the adult literature

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