What are the classification criteria for small-vessel vasculitides?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
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Henoch-Schönlein purpura is the most common vasculitis in children, and is associated with immunoglobulin A (IgA) immune deposition in small vessels. Presenting features include palpable purpura, abdominal pain (which may be associated with GI hemorrhage and/or intussusception), arthritis, and evidence of glomerulonephritis (hematuria, proteinuria).

Classification criteria for Henoch-Schönlein purpura include palpable purpura (mandatory criterion) in the presence of at least one of following 4 features:

  • Diffuse abdominal pain

  • Any biopsy finding that reveals predominant lgA deposition

  • Arthritis or arthralgia (arthritis is acute in any joint)

  • Renal involvement (any hematuria, proteinuria)

Antineutrophil cytoplasmic antibody (ANCA) positive small-vessel vasculitides are also seen in the pediatric population. Granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis) is a granulomatous vasculitis that most commonly involves the sinopulmonary system but can involve any organ system. Presenting features may include purpuric rash, recurrent sinusitis, epistaxis, shortness of breath, and/or hemoptysis from alveolar hemorrhage. It is also commonly associated with a necrotizing glomerulonephritis that may cause significant renal impairment. [5] Patients with Wegener granulomatosis have a positive ANCA finding in 90% of cases, most frequently in a cytoplasmic pattern (c-ANCA), with antibodies against proteinase 3 (anti-PR3). [6]

Classification criteria for GPA include 3 of the following 6 features:

  • Abnormal urinalysis findings (hematuria and/or significant proteinuria)

  • Granulomatous inflammation on biopsy (If a kidney biopsy is done it characteristically shows necrotizing pauci-immune glomerulonephritis.)

  • Nasal sinus inflammation

  • Subglottic, tracheal, or endobronchial stenosis

  • Abnormal chest radiography or CT findings

  • PR3 ANCA or c-ANCA staining

Microscopic polyangiitis (MPA) is a necrotizing vasculitis associated with glomerulonephritis and pulmonary capillaritis. Presenting features include purpuric rash, proteinuria and/or hematuria, hemoptysis, CNS involvement, and arthralgias. [7] MPA is associated with ANCA, most commonly with a perinuclear pattern (p-ANCA) and antibodies against myeloperoxidase (anti-MPO).

Churg-Strauss syndrome (CSS) is an eosinophilic granulomatous vasculitis characterized predominantly by pulmonary involvement. Patients typically have a previous history of asthma, allergic rhinitis, and/or sinusitis. A characteristic feature is the finding of nonfixed pulmonary infiltrates. Eosinophilic infiltration results in multiorgan involvement, including neuropathy and cardiovascular disease (pericarditis). [8] ANCA positivity is seen in approximately 40% of patients, usually with an “atypical” or “indeterminate” pattern.

Isolated cutaneous leukocytoclastic vasculitis can be either primary (rarely) or secondary to various medications, infections, or collagen vascular disease.

Hypocomplementemic urticarial vasculitis is a cutaneous vasculitis that may result from primary hypocomplementemia or as part of a disease associated with low complement levels (eg, systemic lupus erythematosus).

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