EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

Abstract

Objectives To validate the previously proposed classifi cation criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). MethodsStep 1: retrospective/prospective webdata collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis ≤18 years.Step 2: blinded classifi cation by consensus panel of a representative sample of 280 cases.Step 3: statistical (sensitivity, specifi city, area under the curve and κ-agreement) and nominal group technique consensus evaluations. Results 827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classifi ed as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classifi cation of c-PAN required a systemic infl ammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of fi ve criteria: (1) skin involvement; (2) myalgia/ muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classifi cation of c-WG required three of six criteria: (1) histopathological evidence of granulomatous infl ammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classifi cation of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of fi ve criteria: (1) pulse defi cit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) Paediatric Rheumatology European Society propose validated classifi cation criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specifi city. INTRODUCTIONIn 1990 the American College of Rheumatology (ACR) proposed classifi cation criteria for patients with vasculitides 1-5 by analysing 807 adults patients with different form of vasculitis: 85 with Henoch-Schönlein purpura (HSP), 118 with polyarteritis nodosa (PAN), 85 with Wegener granulomatosis (WG), 63 with Takayasu arteritis (TA) and 456 with other vasculitides (Churg-Strauss, hypersensitivity, giant cell arteritis and other unspecifi ed forms). 6 Patients with each specifi c vasculitis were compared with all the remaining diseases grouped into a single control category.The ACR criteria for HSP (sensitivity 87.1%, specifi city 87.7%) require the presence of at least two of the following: (1) age ≤20 years at disease onset; (2) palpable purpura; (3) acute abdominal pain; (4) biopsy showing granulocytes in the walls of small arterioles/venules. 1 The ACR criteria for PAN (sensitivity 82.2%, specifi city 86.6%) require at least three of the 10 following criteria: (1) granulocyte or mixed leucocyte infi ...

Vasculitis: do we know more to classify better?

“…With this background, in 2005, a preliminary classification criteria were proposed by a group of pediatric rheumatologists and nephrologists for some of the most common vasculitides in childhood, namely HSP (IgAV), KD, polyarteritis nodosa (PAN), granulomatous polyangiitis (GPA) [at that time Wegener granulomatosis (WG)] and Takayasu arteritis (TA) [15]. At the 2008 Ankara Consensus Conference, these criteria were validated, and the final form was endorsed by European League Against Rheumatism (EULAR), Paediatric Rheumatology European Society (PRES) and the Pediatric Rheumatology International Trials Organization (PRINTO) [10,11].…”

“…the members of the expert panel were provided with 17 points to be considered in the development of revised classification and diagnostic criteria [16]. Many of these points had already been incorporated in the Ankara 2008 criteria, such as the use of ANCA, magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the diagnosis [10]. This study also emphasized the need for the development of a classification tree, which was provided by CHCC 2012 [6].…”

“…The differences between the ACR criteria [32,[36][37][38] and the Ankara 2008 criteria [10] are summarized in Table 2.…”

“…IgAV (Henoch-Schönlein purpura) During the revision process at the 2008 Ankara Consensus Conference, palpable purpura became a mandatory criterion, and any biopsy demonstrating "predominant" IgA deposits was accepted to be consistent with IgAV (HSP) [10]. This revision was mainly possible due to recent observations that IgA immune-complexes define the pathogenesis of the disease [10,15].…”

“…The American College of Rheumatology (ACR) and Ankara Consensus Conference (Ankara) 2008 criteria are classification criteria [8][9][10][11].…”