A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature

Abstract

Objective-To test the usefulness of the Chapel Hill nomenclature, supplemented with surrogate parameters, as diagnostic criteria for primary vasculitides. Methods-To prospectively evaluate vasculitis patients according to a standardised clinical and para-clinical programme. In accordance with the Chapel Hill publication surrogate parameters were used: proteinuria, haematuria and red blood cell casts (glomerulonephritis), angiographic or ultrasonic demonstration of aneurysms or stenoses (arteritis), radiological lung infiltrates or cavitations of more than one month's duration (granuloma in the lungs), bloody nasal discharge or crusts, chronic sinusitis, otitis and/or mastoiditis, bone and/or cartilage destruction, and acute hearing loss (granuloma in upper airways). Results-The following entities were diagnosed: giant cell arteritis (n=14), Takayasu arteritis (n=1), polyarteritis nodosa (n=2), Wegener's granulomatosis (n=27), ChurgStrauss syndrome (n=2), microscopic polyangiitis (n=12), Henoch-Schönlein purpura (n=2), cutaneous leucocytoclastic angiitis (n=37), and secondary vasculitis (n=21). Giant cell arteritis and cutaneous leucocytoclastic angiitis were in all cases diagnosed by biopsy. Using the Chapel Hill nomenclature supplemented with surrogate parameters, only 8 of 27 patients were diagnosed with Wegener's granulomatosis, and 3 of 12 cases with microscopic polyangiitis. The number of patients in the remaining diagnostic entities were considered to few to evaluate. The most recent proposal was made at the Chapel Hill conference in North Carolina in 1992, where a nomenclature defining 10 primary vasculitides was formulated. Conclusions-The3 The Chapel Hill Group made "great eVorts to adopt names and definitions already widely accepted". However, it diVered fundamentally from other classification schemes in that classic polyarteritis nodosa (PAN) was restricted to necrotising inflammation of medium sized and small arteries only without glomerulonephritis or vasculitis in arterioles, capillaries or venules. Furthermore, IgA dominant immune deposits were required in the definition of HenochSchönlein purpura, and cryoglobulin immune deposits were required for defining essential cryoglobulinaemic vasculitis. In the Chapel Hill description of Wegener's granulomatosis (WG) it was mentioned that necrotising glomerulonephritis was common, and that in microscopic polyangiitis (MPA) necrotising glomerulonephritis was very common. Thus, the presence of glomerulonephritis was not a necessary requirement in the definition of these diseases (table 1).Even though it was emphasised that WG, Churg-Strauss syndrome and MPA are associated with ANCA, the presence of ANCA was not included in the definition of any of these three diseases.Although the Chapel Hill report emphasised that the aim was to create a nomenclature of systemic vasculitides, we felt that the proposals might serve as diagnostic guidelines in the daily clinic, because of the clear definition of the type of vessel involved, and the presence or absen...

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

“…There has yet to emerge a single, unified system that can be applied to clinical diagnosis. 7,8 For most physicians, cutaneous vasculitis evokes a crop of lower-extremity palpable purpura that correlates histologically with small vessel necrotizing vasculitis. 9 For dermatologists, it would be preferable to devise a classification system dedicated to those vasculitides that present cutaneously.…”

Increased serum levels of macrophage migration inhibitory factor (MIF) in patients with microscopic polyangiitis

“…All vasculitis patients were divided into three groups based on the size of the affected vessels following the defi nitions adopted by the Chapel Hill Consensus Conference. 21 Microscopic polyangiitis (MPA) was diagnosed on the basis of the criteria proposed by Sorensen and colleagues 22 (n = 14) and was considered small vessel vasculitis (SVV). Polyarteritis nodosa 23 (PAN; n = 7) was considered medium-sized vessel vasculitis (MVV).…”

Recent Aspects of Vasculitis and Future Direction

“…Sorenson et al defined a surrogate biomarker to CHCC definition for glomerulonephritis and granulomatous inflammation intended for clinical practice. On the other hand, the use of a single classification would lead to more unclassified patients and overlapping diagnosis (25).…”