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

Objective-To test the usefulness of the Chapel Hill nomenclature, supplemen ted with surrogate parameters, as diagnostic criteria for primary vasculiti des. Methods-To prospectively evaluate vasculitis patients according to a standa rdised clinical and para-clinical programme. In accordance with the Chapel Hill publication surrogate parameters were used: proteinuria, haematuria an d red blood cell. casts (glomerulonephritis), angiographic or ultrasonic de monstration of aneurysms or stenoses (arteritis), radiological lung infiltr ates or cavitations of more than one month's duration (granuloma in the lun gs), bloody nasal discharge or crusts, chronic sinusitis, otitis and/or mas toiditis, bone and/or cartilage destruction, and acute hearing loss (granul oma in upper airways). Results-The following entities were diagnosed: giant cell arteritis (n=14), Takayasu arteritis (n=1), polyarteritis nodosa (n=2), Wegener's granulomat osis (n=27), Churg-Strauss syndrome (n=2), microscopic polyangiitis (n=12), Henoch-Schonlein purpura (n=2), cutaneous leucocytoclastic angiitis (n=37) , and secondary vasculitis (n=21). Giant cell. arteritis and cutaneous leuc ocytoclastic angiitis were in all cases diagnosed by biopsy. Using the Chap el Hill nomenclature supplemented with surrogate parameters, only 8 of 27 p atients were diagnosed with Wegener's granulomatosis, and 3 of 12 cases wit h microscopic polyangiitis. The number of patients in the remaining diagnos tic entities were considered to few to evaluate. Conclusions-The Chapel Hill nomenclature, supplemented with surrogate param eters, failed to act as diagnostic criteria in Wegener's granulomatosis and microscopic polyangiitis. The following diagnostic criteria are proposed f or Wegener's granulomatosis: (1) Biopsy or surrogate parameter for granulom atous inflammation in the respiratory system and (2) Biopsy verified necrot ising vasculitis in small to medium sized vessels or biopsy/surrogate param eter for glomerulonephritis or positive PR3-ANCA test and (3) Lack of eosin ophilia in blood and biopsy samples. The following diagnostic criteria are proposed for microscopic polyangiitis: (1) Biopsy verified necrotising vasc ulitis in small vessels and/or glomerulonephritis with few or no immune dep osits and (2) Involvement of more than one organ system as indicated by bio psy verified vasculitis in small to medium sized vessels or surrogate param eter for glomerulonephritis and (3) Lack of biopsy and surrogate parameter for granulomatous inflammation in the respiratory system. Using these crite ria all Wegener's patients and 9 of 12 patients with microscopic polyangiit is could be diagnosed.