Sf. Sorensen et al., A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature, ANN RHEUM D, 59(6), 2000, pp. 478-482
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.