A Prospective Investigation, 1982-1985 PER BOESEN and S(bREN FREIESLEBEN SqRENSEN The annual incidence of giant cell arteritis (the term used in this study to encompass the syndromes of temporal arteritis and polymyalgia rheumatica, occurring either together or alone) was prospectively determined in a Danish county that had a population of approximately 200,000. In a single year, 46 new cases of giant cell arteritis were diagnosed, a number which corresponds to an incidence in the overall population of 21.5/105, and to an incidence of 76.6/105 for individuals age 50 years or older. These rates are higher than those previously reported in retrospective studies. The 3-year followup of all patients showed no onset of other diseases that would require a revision of the original diagnosis. There was no deviation from the age-and sex-specific malignancy rate or the mortality rate in the overall population. Women had an incidence rate 4 to 5 times higher than that seen in men. Symptoms, for the most part, were the same as those found in other studies; however, vision loss was not observed during the followup period. Point prevalence at the start of the study was 37.8/105, which is below the rates previously reported. This is probably because of failure on the part of participating physicians to record all cases."Pure" temporal arteritis is characterized by symptoms such as headache, scalp tenderness, visual disturbances, and jaw claudication. On physical examination, the temporal artery may be normal or abnor-

FTD-3 shares clinical and pathologic features with other forms of FTD and fulfills international consensus criteria for FTD. There is involvement of the parietal lobes clinically, radiologically, and pathologically in FTD-3 in contrast to some forms of FTD. This more diffuse involvement of the cerebral cortex leads to a distinctive, global pattern of reduced blood flow on PET scanning.

We aimed to provide an overview of kidney allocation policies related to children and pediatric kidney transplantation (KTx) practices and rates in Europe, and to study factors associated with KTx rates. A survey was distributed among renal registry representatives in 38 European countries. Additional data were obtained from the ESPN/ERA-EDTA and ERA-EDTA registries. Thirty-two countries (84%) responded. The median incidence rate of pediatric KTx was 5.7 (range 0À13.5) per million children (pmc). A median proportion of 17% (interquartile range 2À29) of KTx was performed preemptively, while the median proportion of living donor KTx was 43% (interquartile range 10À52). The median percentage of children on renal replacement therapy (RRT) with a functioning graft was 62%. The level of pediatric prioritization was associated with a decreased waiting time for deceased donor KTx, an increased pediatric KTx rate, and a lower proportion of living donor KTx. The rates of pediatric KTx, distribution of donor source and time on waiting list vary considerably between European countries.The lack of harmonization in kidney allocation to children raises medical and ethical issues. Harmonization of pediatric allocation policies should be prioritized.

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...