Takikawa et al[2
] have been pioneers in establishing the characteristics of PSC in Japanese patients. In an early analysis of 192 patients[2
], they discovered two peaks for age distribution, a characteristic which they reported as “unique”, with the disease being associated more often with IBD in the younger age group. In a more recent study[3
], the same group performed a nationwide survey, comparing the results with their previous study. They reported on male predominance (59%) with a mean age of 47 years at diagnosis. Although most patients were asymptomatic at the time of diagnosis, jaundice (28%) and pruritus (16%) were the most commonly encountered presenting symptoms.
Ponsioen et al[4
] evaluated the natural history of PSC in a Dutch population. Of the 174 patients included in this study, 60% were male, with a mean age of 40.4 years[4
]. In a similar study by Broome et al on 305 Swedish PSC patients, 64% were male and the median age at the time of diagnosis was 39 years (range 5-80)[5
]. Of the symptomatic 171 (56%) patients, abdominal pain (37%), jaundice (30%), pruritus (30%), and fever (17%) were most commonly reported complaints. In a more recent multicenter study on 273 German patients, again male predominance was established at 71.4%, with a mean age at time of diagnosis of 32.4 years (range 9-72 years)[6
]. Slightly more than half of these patients were symptomatic initially, with right upper quadrant abdominal pain once being again the most prevalent symptom (34.4%).
On the other side of the Atlantic, in an early study, Weisner et al[7
] evaluated the natural history of PSC in 174 patients; 37 were asymptomatic and 137/174 (79%) had symptoms related to underlying liver disease. At the time of diagnosis, the mean age was 39.9 years, and 66% of the patients were male. Long-term follow-up (mean: 6.0 years; range: 2.7-15.5 years) was available in all patients. In a more recent population based study in Minnesota by the Mayo clinic, it was projected that approximately 29 000 cases of PSC exist in the white USA population. In this study, the median age at diagnosis was 40 years, and 68% of the patients were men. Although asymptomatic patients with incidental abnormal liver tests was not an infrequent clinical scenario, most patients presented with symptoms of advanced stages of the disease, including jaundice, pruritus, fever, or manifestations of portal hypertension.
In an earlier study from Turkey, the “crossroad between east and west”, by Bayraktar et al[8
] evaluating the association between PSC and IBD, the median age of presentation for patients with PSC was 35 years (range 19-48 years). The most intriguing feature was that 15 of the 16 patients with PSC were females, a predominance that had never been previously reported. Although results of two subsequent studies originating from Turkey[9,10
] were found to be consistent with those from the West, the reasons behind the findings in the Bayraktar study remain elusive.
It would seem that PSC patients worldwide share the same characteristics regarding sex, age and symptoms on presentation (Table ). The only differences of note would be the second peak for age of presentation observed in the Japanese population, as well as of course the overwhelming female predominance reported in the Turkish study.
Comparison of characteristics of PSC patients: European and Japanese studies