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Войти в исследования Seminars in arthritis and rheumatism · 2026
Epidemiology and clinical characteristics of polyarteritis nodosa in Japan: findings from a nationwide survey.
СтатьяНазвание и аннотация: Перевод в очереди
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Kawazoe Mai, Nanki Toshihiro, Saeki Keigo, Ishikawa Hideki, Nakamura Yoshikazu, Kawashima Soko, Ito Shuichi, Kodera Masanari, Konda Naoko, Kaname Shinya, Dobashi Hiroaki, Tamura Naoto, Harigai Masayoshi, Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis (JPVAS)
Аннотация
OBJECTIVES: This study aimed to elucidate the epidemiological and clinical characteristics of patients with polyarteritis nodosa (PAN) in Japan.
METHODS: We sent a secondary questionnaire to facilities randomly selected from a nationwide list of hospitals treating patients with PAN in the first nationwide epidemiological survey.
RESULTS: We analyzed data from 547 patients out of the 868 reported in the first survey. Of these, 233 were male and 314 were female (male-to-female ratio, 1:1.3), with a mean age at diagnosis of 51.7 ± 17.6 years. Fever was observed in 43.4% of cases. Among organ manifestations, skin involvement (82.4%) and joint/muscle involvement (51.6%) were the most frequently reported. Most patients (95.4%) were prescribed oral glucocorticoids, and concomitant immunosuppressants were used in 78.6%. Frequently administered agents included azathioprine (59.3%) and cyclophosphamide (49.3%). Remission was achieved in 91.2% of cases, but relapse occurred in 47.8%. Skin involvement was identified as a risk factor for relapses, while fever was associated with a lower risk. Of the 547 patients, 14.4% met the diagnostic criteria for cutaneous arteritis; the male-to-female ratio was 1:3.9, and the mean age at onset was 47.5 ± 14.5 years. Additionally, 22 patients had pediatric-onset PAN, defined as disease onset at age 18 years or younger, exhibiting a higher male ratio (1.8:1), and a higher frequency of fever and skin involvement.
CONCLUSIONS: This epidemiological study revealed the number and sex ratio of patients with PAN in Japan, along with their clinical characteristics. It also detailed the features of cutaneous arteritis.
METHODS: We sent a secondary questionnaire to facilities randomly selected from a nationwide list of hospitals treating patients with PAN in the first nationwide epidemiological survey.
RESULTS: We analyzed data from 547 patients out of the 868 reported in the first survey. Of these, 233 were male and 314 were female (male-to-female ratio, 1:1.3), with a mean age at diagnosis of 51.7 ± 17.6 years. Fever was observed in 43.4% of cases. Among organ manifestations, skin involvement (82.4%) and joint/muscle involvement (51.6%) were the most frequently reported. Most patients (95.4%) were prescribed oral glucocorticoids, and concomitant immunosuppressants were used in 78.6%. Frequently administered agents included azathioprine (59.3%) and cyclophosphamide (49.3%). Remission was achieved in 91.2% of cases, but relapse occurred in 47.8%. Skin involvement was identified as a risk factor for relapses, while fever was associated with a lower risk. Of the 547 patients, 14.4% met the diagnostic criteria for cutaneous arteritis; the male-to-female ratio was 1:3.9, and the mean age at onset was 47.5 ± 14.5 years. Additionally, 22 patients had pediatric-onset PAN, defined as disease onset at age 18 years or younger, exhibiting a higher male ratio (1.8:1), and a higher frequency of fever and skin involvement.
CONCLUSIONS: This epidemiological study revealed the number and sex ratio of patients with PAN in Japan, along with their clinical characteristics. It also detailed the features of cutaneous arteritis.