Clinical outcome and prognosis of anti-neutrophil cytoplasmic antibody-associated vasculitis in Japan.
OBJECTIVE
We conducted a broad survey of 99 patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and investigated both prognosis and outcomes.


METHODS
Clinical data evaluated were age, sex, patient survival, renal survival, serum albumin, serum creatinine, urinary protein, hematuria, C-reactive protein (CRP), ANCA titer, IgG and the Birmingham Vasculitis Activity Score (BVAS).


RESULTS
The patient survival rate at 6 months after onset was 84.8%, and that at 2 years after onset was 82.0%. Most deaths were within 6 months of onset. Infection accounted for 9 deaths (60.0%). Infection together with pulmonary involvement of active vasculitis accounted for 2 deaths (13.3%). Organ-specific involvement of active vasculitis alone caused 3 deaths (20.0%). Others died of cardiac events. At 1 and 3 months after onset, BVAS (p < 0.0001, p = 0.002), albumin (p = 0.006, p = 0.0004) and CRP (p = 0.04, p = 0.0002) were also associated with patient death.


CONCLUSIONS
To improve the prognosis of those with ANCA-associated vasculitis, the intensity of initial treatment should be aimed at disease severity. Employing BVAS improved the ability to evaluate therapeutic responses. Finally, prescription with sulfamethoxazole-trimethoprim during the induction therapy with immunosuppressive agents may be advised.
Itabashi
Mitsuyo
M
Department of Medicine, Kidney Center, Tokyo Women's Medical University, Tokyo, Japan. m-itaba@fb-4.so-net.ne.jp
Takei
Takashi
T
Yabuki
Yasuko
Y
Suzuki
Hitoe
H
Ando
Minoru
M
Akamatsu
Mayuko
M
Yamazaki
Mayuko
M
Mitobe
Michihiro
M
Watanabe
Yoshihiko
Y
Mochizuki
Takahiro
T
Nitta
Kosaku
K
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000401:mortality* / Q000628:therapy*; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D015995:Prevalence; D011379:Prognosis; D018570:Risk Assessment / Q000379:methods; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents