Background: Clinical phenotype of ANCA-associated vasculitis (AAV) is different between Japan and European countries with microscopic polyangiitis (MPA) predominating in Japan.¹

Objectives: To investigate the diagnosis and prognosis of AAV in a community-based teaching hospital in Japan.

Methods: We performed a retrospective chart review of adult patients with AAV who admitted to Kameda Medical Center from January 2003 to July 2011. We evaluated the diagnoses by applying the European Medicines Agency (EMEA) algorithm² and examined the remission rates. Remission is defined as symptom free, normal CRP/ESR and absence of renal disease activity.

Results: Forty-eight patients were included [33females; mean age at the time of diagnosis, 70.5 years (range, 23–92)]. The mean follow-up was 31.2 months (range, 0.5–92). The original diagnoses of 48 patients were Churg-Strauss syndrome (CSS) (n=7), granulomatosis with polyangitis (GPA) (n=9), MPA (n=32). After applying the EMEA algorithm, 4 of 32 patients with MPA were classified as GPA, and 4 as unclassified vasculitis. One patient with GPA was classified as MPA. ANCA tests were performed by enzyme-linked immunosorbent assay (for MPO-ANCA and PR3–ANCA) for all patients. Thirty-five (72.9%) and 5 (10.4%) patients were MPO-ANCA and PR3–ANCA positive respectively, and other one patient was both MPO-ANCA and PR3–ANCA positive. Among all, 34 patients received glucocorticoid (GC) alone (GC group), and 11 patients were treated with GC/cyclophosphamide (CYC) combination (CYC group). There were no difference in 6 months remission rates and GC dose at 12 months between two groups; however, remission rate at 12 months was numerically higher in CYC group (45.8% GC group vs. 87.5% CYC group). A total of 13 deaths were observed. These deaths were predominantly vasculitis-related, explaining 8/13 (62%) of them and comprised the following: acute renal failure for 3, interstitial lung disease for 2, alveolar hemorrhage for 1, cerebral infarction for 1, and cardiomyopathy for 1. Three of 13 (23%) deaths were attributed to infection: septic shock for 2 (1 with pyelonephritis and 1 with cholecystitis), and pneumonia for 1. The remaining 2 (15%) deaths were attributed to miscellaneous causes: congestive heart failure for 1 and sudden death for 1.Overall estimated 5 year survival rate was 63.2% by using Kaplan-Meier method, and it was 78.3% in patients with the Five-Factors Score (FFS)³ =0 (54.1% FFS ≥1, 54.5% FFS ≥2).

Conclusions: MPA accounted for the large part of AAV, as in previous studies. It is the same also after applying EMEA algorithm. At one year, more patients tend to be in remission in CYC group compared to GC group.

Disclosure of Interest: None Declared