Difference in relapse-rate and clinical phenotype by autoantibody-subtype in Japanese patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.
OBJECTIVE
To correlate the serotype specificity to myeloperoxidase (MPO) and proteinase-3 (PR3) with clinical characteristics in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).


METHODS
Clinical characteristics and outcomes of patients with AAV in our division from 2005 to 2014 were retrospectively compared on the basis of ANCA subtype.


RESULTS
We collected the data from 88 patients with MPO-ANCA vasculitis, and 17 with PR3-ANCA vasculitis. Patients with PR3-ANCA vasculitis were younger, and had higher involvement-rates in the eye, nose, and ear. In both MPO- and PR3-ANCA vasculitis, the most frequently involved organ was the respiratory system. Interstitial pneumonia was more frequent in MPO-ANCA vasculitis (52.3% versus 5.9%, p < 0.01), whereas nodular shadow was more frequent in PR3-ANCA vasculitis (9.1% versus 58.8%, p < 0.01). Multivariable Cox proportional hazard regression analysis showed that the hazard ratio of PR3-ANCA for relapse was 2.48 (95% confidence interval 1.14-5.42, p = 0.02). There was no difference in the survival and the progression to end-stage kidney disease and respiratory failure between the two vasculitides.


CONCLUSIONS
MPO-ANCA vasculitis was a predominant form of AAV in Japan. Classification based on ANCA subtype would be clinically relevant in the prediction of organ involvement and relapse.
a Division of Rheumatology and Clinical Immunology, Department of Medicine , Jichi Medical University , Shimotsuke , Tochigi , Japan.
Takamasa
T
Murosaki
a Division of Rheumatology and Clinical Immunology, Department of Medicine , Jichi Medical University , Shimotsuke , Tochigi , Japan.
Takeo
T
Sato
a Division of Rheumatology and Clinical Immunology, Department of Medicine , Jichi Medical University , Shimotsuke , Tochigi , Japan.
Yoichiro
Y
Akiyama
a Division of Rheumatology and Clinical Immunology, Department of Medicine , Jichi Medical University , Shimotsuke , Tochigi , Japan.
Katsuya
K
Nagatani
a Division of Rheumatology and Clinical Immunology, Department of Medicine , Jichi Medical University , Shimotsuke , Tochigi , Japan.
Seiji
S
Minota
D000328:Adult; D000367:Age Factors; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001323:Autoantibodies / Q000032:analysis; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D010641:Phenotype; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors
D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D009195:Peroxidase; D053146:Myeloblastin
ANCA-associated vasculitis; MPO-ANCA; PR3-ANCA; Relapse; Survival