Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

Abstract

IntroductionWe investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.MethodsIn total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.ResultsUsing the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).ConclusionsMPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.Trial RegistrationThe University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.

Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

“…In the RemIT-JAV study, we confirmed that MPO-ANCA-positive MPA/renal limited vasculitis RLV) was the most common form of AAV in Japanese patients and revealed that one-half of patients with GPA had MPO-ANCA positivity and that interstitial lung disease (ILD) was an important clinical manifestation in Japanese patients with AAV [5,6]. This high MPO-ANCA positivity in patients with GPA was also confirmed in a recent retrospective report [7].…”

“…The patients with threatened vital organ function were classified as having a generalized disease, and the patients with organ failure were classified as having severe disease. Detailed definitions of disease severity were described in our previous report [5].…”

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

“…Although this has been previously reported in the literature (4,5,7,8,(27)(28)(29)(30)(31), we analyzed CT images in sequential hospital-based patients before they received treatment and determined the prevalence of each imaging component of ILD (Table 2). Ground-glass opacity was the most prevalent component, which indicates that a large proportion of MPA patients have an active inflammatory process in the lungs before they receive treatment.…”

Fatal cardiac small-vessel involvement in ANCA-associated vasculitis: an autopsy case report

“…Pauci-immune type necrotizing crescentic glomerulonephritis is common in GPA, as well as MPA. In the Japanese cohort, 54.6% of the patients with GPA are positive for serum MPO-ANCA with frequency noted to be higher than in western countries [1]. Lastly, EGPA is an eosinophil-rich and necrotizing granulomatous vasculitis that predominantly affects small to medium-sized vessels.…”

“…MPA is a necrotizing vasculitis that preferentially affects the renal glomeruli with few immune deposits. In Japan, the majority of patients with MPA are positive for serum myeloperoxidase (MPO)-ANCA [1]. Inflammation that is not centered on the vessels, including granulomatous inflammation, is generally absent.…”