Incidence of ANCA-Associated Primary Renal Vasculitis in the Miyazaki Prefecture

Fujimoto, Shouichi; Uezono, Shigehiro; Hisanaga, Shuichi; Fukudome, Keiichi; Kobayashi, Shigeto; Suzuki, Kazuo; Hashimoto, Hiroshi; Nakao, Hiroyuki; Nunoi, Hiroyuki

doi:10.2215/cjn.01461005

Cited by 94 publications

(64 citation statements)

References 30 publications

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“…The geographical studies of AAV indicated the predominance of MPA and MPO-ANCA positivity as epidemiologically specific to Japan, whereas GPA and PR3-ANCA positivities are dominant in European countries. 9,[17][18][19] According to the results in the present study, this Japanese specificity is consistent with the epidemiology of patients without HP; however, the epidemiology of patients with HP is similar to that of patients in European countries. With regard to the pattern of ANCA positivity, frequency of PR3-ANCA was equal to that of MPO-ANCA in patients with HP, although the predominance of MPO-ANCA has been demonstrated in the Japanese population with GPA.…”

Section: Discussionsupporting

confidence: 88%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

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Aim: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods:We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.Results: Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.Conclusion: AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.

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Section: Discussionsupporting

confidence: 88%

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

et al. 2017

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“…Three other studies did give rise to some concerns: the study from Lithuania (33) was hospital based and may have missed community cases, the diagnostic criteria used by Zeft et al (34) were not stated in their abstract, and the study from Japan (35) was based on referral to a renal unit and may have missed those with nonrenal disease. However, the denominator population in this case was clearly defined (35), and other sources (36) attest to the rarity of WG in this region. We extracted the number of cases of each disease entity, noting the location, year of publication, and the source population, e.g., general, adult-only.…”

Section: Methodsmentioning

confidence: 66%

“…Conceivably, the very low reported incidence of WG in Kuwait (30), Japan (35), and Lima, Peru (61) could be caused by differences in genetic susceptibility of the underlying populations. Nevertheless, a prevalence survey within a relatively genetically homogeneous population, the New Zealand European population, provides supportive evidence for a latitudinal gradient in WG that is not confounded by variation in ethnicity (8).…”

Section: Association Between Uv Radiation and Wg Mpa And Cssmentioning

confidence: 99%

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Gatenby

Lucas

Engelsen

et al. 2009

Arthritis & Rheumatism

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Objective. This ecological study describes and quantifies the association between ambient ultraviolet (UV) radiation levels, including daily winter vitamin D effective UV radiation levels and the incidence of the 3 antineutrophil cytoplasmic antibody-associated vasculitides (AAVs): Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS). Latitudinal variation in occurrence of the AAVs, especially WG, has been previously reported. For other autoimmune diseases such as multiple sclerosis and type 1 diabetes mellitus, inverse associations with latitude are hypothesized to indicate a causative role for low UV radiation exposure, possibly acting via vitamin D status. Methods. Published epidemiologic studies provided data on incident cases, total population of study regions, age-specific incidence rates, and study location. From these data and online age-specific population data, we calculated crude incidence rates, the expected number of cases (to control for possible age confounding), and measures of ambient UV radiation. Negative binomial regression models were used to calculate the incidence rate ratio (IRR) for a 1,000 joules/m 2 increase in ambient UV radiation.

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“…AAV is classified into four types according to clinical symptoms, clinical presentation, and laboratory findings: eosinophilic granulomatosis with polyangiitis (EGPA), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and polyarteritis nodosa (PAN) (Jennette et al 1994;Wiik 2002;Jennette et al 2013). Among these types of AAV, the incidence of MPA is higher in Japan than it is in the US and Europe (Fujimoto et al 2006(Fujimoto et al , 2011Shiraki et al 2007;Watts et al 2008). The effects of AAV are often restricted to small-and medium-sized vessels, resulting in kidney and lung pathologies in patients with MPA.…”

Section: Introductionmentioning

confidence: 99%

Remission Induction Therapy with Rituximab for Microscopic Polyangiitis: A Feasibility Study

Saito¹,

Takeuchi²,

Kagaya³

et al. 2017

Tohoku J. Exp. Med.

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is systemic vascular inflammation. Microscopic polyangiitis (MPA) is a major type of AAV in Japan. MPA often affects the kidneys and lungs, leading to death if untreated. Induction therapy (i.e., initial treatment) for MPA has not been optimized, although methylprednisolone and cyclophosphamide are commonly used. Recently, rituximab (RTX) (a monoclonal antibody against the protein CD20) has also been used to treat refractory AAV. RTX at 375 mg/m 2 /week for 4 weeks (i.e., the conventional lymphoma dosing schedule) is used, but the optimal dosing schedule is controversial. Indeed, a single-dose of RTX successfully controlled nephrotic syndrome. However, to date, the effectiveness of a single RTX dose in treating MPA has not been fully investigated in Japan. This was a retrospective observational study. Six newly diagnosed patients with MPA were initially treated with methylprednisolone and a single dose of RTX (375 mg/m 2 ). We investigated the patients' clinical features, as well as the efficacy and safety of RTX treatment. All patients attained remission on a tapered prednisolone dose of < 10 mg/day during the first 12 months. One patient relapsed after 12 months whereas another required hospitalization owing to infective spondyloarthritis. Adverse reactions to RTX infusion and late-onset neutropenia were not observed. Therefore, a single-dose treatment with RTX induced remission with few complications, and allowed tapering the prednisolone treatment. We conclude that a single dose of RTX is a promising induction therapy for MPA, reducing the cost associated with multiple doses.

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Incidence of ANCA-Associated Primary Renal Vasculitis in the Miyazaki Prefecture

Abstract: There were no WG or CSS patients. The incidence of PRV did not differ between Japan and Europe, but WG was not widespread in Japan. Furthermore, the ratio of serum MPO to PR3-ANCA among Japanese with PRV was much higher than that found among European and US patients.

Cited by 94 publications

References 30 publications

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Remission Induction Therapy with Rituximab for Microscopic Polyangiitis: A Feasibility Study

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