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Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan
Koichi Sugiyama1, Ken-ei Sada, Michiko Kurosawa
1Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
Background:
This study aimed to describe the epidemiologic characteristics of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in Japan.
Methods:
We used the database of the Ministry of Health, Labour and Welfare (MHLW) from 2006 to 2008, and analyzed data from 938 patients (MPA = 697, GPA = 241) who fulfilled the MHLW diagnostic criteria and had registered within a year after onset.
Results:
The mean ages of the MPA and GPA patients were 69.4 ± 0.4 and 58.4 ± 1.1 years, respectively. Renal (86.9 %), chest (73.7 %), and nervous system (45.2 %) symptoms were common in MPA patients. Ear, nose, and throat (86.7 %), chest (78.0 %), and renal (60.6 %) symptoms were frequently observed in GPA patients. The concomitant use of cyclophosphamide (CY) with corticosteroids was observed in 22.2 % of the MPA patients and 58.5 % of the GPA patients. In multivariate analysis, the concomitant use of CY was associated with a younger age and pulmonary hemorrhage in MPA patients, and the avoidance of CY was associated with nervous system symptoms and rapidly progressive glomerulonephritis in GPA patients. Plasma exchanges were inducted in 5.2 % of the MPA patients and 4.1 % of the GPA patients. The addition of plasma exchange was associated with elevation of the serum creatinine level in patients with both MPA and GPA.
Conclusion:
A dominance of MPA and a reduced frequency of renal involvement in GPA patients may be significant features of the Japanese population. Clinical practice relating to MPA and GPA in Japan can be characterized as follows: CY is used less commonly, and plasma exchange is employed for patients with deteriorated renal function.
Insights
This study describes the epidemiology of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in Japan. Findings highlight distinct features in the Japanese population, including less common cyclophosphamide (CY) use and plasma exchange for renal dysfunction.
Area of Science:
- Rheumatology
- Epidemiology
- Vasculitis
Background:
- Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are rare systemic vasculitides.
- Understanding their epidemiologic characteristics is crucial for public health and clinical management.
Purpose of the Study:
- To describe the epidemiologic characteristics of MPA and GPA in Japan.
- To identify specific features of these diseases within the Japanese population.
Main Methods:
- Utilized the Ministry of Health, Labour and Welfare (MHLW) database from 2006-2008.
- Analyzed data from 938 patients diagnosed with MPA (n=697) or GPA (n=241) meeting MHLW criteria.
Main Results:
- MPA patients were older (mean age 69.4 years) than GPA patients (mean age 58.4 years).
- Common symptoms included renal and chest involvement in MPA, and ENT, chest, and renal involvement in GPA.
- Cyclophosphamide (CY) use was less frequent in MPA (22.2%) than GPA (58.5%), associated with specific clinical features in each disease.
Conclusions:
- MPA dominance and reduced renal involvement in GPA are notable in Japan.
- Japanese clinical practice shows less common CY use and increased plasma exchange for renal impairment in MPA and GPA.
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