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.

Abstract

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.

Related Concept Videos

Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists01:23

Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists

Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Peripheral Artery Disease III: Interprofessional Care01:27

Peripheral Artery Disease III: Interprofessional Care

Peripheral Artery Disease (PAD) is characterized by narrowed arteries that diminish blood flow to the extremities. Effective management of PAD requires an interprofessional approach involving various healthcare professionals. The critical aspects of interprofessional care for PAD patients focus on risk factor modification, drug therapy, exercise therapy, nutrition therapy, critical limb ischemia care, and interventional radiology and surgical procedures.The primary treatment goal for PAD...
Myocarditis III: Medical Management01:14

Myocarditis III: Medical Management

Myocarditis: Comprehensive Medical ManagementMyocarditis, the heart muscle inflammation, requires a comprehensive medical management strategy that addresses the underlying cause, provides supportive care, manages symptoms, and reduces cardiac workload.Infections and Autoimmune CausesAdminister appropriate antimicrobial therapy when an infectious agent causes myocarditis. For instance, penicillin treats infections caused by Group A Streptococcus. In cases where autoimmune processes are...
Acute Pyelonephritis II: Diagnostic Studies and Management01:28

Acute Pyelonephritis II: Diagnostic Studies and Management

Introduction:For diagnosing acute pyelonephritis, a comprehensive patient history is collected to identify symptoms such as dysuria, frequent or urgent urination, flank pain, or costovertebral angle (CVA) tenderness that may suggest a kidney infection.Physical ExaminationDuring the physical examination, CVA tenderness is assessed. This involves gentle percussion over the costovertebral angle, where tenderness often indicates a kidney infection.Diagnostic TestsUrinalysis: Used to identify white...