In most patients with IgG4-related kidney disease (IgG4-RKD), corticosteroid therapy leads to a rapid improvement of renal function, radiology findings, and serological abnormalities within 1 month. Consequently, corticosteroids are often considered the first-line therapy for IgG4-RKD. However, in patients with advanced renal damage before therapy, renal function does not recover completely, and therefore early diagnosis and treatment are essential. In corticosteroid monotherapy for IgG4-RKD, a moderate dose is sufficient for induction, and recovery of renal function can be maintained for a long period on low-dose maintenance in patients for whom induction corticosteroid therapy has been successful. Unfortunately, disease relapse can occur even in patients receiving maintenance therapy.
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This work was supported in part by grants from Health and Labour Sciences Research Grants for the Study of Intractable Disease from Ministry of Health, Labor and Welfare, Japan and ‘IgG4-related Kidney Disease’ working group of the Japanese Society of Nephrology.
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