IgG4-related kidney disease / 国际儿科学杂志

IgG4-related disease is a newly recognized systematic inflammatory and fibrotic disease which affects one or more organs.This disease mainly occurs in the middle-aged and old males.Currently only a few cases were reported in children.Renal involvement was referred to as IgG4-related kidney disease,including IgG4-related tubulointerstitiai nephrisis,membraneous nephropathy and renal pyelitis.The clinical features,laboratory tests,pathological findings and treatment progresses have been summarized in this review.

A case presentation of renal involvement in MCTD / 대한내과학회지

MCTD (Mixed Connective Tissue Disease) is a rare disease characterized by clinical characteristics of patients with overlapping features of SLE (systemic lupus erythematosus), SS (systemic sclerosis), and PM (polymyositis), and serologically characterized by high titers of Anti U1 RNP Ab. At early stage, finger swelling or Raynaud's phenomenon with high titers of Anti U1 RNP Ab are only apparent. Proteinuria and membranous nephropathy are characteristic renal manifestation of MCTD. Recently we had the opportunity to observe patient with Raynaud's phenomenon, finger swelling, high titers of Anti U1 RNP Ab, and asymptomatic proteinuria who underwent a renal biopsy. The patient was diagnosed as early MCTD and renal histology revealed membranous nephropathy. Our purpose is to report this patient and to review the literature.

Recombinant Alfa-interferon Therapy in HBGN with HBe Antigenemia / 대한신장학회잡지

Recently the results of alpha-interferon treatement in hepatitis B virus associated glomerulonephritis showed a reduction of proteinuria and a loss of HBeAg in some treated patients. But, alpha- interferon therapy was mainly tried in membranous nephropathy of children. So, we treated 13 adults patients with recombinant alpha-interferon who were diagnosed as HBV associated membranous nephropathy(2) and membranoproliferative GN(11) at Seoul National unversity hospital. All of them had nephrotic range proteinuria and HBe antigenemia for more than 6 months, normal serum creatinine level and had no other systemic disease. Three million units of recombinant alpha-interferon was given six times a week for 16 weeks intramuscularly and the therapeutic effect was analyzed during treatment periods, especially in terms of changes in urine protein excretion and serum HBeAg. And we compared them with 14 control patients who had received conservative therapy only. As a results, at the end of interferon therapy, serum HBeAg disappeared in 4 of 13 treated patients, and serum HBsAg disappeared in 1 of 4. At the end of therapy, proteinuria diminished to non-nephrotuc range in 6 of 13 treated patients and decrement of proteinuria was accompanied with disappearance of serum HBeAg in 3 patients. And proteinuria diminished in 5 of 11 MPGN patients and serum HBeAg disappread in 3 of them. But in 14 controls there were no significant changes in 24 hour urine protein excretion and serum HBeAg. During interferon therapy, mild febrile reaction was developed in 8 patients, anemia in 3 patients, and cytopenia in 7 patients, but most of these adverse effects resolved spontaneously after discontinuation of interferon therapy. During follow up periods over 1 years, proteinuria relapsed to nephrotic range in 3 of 6 patients and serum HBeAg reappreared in 2 of them. In conclusion, the alpha-interferon at the dose induced a clearance of HBeAg and the decrement of the proteinuria in some adult MN and MPGN patients. And these results suggested the possibilities that HBeAg might be involved in the pathogenesis of HBV associated MPGN and alpha-interferon might be effective in some HBV associated MPGN.