Frequency Due to Bladder Amyloidosis in Patients with Rheumatoid Arthritis: Two Cases Reports / 대한류마티스학회지

Secondary amyloidosis is characterized by accumulation of an amorphous proteinous material in the various tissue and organs with infectious disease or inflammatory disease. Symptoms of the amyloidosis are variable according to the involved organs. Reactive amyloidosis of bladder in rheumatoid arthritis (RA) is a rare condition with hematuria in the most cases. However, we report two cases of patients with RA, who have frequency due to secondary amyloidosis of bladder without hematuria. Therefore secondary amyloidosis of urinary bladder should be considered as a possible cause of frequency in patients with long-term RA.

Clinical characteristics of rapidly progressive glomerulonephritis / 대한내과학회지

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is microscopically characterized by formation of crescents in more than 50% of glomeruli observed. The patients usually move on rapidly to renal failure and the prognosis is not favorable. But there was only a few study because of the rarity in incidence. METHODS: We reviewed and analyzed the records of 15 patients diagnosed as crescentic glomerulonephritis (CrGN) by renal biopsy from March 1990 to December 2003. RESULTS: Fifteen out of 1055 biopsy cases were CrGN including 6 (40%) of pauci-immune glomerulonephritis (PIGN) and 9 (60%) of immune complex glomerulonephritis (ICGN). Underlying diseases of PIGN were: unknown 2, Wegener's granulomatosis 2, focal segmental glomerulosclerosis 1, and rectal cancer 1. For ICGN were: IgA nephropathy 3, lupus nephritis class IV 3, Henoch-Schonlein purpura 2, and HBV-associtated membranoproliferative glomerulonephritis type I. The incidence of major manifestation in PIGN vs. ICGN was respectively: hypertension 50% vs. 22.2%, nephrotic syndrome 50% vs. 88.9%, percents of crescents 73.9% vs. 57.3%. The levels of BUN (mg/dL) and serum creatinine (mg/dL) were higher in PIGN as 76.8 +/- 14.3 and 6.6 +/- 1.2 vs. 26.9 +/- 8.9 and 1.6 +/- 0.3 in ICGN. With methylprednisolone pulse, 5 out of 7 patients showed some improvement in their renal function. A case of Wegener's granulomatosis taken oral prednisolone and another case of lupus nephritis given cyclophosphamide pulse also had relatively favorable course. At the end of follow-up, the more crescents they had the higher creatinine level (r=0.711, p<0.01). CONCLUSION: RPGN manifested nephrotic syndrome commonly and many of them progressed to the chronic kidney disease or even developed end stage renal disease. But appropriate immunosuppre- ssive treatment could help to preserve renal function. When considering the proportion of crescentic glomeruli, it was related to the worse prognosis. It is necessary to make an effort to diagnose early and treat vigorously.

Clinical courses of the adult patients with idiopathic membranous nephropathy / 대한내과학회지

BACKGROUND: Idiopathic membranous nephropathy (IMN) causes variable clinical courses, such as from asymptomatic urinary abnormalities, nephrotic syndrome to end-stage renal failure. We evaluated clinical findings and effects of steroid and steroid with chlorambucil in patients with IMN. METHODS: We reviewed 37 cases of biopsy-proven patents of IMN whose follow-up duration was at least 2 years, retrospectively. The mean follow-up duration of the cases was 74 +/- 49 months. In the cases of steroid therpy, prednisolone 40, 50 or 60 mg/day was given for maximal 16 weeks. Steroid-chlorambucil treatment was done for the cases of no response or relapse after steroid therapy, severe nephrotic syndrome or elevated serum creatinine more than 1.2 mg/dL. We did 3 cycles of treatment. Every cycle was consisted of intravenous 1 gram of methylprednisolone for 3 days followed by prednisolone 0.5 mg/kg/day orally for 27 days then chlorambucil 0.2 mg/kg/day for 28 days. Therapeutic results were evaluated. RESULTS: The mean age was 41 +/- 15 years and 5-6th decade was 48.6%. Male to female ratio was 1.3 : 1. Nephrotic syndrome was in 86.5% in the cases. The results of prednisolone therapy was done in 25 cases were 20% of complete remission (CR), 28% of patial remission (PR) and 52% of no respone (NR). There was no difference between the response rate and dosage of prednisolone. Steroid-chlorambucil therapy was done in 18 cases totally, 5 cases in the first treatment and 13 cases of no response or relapsed cases after prednisolone treatment. The results were 22.2% of CR, 50% of PR and 27.8% of NR. This results were no difference between steroid and chlorambucil combined therapy. Spontaneous remission was observed 35.1% of the total cases, 21.6% of spontaneous CR and 13.5% of spontaneous PR after the final observations. The final results were 16.2% of CR and 10.8% of PR after prednisolone or chlorambucil combined therapy. Progressive renal disease were developed in 6 cases (16.2%) and the mean renal surval time measured by projected reciprocal creatinine from diagnosis to the point of 0.1 was 129 +/- 79 months. CONCLUSION: Nephrotic syndrome was presented in 86.5% of cases and with high remission rates such as 37.8% of complete and 24.3% of partial remission in IMN. Progressive renal failure was occurred in 16.2% of cases and most of the cases progressed very slowly. There was no different results between steroid alone and chlorambucil combined therapy.