To clarify the importance of the duration from the onset of a urinary abnormality until a biopsy is actually performed (UA-Bx time) in making a renal prognosis, we investigated 496 patients with IgA nephropathy (male/female : 222/274, mean age : 33.0±13.7 yrs, mean follow-up period : 10.8±4.3 yrs). All patients were found to have a urinary abnormality, including both hematuria and proteinuria, at clinical onset while demonstrating a normal renal function, and showing a serum creatinine level of ≤ 1.2 mg/dl or a creatinine clearance level of ≥ 80 ml/min. The UA-Bx time was divided into 3 groups : < 1 yrs (S-G), 1 ≤ < 3 yrs (M-G), ≥ 3 yrs (L-G). The severity of glomerular damage was divided into 5 groups based on the occupational rate of segmental sclerotic glomeruli. Based on a multivariate analysis of independent prognostic factors relating to renal death, the severity of glomerular damage was the most independent factor, while the UA-Bx time showed no risk for renal death. However, based on a multivariate analysis of the UA-Bx time regarding the timing of a renal biopsy, patients in L-G, which had the most glomerular damage, showed twice the hazard ratio as those in S-G or M-G and the difference was significant. These results thus indicate that because the glomerular damage is able to progress for 3 yrs or longer after the clinical onset of renal disease, a renal biopsy should therefore be performed within 3 yrs from the clinical onset in patients demonstrating both hematuria and proteinuria when such patients are also suspected of having IgA nephropathy.

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A 55-year old female with rheumatoid arthritis (RA) had presented with proteinuria since She was referred to us for persistent edema on her face and legs in On admission, her 24-hour urinary protein excretion was 4.4 g/day, total serum protein level was 5.3 g/dl, and serum level of amyloid A protein (SAA) was elevated to 45.8 mg/ml. A percutaneous renal biopsy was performed, and light microscopy revealed varying degrees of amyloid deposits in the mesangial areas and arteriolar walls. The diagnosis of secondary amyloidosis (AA amyloidosis) was based on immunohistochemical staining for amyloid A protein using monoclonal antibody against SAA. Four weeks after treatment with salazosulfapyridine(SASP) and dipyridamole, proteinuria began to decrease and the edema had disappeared. Finally she recovered from nephrotic syndrome. AA amyloidosis has been thought to have a poor prognosis, with progression to renal failure. Since there is no specific effective therapy for the disease, it is very important to reduce the activity of the underlying cause. In our patient with renal amyloidosis following RA, SASP was evidently effective for arthritis and improvement of renal function. SASP might have a beneficial effect on AA amyloidosis by suppressing inflammatory cytokines.

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A 54-year-old man, who had been diagnosed as having MPO-ANCA-related glomerulonephritis in developed severe anemia and was admitted to our hospital on Endoscopic examination of the upper gastrointestinal tract revealed melena due to duodenal ulcer (Dieulafoy type) . The level of ANCA titer was elevated considerably (640 EU), but otherwise there was no evidence of systemic vasculitis activation such as fever, arthralgia, skin eruption, renal insufficiency, and rise in C reactive protein. A renal biopsy showed neither crescentic formation nor necrosis of glomerulus. Subsequently he developed hematochezia and renal dysfunction rapidly progressed thereafter. Angiographical examination of superior mesenteric artery revealed that the bleeding was responsible for the lesion of the small intestine, probably the ileum. In spite of TAE (transarterial embolization) he had recurrence of severe hematochezia three days later. Partial ileotomy was perfomed and progression of the anemia was stopped. Multiple ulcer was found in the resected ileum. The small arteries in the submucosa at the ulceration showed fibrinoidnecrosis of the vessel walls. These findings suggested that ANCA-related vasculitis had relapsed. The patient received methylprednisolone pulse therapy, followed by oral administration of prednisolone after the operation. Both serum levels of creatinine and MPO-ANCA gradually decreased after the initiation of treatment. However, 24 days later, he suddenly manifested severe abdominal pain, and was diagnosed as having perforation of the stomach or duodenum. Due to supportive therapy and reduction of the steroid dose, peritonitis subsided, but symptoms caused by systemic vasculitis developed. Later raised the dose of steroid suppressed the activity of systemic vasculitis. In this case, elevation of the ANCA titer demonstrated recurrence of MPO-ANCA-related vasculitis as gastrointestinal bleeding.

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