日本腎臓学会誌

A clinicopathological study was carried out using 55 autopsy cases of collagen disease. Incidence of vascular involvement of the kidney was the highest in polyarteritis nodosa (PN), and higher in Wegener's granulomatosis (WG), mucocutaneous lymph node syndrome (MCLS), allergic granulomatous angiitis (AGA), rheumatoid arthritis (RA) progressive systemic sclerosis (PSS), and systemic lupus erythematodes (SLE), than in dermatomyositis (DM). SLE showed more distinctive and more variegated glomerular lesions, while RA showed equal involvement of small arteries and glomeruli. Veins were damaged by angiitis in RA. Five cases of multiple glomerular microthromboses were noticed, and those were often related to rapidly progressive renal failure. From our previous observations on the fibrinolytic and fibrinolytic inhibitory activities of the kidney (Tanaka K: In. Soc. Path. Jap.1977, 66: 3-26), it is suggested that the kidney is easily affected by the formation of thrombi due to low fibrinolytic and high fibrinolytic inhibitory activities in the cortex, and that such glomerular microthromboses deteriorate renal lesions due to angiitis. When renal functions rapidly deteriorate in patients with systemic vasculitis, a possibility of disseminated intravascular coagulation syndrome (DIC) should be considered.

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Renal function studies in cystic renal disease. Renal function studies were carried out in normal control subjects (n=6), patients with adult type polycystic kidney diseas (PCK) (n=11), patients with medullary sponge kidney (MSK) (n=11), and patients with bilateral renal stones (BRS) (n=10). The patients with MSK and BRS had normal glomerular filtration rate (GFR), whereas variably decreased GFR was noted in PCK. This decrease in GFR of PCK was closely related to the decrease in renal plasma flow (RPF). Regardless of the GFR, impaired urine concentrating ability was observed in PCK, MSK, and BRS. The maximum urinary osmolality in PCK declined further as GFR was reduced. The capacity to reabsorb chloride along the distal nephron segment was reduced in PCK, mostly due to the reduction in functioning renal mass. Impaired urine acidification but normal urinary acid excretion were observed in MSK, while both urine acidification and urinary acid excretion were impaired in PCK. Although arterial pH was within the normal range in MSK, bicarbonate level was significantly lower than the control value. Since no bicarbonate wastage in urine was noted, incomplete renal tubular acidosis of the distal type was discerned in MSK. On the other hand, more than one pattern of urine acidification was identified following acute acid challenge in PCK. Uremic renal acidosis developed when GFR fell below 50 ml/min in PCK.

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