PMC3134061_01
Clinical course during hospitalization. PSL = Prednisolone; M-PSL = methylprednisolone; CPA = cyclophosphamide; MZR = mizoribine. Parameters indicate total protein ( ) and albumin ( ). PSL (50 mg/day) was administered after the diagnosis of PLGE. However, hypoproteinemia was aggravated with total serum protein at 2.8 g/dl and albumin at 1.0 g/dl (point A). Although steroid pulse therapy was partially effective, it did not adequately improve symptoms (point B). The patient's symptoms improved with the use of CPA pulse therapy, and consequently, oral administration of MZR followed the steroid pulse therapy.
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F1
After admission, the patient was concomitantly treated with intravenous albumin infusion and diuretic injection (fig. 1), and further examinations were performed to detect the cause of her symptoms. The diagnosis of PLGE complicated by SS was made as described above and 50 mg/day prednisolone (PSL) was administered (fig. 1).
99mTc-HSA scintigraphy. Accumulated radioactivity was detected in areas of the stomach (arrows) 60 min after the intravenous injection of 99mTc-HSA.
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F2
Although the mucosal surface appeared normal, lower GI endoscopy revealed mild edema and congestion of the entire colon; however, no tumor formation or erosive ulcerative lesion was observed. 99mTc-human serum albumin (99mTc-HSA) scintigraphy revealed early radioisotope accumulation in the stomach (fig. 2) and a1-antitrypsin (a1-AT) clearance was high at 205 ml/day (normal <13 ml/day), and thus a diagnosis of PLGE was made.