The significance of necrosis (karyorrhexis), among the most characteristic findings in lupus nephritis, was evaluated by studying the correlation between the existence of necrosis in renal biopsy specimens and laboratory findings. The subjects were 54 patients with diffuse proliferative lupus nephritis and 6 patients with focal proliferative lupus nephritis selected from 143 patients with lupus nephritis. We also compared the clinical course of oral prednisolone and intravenous methylprednisolone pulse therapies after steroid administration. Compared with the non-necrosis group, the necrosis group had significantly lower CH50 levels and more proteinuria. Patients with necrosis were effectively treated with repeated pulse therapy judging by immunological activity and the decrease in proteinuria at an early stage, but responded poorly to oral steroid therapy. As the presence of necrosis in cases of lupus nephritis means high immunological activity of the lesion and there is responsiveness to a large dose of steroids, extensive immunosuppressive therapy including methylprednisolone pulse therapy should be applied to these patients.
(Internal Medicine 33: 461-465, 1994)

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The in vitro effect of one traditional Chinese herbal medicine (Japanese name: "Keishi-bukuryo-gan"), which has been empirically used in scleroderma patients in China and Japan, on collagen production in tibroblast cultures was studied. Fibroblasts from 3 scleroderma patients and 2 normal controls were incubated with various concentrations of "Keishi-bukuryo-gan" and collagen production was then determined by a radiochemical method. "Keishi-bukuryo-gan" significantly and selectively inhibited collagen synthesis in a dose-dependent manner, with a tendency of a stronger effect on scleroderma fibroblasts than control cells. The results may explain the clinical usefulness of this medicine, and it may become a promising new agent for the treatment of scleroderma.
(Internal Medicine 33: 466-471, 1994)

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We studied a patient with hyperglycemia who developed choreic involuntary movements in the right extremities using single photon emission computed tomography (SPECT) with ¹²³I-N-isopropyl-p-iodoamphetamine. SPECT revealed an increased blood flow in the left striatum and thalamus. Through the control of blood glucose and the administration of haloperidol, the hemichorea was resolved, and the increased blood flow in the striatum and thalamus disappeared. These findings suggest that the increased blood flow, which probably indicates increased neuron activity in the striatum and thalamus, is an underlying pathophysiological state in hemichorea.
(Internal Medicine 33: 472-475, 1994)

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A 35-year-old man developed a cerebral infarction and experienced transient ischemic attacks originating from the vertebrobasilar artery, as well as locked-in syndrome. He recovered with minimal neurological deficit. On cerebral angiography, the orientation of the right vertebral artery was markedly abnormal as it entered the foramina of the transverse process at the level of the third cervical vertebra. We concluded that the abnormal orientation of the vertebral artery caused the thrombosis and that the transient ischemic attacks, locked-in syndrome and cerebral infarction were brought about by thromboemboli originating in this artery.
(Internal Medicine 33: 476-480, 1994)

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A 39-year-old housewife who underwent intramammary injections of a proprietary silicone fluid mixture showed clinical and novel transbronchial lung biopsy (TBLB) findings. She presented with complaints of progressive dyspnea, dry cough, and pleuritic chest pain 2 days after the last silicone injections. The chest X-ray and CT scan showed diffuse interstitial infiltrates. TBLB demonstrated translucent, presumably silicone globules embolized within the pulmonary capillaries. The documentation of intramammary injections, the clinical and radiographic features of acute pneumonitis, and the histopathologic evidence by TBLB, may support the causal relationship between illicit injections and the silicone embolism. We discuss the pathogenesis and urge that this potentially toxic source of pulmonary embolism be removed.
(Internal Medicine 33: 481-483, 1994)

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A female patient with a history of migraines and chorea developed polyarthralgia at age 24 and was diagnosed with rheumatoid arthritis. In 1991 she was hospitalized because of impaired renal function and hypertension. Examination revealed thrombocytopenia and the presence of lupus anticoagulant. Antinuclear antibody was weakly positive, but anti-DNA antibody was negative, and no decrease in leukocyte count or complement level was observed. Rheumatoid arthritis with antiphospholipid syndrome was diagnosed. Renal biopsy showed renal thrombotic microangiopathy. This renal lesion was considered to be associated with antiphospholipid syndrome. Cyclophosphamide pulse therapy and anticoagulation therapy decreased proteinuria and improved renal function.
(Internal Medicine 33: 484-487, 1994)

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A 69-year-old female was admitted to our hospital because of leg edema, proteinuria (2.1 g/day), and gross hematuria. She had non-alcoholic liver cirrhosis of unknown etiology. Esophageal varices also were found. Examination of the renal biopsy specimen revealed mesangial proliferative glomerulonephritis with IgA deposits. Propranolol was administered orally to reduce portal hypertension, resulting in a progressive decrease in urinary microalbumin excretion. This case suggests that portal hypertension is involved in the pathogenesis of IgA nephropathy in liver cirrhosis.
(Internal Medicine 33: 488-491, 1994)

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A male patient suffered chronic renal failure due to lupus nephritis and was undergoing hemodialysis. Six years after beginning hemodialysis, anemia developed, which improved by erythropoietin. Unresponsiveness to erythropoietin gradually appeared, and with a suspicion of pure red cell aplasia, he was treated with a high-dose corticosteroid but the unresponsiveness did not improve. Neither his serum nor lymphocytes inhibited erythropoiesis of either normal bone marrow stem cells or his own in vitro. These observations suggest an impaired hematopoietic microenvironment in his bone marrow.
(Internal Medicine 33: 492-495, 1994)

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A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.
(Internal Medicine 33: 496-500, 1994)

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A 76-year-old man with small cell lung cancer associated with the syndrome of inappropriate secretion of ADH (SIADH) visited our hospital. The serum Na level was normal on the first visit, but 2 weeks later it decreased to 114 mEq/L with an extremely high plasma vasopressin (VP) level of 1520 pg/ml. Serum Na was normalized after the reduction of the tumor size by chemotherapy, but the plasma VP level remained between 150 to 600 pg/ml. On gel filtration of plasma VP two peaks of immunoreactive VP were eluted at the positions of a larger molecule than authentic VP and authentic VP, and VP in urine gave only one peak compared to that of authentic VP. The dilution curve of plasma VP was almost parallel and that of urine was completely parallel to the standard curve. These findings suggest that a larger VP with low physiological activity was predominantly secreted in the present patient and manifested relatively mild symptoms despite the extremely high plasma VP level.
(Internal Medicine 33: 501-504, 1994)

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Concomitance of idiopathic hypouricemia and Sjögren's syndrome is reported. A 37-year-old Japanese woman with Sjögren's syndrome and her 39-year-old sister without this syndrome both had extremely low levels of serum uric acid. Markedly increased urinary excretion of uric acid and poor response to the pyrazinamide suppression test revealed that the hypouricemia in these sisters was caused by a defect in the pre-secretory reabsorption of uric acid. It is categorized as idiopathic renal hypouricemia than hypouricemia rather secondary to Sjögren's syndrome. Thus, idiopathic renal hypouricemia should be considered even in cases with autoimmune diseases.
(Internal Medicine 33: 505-507, 1994)

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A 75-year-old man was diagnosed with alcaptonuria by direct identification of homogentisic acid in the urine using gas chromatography-mass spectrometry. Complications included valvular heart disease with marked calcification detected by two-dimensional color-Doppler echocardiography and recurrent bacterial infection. Immunological analyses revealed a reduced number of T cells with compensatory expansion of CD56⁺, CD57⁺ natural killer (NK) cell population and impaired functions of cellular immunity such as phytohemagglutinin response, antibody-dependent cellular cytotoxicity, NK activity and interleukin-2 production. Humoral immunity and neutrophil functions were considered to be normal. This is the first reported case of alcaptonuria to our knowledge in which immunological abnormality was documented.
(Internal Medicine 33: 512-516, 1994)

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