Life-style activities in systemic lupus erythematosus.

OBJECTIVE: We evaluated the life-style activities of outpatients with SLE and factors that reduce their social activities. SUBJECTS: SLE group = 60 patients, Control 1 = 30 healthy subjects and Control 2 = 30 patients with other autoimmune diseases. The Frenchay Activity Index (FAI), Zung's self-rating depression scale (SDS), and the Japanese version of the Philadelphia Geriatric Center morale scale-revised (MS) were compared between groups. Relation between FAI and age, disease duration, steroid dose, SDS, and MS were examined in the SLE group, Control 1, and Control 2. RESULTS: Total scores by FAI was 28.1 +/-8.0 points in Control 1, whereas it was 26.5 +/- 5.8 points in Control 2 and 24.5 +/- 7.7 points in the SLE group. While there was no statistical difference between the SLE group and Control 2, the scores were significantly lower in the SLE group than in Control 1 (P < 0.05). In SLE patients, age, the duration of the disease, and the steroid dose had no correlation, but MS had a positive correlation (P < 0.05) and SDS had a negative correlation (P < 0.05). In Control 2, age, the duration of the disease, the steroid dose, MS and SDS had no correlation whereas there was significant negative relation between FAI and SDS in Control 1 (r= -0.516, P<0.005). CONCLUSION: The significant relation between life-style activities and subjective well-being, and depression in SLE suggests that detection and treatment of mental status is important in improving the life-style activities of SLE patients.

A case of systemic lupus erythematosus with giant hepatic cavernous hemangioma.

A 39-year old woman with antiphospholipid antibody positive systemic lupus erythematosus (SLE) was admitted to our department because of high fever, liver dysfunction and high level of C-reactive protein. At hospitalization, there was no anemia or jaundice. A tumor was palpable in the epigastric region, and there was tenderness in this region, but no muscular defense. There were no findings which indicated disease activity of SLE. The result of abdominal ultrasonography showed that there was a giant tumor, which occupied the majority of the left lobule of the liver, and a nonuniform ultrasound image was observed inside the tumor. The result of dynamic computed tomography (CT) showed peripheral globular enhancement, and enhancement then extended to the tumor center with time. Consequently, the patient was diagnosed with multiple hepatic hemangiomas. After admission, anemia rapidly deteriorated, and platelet count tended to decline. Therefore, intratumor hemorrhage was suspected, and emergent angiography was performed. For hemostatic purposes, transcatheter arterial embolization (TAE) and extended left hepatic lobectomy were performed. In patients with autoimmune diseases such as SLE and antiphospholipid syndromes, when thrombocytopenia is observed, care should be paid to identifying its cause, considering thrombocytopenia may be induced by hemangioma, although these cases are extremely rare.

Massive gastrointestinal hemorrhage in a case of amyloidosis secondary to rheumatoid arthritis.

A case of a 60-year-old woman with secondary gastrointestinal amyloidosis to rheumatoid arthritis is reported. Biopsy findings in the mucosa of the stomach and lower gastrointestinal tract revealed amyloidosis. Endoscopic examination of the lower gastrointestinal tract revealed multiple nodular elevations. The patient showed massive melena. Emergency angiography was performed and an extravasation was found at branches of the jejunal artery. Embolization was performed and this lead to a good prognosis. Patients with massive hemorrhages following gastrointestinal amyloidosis generally have a poorer prognosis. Embolotherapy performed for the present case might represent an effective therapeutic method for gastrointestinal hemorrhage in gastrointestinal amyloidosis.

Successful pregnancy and delivery in a case of systemic lupus erythematosus treated with immunoadsorption therapy and cyclosporin A.

A 32-year-old woman diagnosed as systemic lupus erythematosus (SLE) became pregnant. During pregnancy she was treated with a daily dosage of prednisolone 15 mg. However, because the exanthema became worse, she was hospitalized on January 14, 1997 in order to receive immunoadsorption therapy. Before delivery we implemented the immunoadsorption therapy twice and cyclosporin A (CsA) was administered simultaneously. She gave birth in her 37th week. The baby weighed 2260 g at the time of delivery and had no deformities. The mother also had no side effects. The success of pregnancy and childbirth in our case, without any side effects, shows the possibility that the combination of CsA and immunoadsorption therapy may be considered safe to control a pregnancy complicated by SLE.

[Subjective happiness and psychological condition in SLE].

We reported the relationship between subjective happiness and psychological condition to investigate which factor effects the quality of life (QOL) in 18 patients with systemic lupus erythematosus (SLE) and compared them with age, sex and dose of steroid matched 18 patients with chronic glomerular nephritis (CGN). The patients were evaluated in morale scale-revised (Japanese version) which is a standard method of evaluating subjective happiness in relation to QOL. State-trait anxiety inventory (STAI) of Spielberger was used for evaluation of anxiety condition with self rating depression scale (SDS) of Zung in evaluation of depressive state. We examined the relationship between morale scale and SDS and morale scale and STAI. The relationship between morale scale and age; period of the disease, dose of steroid and activity were also investigated. There were no significant differences between the groups on morale scale, SDS and STAI and there were no significant correlations between morale scale and age; period of the disease and the dose of steroid in the both groups. There were correlations between morale scale and state and trait anxiety in the group with SLE and morale scale and trait anxiety in the group with CGN. We concluded that anxiety decreased the QOL in patients with SLE and CGN.

[Relationship between higher cortical dysfunction and the findings of magnetic resonance imaging in systemic lupus erythematosus].

The relationship between systemic lupus erythematosus (SLE) and organic lesions was investigated by magnetic resonance imaging (MRI) to clarify the etiology of higher cortical dysfunction in SLE. The subjects were 10 patients with SLE, and higher cortical dysfunction was observed in 8 (80%) of the 10 patients. 5 (82.5%) of the 8 patients showed abnormal MRI findings. The findings of higher cortical dysfunction were consistent with the MRI findings in 1 of the 5 patients, but not in the remaining four. MRI revealed no lesion despite the presence of higher cortical dysfunction in three patients. These results suggest that the association of organic changes and functional changes in cerebral nerve cells is important in etiology of higher cortical dysfunction in SLE.

[A case of amnesic syndrome caused by hematoma in the left anterior medial thalamus].

We reported a case of amnestic syndrome caused by a hematoma in the left thalamus. The case was that of a 68-year-old, right-handed man who suddenly showed amnestic syndrome. He had neither motor paresis nor sensory disturbance. Clinical examination showed he had disorientation, anterograde amnesia and mild retrograde amnesia. Immediate recall and remote memory were intact, but recent memory was severely impaired. CT scan revealed a high density area in the anterior medial part of the left thalamus. We concluded that amnesia in this patient was caused by fasciculus mamillothalamicus damage because of a hematoma during thalamic hemorrhage.

[A case of malignant rheumatoid arthritis with lupus anticoagulant and cerebral infarction].

We reported a case of malignant rheumatoid arthritis (MRA) with cerebral infarction associated with a possible cause of lupus anticoagulant. The patient was a 68-year-old woman who had received treatment for rheumatoid arthritis (RA) from 15 to 16 years ago. She consulted to our hospital with a major complaint of right hemiplegia. Brain CT revealed a low density area in the left hemisphere. She was diagnosed as cerebral infarction and hospitalized. Since she was noted to have hypocomplementemia, interstitial pneumonia and pericarditis, she was diagnosed as MRA. Coagulation test disclosed positive lupus anticoagulant (LA). Generally, CNS disorders in MRA are uncommon. Cerebral infarction was complicated in the present case, suggesting the involvement of antiphospholipid antibodies as its pathogenesis.

[Cortical atrophy and higher cortical dysfunction in systemic lupus erythematosus].

The relationships between cortical atrophy and higher cortical dysfunction in systemic lupus erythematosus (SLE) were investigated. The subjects were 27 females, right-handed patients with SLE. None of them had a history of cerebro-vascular disorders. The CT index of cortical atrophy included Huckman number, Evans' ratio, Ventricular index, and Cella-media index calculated from the brain CT. As controls, 27 age- and sex- matched, healthy subjects were studied and compared with the SLE group. All underwent the following neuropsychological examinations: intelligence and mental state, linguistic function, and recognition and praxis. As a result, Huckman number and Evans' ratio were significantly higher in the SLE group than in controls, clearly showing evidence of cortical atrophy. With regard to the relationships between cortical atrophy and neuropsychological tests, there were significant relationships between digit span and Huckman number and Evans' ratio, and between word fluency and Evans' ratio. These findings suggested that the atrophy of frontal lobe was characteristic in SLE, being associated with dysfunction of frontal lobe.

[Antiphospholipid antibodies and regional cerebral blood flow in systemic lupus erythematosus].

We investigated the relationship between antiphospholipid antibodies (APA) and regional cerebral blood flow (rCBF) on 25 patients with systemic lupus erythematosus (SLE). Lupus anticoagulant (LA) and anticardiolipin antibody-IgG (ACA-IgG) as a marker of APA were determined. Brain CT and single photon emission CT (SPECT) were performed to evaluate the rCBF. LA was positive in 8 cases (32%), and ACA-IgG was positive in 3 cases (12%), respectively. Brain CT revealed brain atrophy in 8 cases (32%), but none of them had localized lesions. SPECT disclosed a reduced rCBF in 11 cases (44%). A reduced rCBF was noted to have a significant relationship with LA (p < 0.005), but not with ACA-IgG. It was suggested that LA might be an important factor as the cause of rCBF disturbances detected by SPECT in SLE patients.

Higher cortical dysfunction, antiphospholipid antibodies and neuroradiological examinations in systemic lupus erythematosus.

We performed neuropsychological tests to investigate higher cortical dysfunction in 21 patients with systemic lupus erythematosus (SLE). We also measured antiphospholipid antibodies (APA), performed brain computed tomography (CT), and obtained a single photon emission CT (SPECT) to measure regional cerebral blood flow (rCBF) in order to elucidate a possible relationship between APA and higher cortical dysfunction. Higher cortical dysfunction was noted in as many as 16 (76%) out of 21 cases. APA were positive in 8 (38%) out of 21 cases. Although the relationship between APA and higher cortical dysfunction was not significant, patients positive for lupus anticoagulant (LA) were found to have higher cortical dysfunction. Brain CT revealed at least one abnormality in 6 cases (29%) but none had a localized lesion, SPECT disclosed a reduced rCBF in 9 cases (43%). The findings on brain CT and SPECT were unrelated to higher cortical dysfunction.

Study on the inhibitory effect of uremic plasma on lipoprotein lipase.

An investigation was undertaken to determine which plasma factors from normal controls and patients with chronic renal failure (CRF) exert have inhibitory effects on the activity of lipoprotein lipase (LPL) purified from heparinized human plasma by using an accurate LPL assay system. Inhibitors of LPL were found to be present in the plasma. The inhibition of the LPL activity was significantly greater in CRF patients than in normal controls. Following hemodialysis (HD), the same concentration of uremic plasma led to less inhibition. The inhibitors existed only in lipoprotein deficient plasma (LPDS), which demonstrated an LPL-inhibitory activity at extremely high concentrations with a significant difference between the patients and normal controls. There was no difference between the two groups at low concentrations. A specific inhibitory effect on LPL in LPDS was noted in the 7S and 4S fractions separated by gel filtration employing Sephacryl S-200 column chromatography. The inhibitory effect of the 7S fraction was found to be dependent on the concentration, and the difference between the two groups was similar to that for LPDS. The results obtained in the present study suggest that the plasma from CRF patients exhibited a strong inhibitory action on the LPL activity as compared to the plasma from normal controls, and the inhibitory action was due primarily due to poor excretion of dialyzable inhibitors.

[Analysis of circulatory responses to orthostatic stress in the elderly].

An active orthostatic stress test was conducted on 32 elderly patients over 65 years (elderly group) and 17 normal adults, for a comparative evaluation of their blood pressure, heart rates, and plasma catecholamine responses. In addition, 8 patients in the elderly group underwent a study of orthostatic responses with or without sublingual administration of isosorbide dinitrate (ISDN). The results of the orthostatic responses were evaluated by Schellong's method. The elderly group produced a positive reaction in one (3.1%), and a weakly positive reaction in two (6.3%), while the remaining 29 (90.6%) and all of the normal adults showed negative responses. The systolic blood pressure pattern that developed in response to rising among the elderly group was significantly different (p less than 0.001) from that of the normal adult group, but the response pattern seen in the heart rates of the former approximated that of the latter. The plasma norepinephrine concentration 10 minutes after rising increased significantly (p less than 0.001) in both the elderly and normal adult groups. This increase amounted to 1.5-fold in the elderly group and 2-fold in normal subjects, with a less prominent increase shown by the elderly. The norepinephrine levels of the elderly group were significantly higher than those of the normal adults both before and 10 minutes after rising (p less than 0.001 and p less than 0.01, respectively). In the elderly, ISDN caused a significant drop in the systolic blood pressure immediately after rising and a significant increase in the heart rate. Sublingual ISDN administration resulted in a significant increase in the norepinephrine level (p less than 0.05) in association with rising. These findings indicated that the circulatory responses to orthostatic stimuli are inappropriate as clinical data among the elderly and the administration of ISDN exaggerates this shortcoming further.