Background. The role of anti-endothelial cell antibody (AECA) in systemic vasculitis has been reported. One candidate which may disrupt vascular function is AECA. In order to investigate the role of AECA in preeclampsia, the incidence of AECA positive patients, the characteristics of the clinical findings of AECA positive patients and also the cytotoxicity of AECA positive serum for cultured endothelial cells was studied.
Methods. Serum samples were taken from 57 preeclampsia (including 37 severe cases) and 46 normal pregnant women. The AECA were measured by ELISA using human umbilical vein endothelial cells. The cytotoxicity for cultured endothelial cells by test serum was measured by using ⁵¹Cr release assay.
Results. The incidence of IgG and IgM AECA were revealed in 26.3% and 10.5% of preeclampsia respectively. AECA was detected more frequently in severe (29.7%) than in mild preeclampsia (20.0%). In cases with severe proteinuria of greater than 200mg/dl we detected a significantly higher incidence of AECA than in mild cases (p<0.04). The incidence of AECA was not significantly increased in cases with severe hypertension or IUGR. The AECA positive sera had greater cytotoxic activity on endothelial cells than AECA negative sera (p<0.03).
Conclusions. The appearance of AECA is related to the severity of proteinuria and the cytotoxicity to endothelial cells by AECA positive sera may play a role in causing the endothelial damage in preeclampsia.

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A 56-year-old man was admitted to our hospital due to gradually developing consciousness disturbance. He had an 11-year history of sero-negative rheumatoid arthritis (Stage III) maintained by daily administration of 10mg of prednisolone and 300mg of actarit. On admission, he showed meningeal irritation and a marked increase in eosinophils in his cerebrospinal fulid (CSF) (457/μl), while eosinophils in his peripheral blood were not increased (0/μl). Shortly after admission he fell into a coma. Upon measurement in the coma state, his peripheral blood eosinophil count was found to be increased (max: 1742/μl). Parasitic infection, Angiostrongylus cantonensis in particular, was excluded both by repeated microscopic examination of CSF and by immunological approaches for CSF and serum. Serum examinations showed broad cross-reaction between various parasitic antigens and positive myeloperoxdase-antineutrophil cytoplasmic antibody (18 EU/ml). Three pulses of methylprednisolone (500mg/day) followed by conventional prednisolone therapy (60mg/day) was effective for alleviating the signs and symptoms of eosinophilic meningoencephalitis. In this patient, it was considered that the cerebrospinal angiitis resulting in eosinophilic meningoencephalitis had been elicited by immunological abnormalities.

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The patient was a 23-year-old woman with HTLV-I carrier and was diagnosed as pityriasis rubra pilaris soon after her birth. In November 1990 at her age of 16, she began to have fever and polyarthralgia which were not improved despite the administration of antibiotics. Her laboratory findings showed the positive antinuclear antibody and anti-RNP antibody suggesting one of collagen diseases. A tentative diagnosis as unclassified connective tissue disease (UCTD) was made since her symptoms and laboratory findings were not satisfied with any criteria for rheumatic disorders. The steroid therapy was started in February 1991 and showed a good response. On April 9th, 1996, however, she was admitted to our hospital because of recurrence of high fever and chillness. This time, she had sepsis because of the evidence that Enterococcus faecalis was detected in blood culture. Although her condition was improved by antibiotics on June 4th 1996, high fever re-appeared and followed by convulsion and disseminated intravascular coagulation. After the dosis of prednisolone per day was increased to 40mg with antibiotics and anticoagulant, her condition gradually improved. This patient was a rare case of pityriasis rubra pilaris associated with UCTD and sepsis in clinical course.

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A 20-year-old woman initially presented with edematous erythema in the upper eyelids in December 1995. She was admitted to our department in January 1996 because of fever and multiple arthralgia. She was given a diagnosis of dermatomyositis (DM) on the basis of characteristic eruption, elevated serum level of creatine kinase, and increased inflammatory reaction. Chest computed tomography (CT) revealed faint interstitial changes in the left lower lung. The administration of corticosteroid caused improvement in the patient's condition and the interstitial lesion in the lung. Although she was asymptomatic, chest CT showed pneumomediastinum in the pretracheal space and concomitant pneumothorax around left bronchus. Those changes spontaneously disappeared 4 weeks later without treatment. The occurrence of pneumomediastinum in patients with DM has been well documented as an indicator of poor prognosis in the literature. It seems that a severe pulmonary disorder could secondarily cause pneumomediastinum. In our case, however, the pneumomediastinum developed in spite of the low grade severerity of the pulmonary lesion. This finding suggested that the pneumomediastinum may be associated with the development of DM itself.

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We reported a 68-old female who was diagnosed to have systemic lupus erythematosus 18 years ago. She had been well under 5mg of prednisolone until 1995, when she felt severe shortness of breath. Laboratory examinations disclosed severe anemia accompanying mild thrombocytopenia. Bone marrow aspiration revealed a complete absence of erythroid progenitor cells.
She was diagnosed to have pure red cell aplasia (PRCA) as well as antiphospholipid syndrome. A judicious use of methylprednisolone including pulse therapy resulted in a prompt resolution of anemia as well as thrombocytopenia and the dose of corticosteroid was tapered successfully thereafter.
Persistent infection of HPV B 19 in the patient with inactive SLE was considered as a main cause of PRCA because not only IgG-HPV B 19 antibody but viral DNA was demonstrated in her serum at the time of admission. The relationship between PRCA and anti-phospholipid syndrome in this patient was also noted.

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A 71-year-old man had noticed a dry sensation in the mouth with swelling of bilateral parotid glands in 1988. He was given a diagnosis of Sjägren's syndrome (SS) on the basis of characteristic findings of sialography and a minor salivary gland biopsy. He was admitted to our department in Febrary 1995 because of general fatigue of 2 month's duration. Laboratory data showed both positive anti-SSA and anti-SSB antibodies, liver dysfunction, hypoalbuminemia, and thrombocytopenia. Abdominal CT and MRI demonstrated a 2-cm intrahepatic mass (S 6) with enhanced areas at the periphery. The liver biopsy yielded fragments from the intrahepatic mass and hepatic parenchyma. The former was composed of plasma cells, lymphocytes, and histiocytes, compatible with the diagnosis of inflammatory pseudotumor of the liver. The pathological diagnosis of the latter specimen was primary biliary cirrhosis, although antimitochondrial antibody was negative. The intrahepatic mass gradually decreased in size without treatment. Inflammatory pseudotumor is considered to be a benign inflammatory condition simulating a neoplasma and the possibility of an autoimmune reaction is suggested on the basis of etiology. This is the first report of an inflammatory pseudotumor associated with Sjögren's syndrome developing in the liver. The inflammatory pseudotumor should be considered as a possible diagnosis in cases where the tumor is embedded in the liver.

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