The envelope glycoprotein of human immunodeficiency virus (HIV) has been know to play an important role in the induction of immune dysregulation of HIV infection. Recent evidence suggests that HIV requires the chemokine receptors as a coreceptor. In addition, we found that HIV-2 (but not HIV-1) envelope glycoprotein can bind to not only CD 4 but also CD 8 molecules on human T cells. Based upon these findings, we discuss the effect of HIV envelope glycoprotein on the pathogenic role of HIV infection and the difference between HIV-1 and HIV-2 envelope glycoproteins in the biological characteristics and pathogenecity.

View full abstract

The dosage of cyclosporine administered in the treatment of autoimmune diseases has generally been comparable to those required in cases of transplantation. Here we report on the successful treatment using an extremely low dose cyclosporine (1mg/kg/day) on four patients, involving thrombocytopenia with systemic lupus erythematosus, and interstitial pneumonitis with Sjögren's syndrome, and discuss the optimal dose of cyclosporine for autoimmune-mediated manifestations.

View full abstract

Twelve patients with idiopathic thrombocytopenic purpura (ITP) who were resistant to conventional steroid therapy under went partial splenic embolization (PSE). PSE was effective for 7 out of 10 eligible cases. The reduction ratio of PAIgG was significantly higher in effective cases. The PAIgG level before treatment and platelet counts one week after PSE were higher in effective cases than those of non-effective cases. The effects of PSE did not depent on age, sex, the duration of disease, platelet count, embolization rate or the effect of intravenous immunoglobulin therapy. No serious side effects were observed. PSE can be an alternative therapy to splenectomy for ITP.

View full abstract

We encountered a boy highly suspected of Shwachman syndrome. The affected child presented a mild disturbance of the extrapancreatic secretory function followed by a marked decrease in the number of peripheral blood neutrophils and contracted respiratory infections repeatedly. The neutrophilic function of the affected child showed the disturbance of motility both in vivo and in vitro. So the impairment of the neutrophil itself was considered accountable for it. After admission, he contracted a variety of viral infections and then showed an increase in the number of peripheral blood neutrophils.
Furthermore, the disturbance of the extrapancreatic secretory function is also improved slowly. Shwachman syndrome is a rare disease in Japan. From the clinical course of this case, it may be reasonable to infer that there are various variant types in Japan.

View full abstract

We report a case of a 37-year-old man with relapsing polychondritis and IgA nephropathy. He visited our hospital with high fever and the swelling of his ears and eyelids.
His symptoms and the results of the biopsy of his right auricle fulfilled the Damiani's criteria. Laboratory examination on admission showed an increase of serum IgA level, a presence of immune complex, remarkable hematuria (grade III) and proteinuria (grade II). Most of his symptoms were improved by the administration of antibiotic and NSAIDs, however, urinary findings still remained unchanged. The biopsy of his right kidney led to a diagnosis of IgA nephropathy (group II).
Although relapsing polychondritis is known to associate rarely with renal involvement, it is very rare to associate with IgA nephropathy. This case indicates that immune disorders including IgA nephropathy should be investigated in patients with relapsing polychondritis.

View full abstract

A 43-years-old female was admitted to our hospital because of facial erythema and photosensitivity in 1983 and was diagnosed as systemic lupus erythematosus (SLE). She was treated with betamethazone 2.5mg/day as an outpatient. Abdominal pain and diarrhea were developed in September, 1995. So she was admitted to our hospital and diagnosed as having paralytic ileus. Ultrasonography showed marked intestinal edema. Forbidden oral intake and given antibiotics, she was satisfactorily improved in a few days, but the symptoms got worse soon. We forbad oral intake again. We performed a pulse therapy with 1g of methylprednisolone and increased a dose of betamethasone 2.5 to 4.0mg/day, but the symptoms were not improved. Since October 6th, serum ceatinine level (s-CRE) increased to 8.1mg/dl at October, 20th. We suspected the worsening of SLE nephropathy or drug-induced nephropathy, so we stopped a medication of pravastatin and used 50 mg/day of azathioprine. Moreover, we did a pulse therapy of methylprednisolone and a plasmapheresis. By these treatments, s-CRE level returned to normal range and paralytic ileus was completely improved.
The cause of hypercreatininemia was suspected to be rhabdomyolysis due to pravastatin. The main cause of paralytic ileus with intestinal edema was suspected to be vascular disturbance of superior mesenteric arteries.
We consider that pulse therapy and plasmapheresis are useful for a patient of SLE with marked intestinal edema and paralytic ileus.

View full abstract

A 46 year-old female presented with dry eyes and a dry mouth which she had been experienceing for about 15 years. She also began to notice dizziness (orthostatic hypotension) during the last 5 years. The symptoms gradually increased whereupon she began to have polyarthralgia, facial flashing, hyperand hyposweating in some areas on the face and trunk. - Her sialography showed a damaged parotid gland. Minor salivary gland biopsy revealed chronic sialoadenitis. The Sirmer test was low, and the Rosebengal test indicated keratoconjunctivitis sicca. Her serological tests showed hypocomplementemia and were positive for antinuclear antibody and SS-A antibody. The diagnosis of primary Sjögren's syndrome (SjS) was made based on these findings. Prednisolone, at a dose of 15 mg per day, was given orally. As a result of this therapy, arthralgia disappeared immediately, although it had no effect on the neuropathy found in this patient. This is a rare case of SjS associated with peripheral neuropathy and severe dysautonomia.

View full abstract