To investigate the incidence, the specificity and clinical significance of positivity for serum anti-neutrophil cytoplasmic antibody (ANCA) in 31 patients with systemic lupus erythematosus (SLE), the indirect immunofluorescence (IIF) technique and enzyme-linked immunosorbent assay (ELISA) were used to measure ANCA. Purified myeloperoxidase (MPO), lactoferrin (LF), cathepsin-G (CTG) and elastase (HLE) served as ANCA antigens for ELISA. Thirteen (42%) of the 31 SLE patients showed positivity for perinuclear, but not cytoplasmic, ANCA by IIF. Five of 31 sera were positive for MPO, 10 for LF, 1 for CTG and 0 for HLE by ELISA. Patients positive for ANCA had a higher score of SLE disease activity index (SLEDAI) than those without ANCA. There was no correlation between ANCA positivity, clinical manifestations, or organic involvement. While the ANCA in patients with SLE reflected disease activity, it was unrelated to organic involvement.

We determined the effect of 17 -estradiol on tumor necrosis factor (TNF-)-induced cytotoxicity in human peripheral T lymphocytes (T cells) using lactate dehydrogenase assay. Treatment with 17 -estradiol (1-100 nM) for 24 h showed dose-dependent reduction of TNF--induced cytotoxicity in T cells. To further evaluate the mechanism of 17 -estradiol on TNF--induced cytotoxicity in T cells, we identified estrogen receptor (ER) protein in T cells using immunocytochemistry and used the pure ER antagonist ICI 172,780. ER immunoreactivity was clearly observed in T cells. ER immunoreactivity was also detected in some T cells. ICI 172,780 (10 7 M) alone did not affect cytotoxicity in T cells, however, ICI 172,780 (10 7 M) completely abolished 17 -estradiol cytoprotective effects in T cells. TNFtended to increase nuclear factor B (NF-B) protein levels in nuclear extracts but it did not reach statistical significance by Western blotting. In contrast, NF-B protein levels in nuclear extracts followed by TNF-with 17 -estradiol treatment were significantly increased compared with NF-B protein levels in untreated group. NF-B blocker pyrrolidinedithiocarbamate (PDTC) (10 4 M) alone did not affect cytotoxicity in T cells. In contrast, PDTC (10 4 M) completely abolished 17 -estradiol cytoprotective effects in T cells. Caspase -3/-7 activity was significantly increased followed by TNF-, and 17 -estradiol treatment significantly reduced the increment. The present studies suggest the protective effect of 17 -estradiol on TNF--induced cytotoxicity through ERs in T cells and that NF-B activation and caspase suppression may be involved in the mechanism.

A 66-year-old Japanese male, who suffered from persistent proteinuria and leg edema, underwent renal biopsy. Light microscopy revealed marked narrowing of the glomerular capillary lumen with a diffuse accumulation of weakly PAS-positive material. By electron microscopy, abundant abnormal collagen fibers were observed predominantly in the subendothelial space and occasionally in the mesangial matrix. The fibers had a periodicity of about 60 nm and were immunoreactive for anti-type III collagen. Subendothelial electron-dense deposits were also found in some of the capillary walls. The serum level of procollagen III peptides was elevated and changed in parallel with the amount of proteinuria during the patient’s clinical course. On the basis of these findings, a diagnosis of the collagenofibrotic glomerulonephropathy was made. A review of the literature, including 29 similar or identical cases, failed to reveal the etiology and pathogenesis of this disease. We suggest that this disease may be divided into two different clinical subtypes, an adult-onset type and a pediatric type.

Abstract. A 45-year-old Japanese woman, treated for Bartter's syndrome for 14 years, presented with complaints of numbness in her extremities and polyarthralgia.She was diagnosed to have Gitelman's syndrome with chondrocalcinosis, which were effectively treated with spironolactone and magnesium supplementation.Gitelman's syndrome is a primary renal tubular disorder characterized by hypomagnesemia and hypocalciuria with normal calcemia. The persistent hypomagnesemia is one of the causes of chondrocalcinosis, and many cases of Bartter's syndrome with hypomagnesemia are associated with chondrocalcinosis attributed to a tubular magnesium defect. We summarize the reported cases with Bartter's syndrome and chondrocalcinosis, referring to the possibility of Gitelman's syndrome.

Objective-To examine the eVect of iron on the prostaglandin (PG) E 2 production by human synovial fibroblasts in vitro. Methods-Human synovial fibroblasts were isolated from synovial tissue of rheumatoid arthritis (RA) and osteoarthritis (OA) patients and cultured in medium. Synovial fibroblasts were stimulated by human recombinant interleukin (IL) 1 (0.1-10 ng/ml) with or without ferric citrate (Fe-citrate, 0.01-1 mM). The amount of PGE 2 in the culture medium was measured by an enzyme linked immunosorbent assay. Results-The production of PGE 2 by the synovial fibroblasts was increased by stimulation with IL1 at all concentrations tested. Fe-citrate but not sodium citrate (Na-citrate) down regulated the production of PGE 2 by the synovial fibroblasts, both with and without stimulation by IL1 . Fe-citrate inhibited the spontaneous PGE 2 production by the cells in a dose dependent manner, and a maximum inhibition by Fe-citrate was observed at the concentration of 0.1 mM with IL1 stimulation. The down regulation by iron was reversed by the co-addition of desferrioxamine (100 µg/ml), an iron chelator. Conclusion-Iron down regulates the PGE 2 production by synovial fibroblasts in vitro.