Case report: a rare association of primary biliary cirrhosis and systemic lupus erythematosus and review of the literature.

We report a rare occurrence of systemic lupus erythematosus in a patient known to have had well-documented primary biliary cirrhosis for 10 years. The presentation was dramatic with pericardial tamponade, but responded well to high dose corticosteroid. There are only five such definite associations reported in the literature. In the present case, other possible causes were considered, such as drug-induced cholestasis, drug-induced lupus, autoimmune chronic active hepatitis and the overlap syndrome.

Characterization of a glomerular epithelial cell metalloproteinase as matrix metalloproteinase-9 with enhanced expression in a model of membranous nephropathy.

The role of the glomerular visceral epithelial cell in the physiologic turnover and pathologic breakdown of the glomerular extracellular matrix has remained largely unexplored. In this study a 98-kD neutral proteinase secreted by cultured rat visceral glomerular epithelial cells was shown to be a calcium, zinc-dependent enzyme secreted in latent form. In addition, the protein was heavily glycosylated and demonstrated proteolytic activity against Type I gelatin, Type IV collagen gelatin, and fibronectin. The similarity in molecular mass and substrate specificities to the 92-kD human matrix metalloproteinase-9 (MMP-9, or gelatinase B) suggested the identity of this activity, which was confirmed by immunoprecipitation and Northern blot analysis. The differences in molecular mass (98 vs. 92 kD) were not due to species-specific differences in glycosylation patterns, since cultured rat peritoneal macrophages secreted MMP-9 as a 92-kD enzyme. Furthermore, transfection of the human MMP-9 cDNA into rat glomerular epithelial cells yielded the 98-kD product. Using a specific monoclonal anti-MMP-9 antibody and in situ reverse transcription (ISRT) analysis of MMP-9 mRNA, the expression of this enzyme was evaluated in vivo. Normal rat glomeruli expressed little immunohistochemical or ISRT staining for MMP-9, while in rats with passive Heymann nephritis there was a major increase in MMP-9 protein and mRNA staining within the visceral epithelial cells. The temporal patterns of MMP-9 expression correlated with the period of proteinuria associated with this model, suggesting that a causal relationship may exist between GEC MMP-9 expression and changes in glomerular capillary permeability.

Orthodontic abnormalities in patients with eating disorders.

Many anorexic and bulimic patients induce vomiting by inserting their fingers in the oral cavity. We hypothesized that finger pressure could lead to tooth movement, eventually resulting in the development of orthodontic abnormalities, particularly open-bite. Twenty-four females with eating disorders and 24 matched controls underwent orthodontic examination and completed a dental questionnaire. Orthodontic abnormalities were more commonly found in the eating disorder group and open-bite was the most common abnormality seen. The presence of open-bite or other orthodontic abnormality was not associated with patient reports of self-induced vomiting, which would suggest that digital pressure was not the causative factor. The dental appearances indicated that the skeletal base pattern was abnormal in many cases. The findings could, in part, be accounted for by the fact that patients with eating disorders were less likely than controls to have completed courses of orthodontic treatment. Alternatively, the orthodontic abnormality may have contributed to the development of an eating disorder. Further study of this area is proposed.

Cholesterol crystals in shoulder synovial fluid.

Samples of 1945 synovial fluids have been examined from patients with a wide variety of joint disorders. Typical cholesterol crystals were seen in only 14 of these samples, all from the shoulders of six patients with severe rheumatoid arthritis and persistent shoulder effusions.

Effect of lithium therapy on inflammatory response.

Chemiluminescence produced by normal cells was reduced in response to zymosan which was opsonized with serum from patients on prophylactic lithium therapy, compared to control serum from normal subjects (68 +/- 3.1 vs. 93 +/- 3.4 mV/5 X 10(5) cells). Preincubation of normal cells with serum from patients also resulted in reduced chemiluminescence activity when the cells were stimulated with autologous serum-coated zymosan (47 +/- 4.5 vs. 64 +/- 6.3 mV/5 X 10(5) cells). Spontaneous complement conversion was increased in the serum of patients on lithium therapy (46.3 +/- 3.8 vs. 25.3 +/- 2.5% conversion). These studies demonstrated that lithium, at safe therapeutic levels (0.4-0.9 mmol/liter), significantly altered complement conversion and had a marked affect on chemiluminescence activity by normal cells.