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PubMed1983

Lupus pleuritis. Clinical features and pleural fluid characteristics with special reference to pleural fluid antinuclear antibodies.

Eighteen patients with lupus erythematosus (LE) and pleural effusions were evaluated. Fourteen patients had lupus pleuritis and four had pleural effusions of other etiologies. All patients were symptomatic, and the presenting signs and symptoms did not help distinguish between lupus pleuritis and pleural effusions of other causes. The presence of LE cells confirmed the diagnosis of lupus pleuritis in seven of eight patients. In 11 of 13 patients with lupus pleuritis, the pleural fluid antinuclear antibody (ANA) titer was greater than or equal to 1:160, and in nine of 13 patients with lupus pleuritis, the pleural fluid to serum (PF/S) ANA ratio was greater than or equal to 1. In the four patients with LE and a pleural effusion of another etiology, the pleural fluid ANA titer was negative in two and low titer in two (1:40, 1:80); the pleural fluid to serum ANA titer was always less than one. Of 67 patients with pleural effusions of other etiologies, the pleural fluid ANA was negative. The signs and symptoms of lupus pleuritis are nonspecific, however; the findings of LE cells in pleural fluid confirms the diagnosis and a high pleural fluid ANA titer (greater than or equal to 1:160) and a PF/S ANA ratio of greater than or equal to 1 strongly supports the diagnosis.

Adolescent↗

PubMed1997

Molecular cloning of the bovine alpha 1(IV) procollagen gene (COL4A1) and its use in investigating the regulation of expression of type IV procollagen by retinoic acid in bovine lens epithelial cells.

This report is the first to describe the isolation of a 400 base pair cDNA clone encoding part of the bovine alpha 1(IV) procollagen. Using the polymerase chain reaction (PCR), we have amplified a sequence of approximately 400 bp from this gene within the recombinant phage DNA. The cloned sequence encodes 94 amino acids that form part of the protein's helical region. The sequence contains one interruption in the Gly-Xaa-Yaa repeat unit. The third base of the codon for glycine at several sites differs from those seen in murine and human genes, as does the third base of proline codons. The bovine cDNA also contains fewer thymine residues. Northern blot hybridization has shown that the mRNA for bovine procollagen to be 6.2 kb in size. We have used the cDNA clone to investigate the effect of all-trans retinoic acid (RA) on the gene expression of alpha 1(IV) procollagen in cultured bovine lens epithelial (LE) cells. We have also observed that RA decreases total protein production and concomitantly increases type IV procollagen in a concentration dependent manner. An increase in alpha 1(IV)mRNA as well as increase in type IV procollagen suggest that the regulation of alpha 1(IV) gene by RA in the LE cells is at the transcriptional level. Further, our results support the hypothesis that RA inhibition of lens epithelium transformation to fibroblast-like cells may be due to the ability of RA to stimulate the production of basement membrane components by epithelia.

Amino Acid Sequence↗

PubMed2002

H-Type 1 carbohydrate antigen expression by ovine endometrial cells.

Our objective was to determine whether H-Type 1 carbohydrate antigen is expressed by ovine endometrial epithelial cells. Endometrium was obtained from sheep on days (D) 1, 5, 11, 13, and 15 of the estrous cycle or pregnancy and D17 and D19 of pregnancy. Immunofluorescence microscopy of frozen tissue sections revealed intense staining on the apical surface of glandular uterine epithelial (GE) cells from D11 to D17 of pregnancy. Light punctate staining of luminal uterine epithelial (LE) cells was present from D15 to D19 of pregnancy, with isolated areas of intense staining observed only on D15 of pregnancy. There were no noticeable differences in staining patterns on equivalent d of the estrous cycle. Immortalized sheep LE and GE cells were used to determine whether estradiol (E), progesterone (P), or E + P, with or without interferon tau (IFNtau), regulates H-Type 1 antigen expression in vitro. Intermittent punctate surface staining was observed on both cell lines independent of steroid treatment. Treatment with P or IFNtau increased H-Type 1 antigen expression (P < 0.01) and resulted in large aggregates of punctate staining. Domain-specific biotinylation and Western blotting of cell lysates from LE and GE cells were used to identify proteins carrying the H-Type 1 antigen. For both cell types, major immunoreactive apical membrane proteins were detected at 31, 33, 42, 55, 60, and 70 kDa. Therefore, the H-type 1 antigen is expressed mainly on GE cells during pregnancy recognition in utero and up-regulated by P and IFNtau on LE and GE cells in vitro.

Animals↗

PubMed1974

Lupus pericarditis.

This report documents a case of systemic lupus erythematosus in which the major presenting feature was pericarditis and in which the diagnosis was made after pericardiocentesis by the demonstration of typical LE cells in pericardial fluid stained with Wright's stain. Review of the literature indicates that pericarditis in lupus is more common than is generally suspected, and when it is the initial manifestation it is often misdiagnosed as "benign viral pericarditis".Screening for collagen disease should be a part of the work-up of any patient presenting with pericarditis, especially a woman. Occasionally lupus may be present even when the serologic tests have given negative results. Under such circumstances, or when the patient manifests an atypical or prolonged course, a diagnostic pericardiocentesis is in order, provided an effusion can be confirmed on scan. A search for LE cells should be included in the analysis of the aspirated fluid.

Female↗

PubMedDate not supplied

Systemic lupus erythematosus: clinical and laboratory aspects related to age at disease onset.

OBJECTIVE: To analyse the clinical and laboratory parameters in patients with SLE according to their age at disease onset a retrospective study was undertaken of 272 Brazilian patients fulfilling the 1982 ARA criteria for SLE who were referred to the University Hospital of Campinas between 1973-1992. METHODS: The patients were divided into three groups according to their age at disease onset: Group A: under the age of 16 (39 patients); Group B age 17 to 49 (223 patients); Group C over the age of 50 (10 patients). Various clinical and laboratorial parameters were analysed and compared among these groups. RESULTS: There were no significant differences in terms of race, time of disease onset or time of follow-up. Group A had more male patients than Groups B (p < 0.05) or C. Alopecia as an early manifestation, seizures and gastrointestinal involvement were more frequent in Group A (p < 0.05). Raynaud's phenomenon was lower in Group A than in Groups B and C (p < 0.05). Pericarditis was higher in Group C than in Groups A or B (p < 0.05). Nephrotic syndrome was lower in Group C than in Group A (p < 0.05). Positive LE cells were higher in Groups A and C than in Group B (p < 0.05). Anti-DNA antibodies were more prevalent in Group A than in B (p < 0.05). Anti-cardiolipin antibodies were more frequent in adult patients (p < 0.05) (Group B). The mortality rate was higher in Group A than in B or C (p < 0.05). CONCLUSION: The clinical presentation and course of SLE may be influenced by the age at disease onset. Younger patients showed a poorer prognosis with more seizures, gastrointestinal involvement, nephrotic syndrome, and a higher rate of mortality than the other groups. Group A included more male patients and also exhibited more positive LE cells and anti-DNA antibodies. Raynaud's phenomenon was lower in these young patients. Elderly patients (C) presented more pericarditis and showed mild disease.

Adolescent↗

PubMed1980

[Sharp's syndrome. Clinical, immunological and nosographic aspects].

LE cells, ds-DNA antibodies (radioimmunoassay), antinuclear antibodies (ANA) by indirect immunofluorescence (IFI) and anti-ENA antibodies have been sought in 150 clinical cases observed over a 5-year period in the Rheumatology Division of Bari University. For the latter, three parallel techniques were adopted on each serum, each completed by RNA-sensitivity assay for the demonstration of anti-RNP, i.e. IFI, passive haemoagglutination (PHA) and controimmunoelectrophoresis (CIE). The series included systemic lupus erythematodes (SLE), 30 cases; rheumatoid arthritis (RA), 30 cases; progressive systemic sclerosis (PSS), 12 cases; unclassified connective tissue disease (UCTD), 8 cases; mixed connective tissue disease (MCTD), 7 cases; Sjögren's syndrome (SS), 4 cases; dermatomyositis (DM), 3 cases; overlap syndromes (PSS-SLE, SS-SLE), 2 cases; rheumatological and internal miscellanea, 54 cases, LE cells and ds-DNA antibodies were found exclusively in SLE; the anti-ENA were found in various groups of diseases, while the anti-RNP were only demonstrated in the 7 MCTD and in some SLE. Of the three techniques for demonstrating anti-ENA, the PHA proved most sensitive and CIE most specific, whereas IFI was considered most suitable for clinical screening. The clinical aspects of the 7 MCTD faithfully followed the disease picture described by Sharp, but some overlap-syndromes and the unclassified connective tissue diseases did not present anti-RNP. It is also pointed out that nephropathy is not rare in MCTD and that the clinical course of the disease is not always benign. To conclude, it is considered that MCTD merits nosographic autonomy, but further investigations are recommended for more exact nosographical typing of connective tissue diseases.

Antibodies↗

PubMed1993

Modulation of procainamide toxicity by selenium-enriched yeast in rats.

Free radical processes are proposed to play a crucial role in the development of procainamide adverse effects. Therefore, selenium, as a potent antioxidant, may modified procainamide toxicity. To test this hypothesis plasma and liver thiobarbituric acid-reacting substances (TBARS), plasma antioxidant activity (AOA), erythrocyte and liver superoxide dismutase (SOD), catalase, as well as selenium-dependent glutathione peroxidase (Se-GPX) were determined in the following four groups of rats: selenium-treated (Se), procainamide-treated (P), procainamide and selenium-treated (P + Se), and control (C). Morphological studies of leukocytes [tested for lupus erythematosus (LE) cells] and liver were also made. Atypical, i.e. enlarged and swollen, leukocytes resulting from procainamide and selenium treatment were observed. These changes were found in four out of five rats in the Se group, eight out of ten in the P group, and in seven out of ten in the P + Se group. LE-like cells were observed in two rats in the P + Se group. A statistically significant decrease in plasma and liver TBARS by 20% and 36%, respectively, increased activity of SOD by 20%, catalase by 48% and Se-GPX by 15% in erythrocytes, and decreased activity of liver SOD by 17% and catalase by 22% were found in the P + Se group as compared to the P group. These results indicated that selenium exerted antioxidant effects on the procainamide-treated rats. However, selenium did not prevent the development of disturbances in leukocyte morphology, on the contrary, it possibly promoted the conversion of leukocytes to LE cells.

Animals↗

PubMed2000

An established light ear mutant (C57BL/6J-Pdeb(rd1) le) mouse cell line exhibits a block to secretion of lysosomal enzymes.

The hypopigment mutant mice, light ear, pallid, and beige, possess defects in melanosomes, lysosomes, and platelet dense granules, suggesting that these organelles share a common biogenesis and processing. Light ear and pallid mutants are animal models for Hermansky Pudlak syndrome, whereas the beige mouse is an animal model for Chediak Higashi syndrome. An established skin cell line from the light ear mouse was tested along with pallid and beige cell lines for mutant effects on secretion of lysosomal hydrolase activities of six different lysosomal glycosidases and the trafficking of N-[5-(5,7-dimethyl BODIPY)-1-pentanoyl]-D-erythrosphingosine (C(5)-DMB-ceramide). There were no consistently significant differences between the pallid and the beige mutant cell lines or between these two mutant lines and the control cell line in the percentage secretion of lysosomal hydrolase activities. The light ear mutant cell line, however, displayed a significantly lower percentage secretion of lysosomal hydrolase activities than all other cell lines tested. The light ear mutant cells processed C(5)-DMB-ceramide completely, as seen in the control cell line, whereas pallid and beige cell lines retained fluorescent material and exhibited a block in the complete processing of C(5)-DMB-ceramide 20 h after labeling. The block to secretion of lyososomal hydrolase activities in the light ear mutant cell line will be useful for further studies on this mutant's lysosomal defect.

Albinism, Oculocutaneous↗

PubMed1975