Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0349506 (photosensitivity)
 4,145 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 22 year old male of lupus nephritis associated with minimal change nephrotic syndrome was described. The patient was well until 4 years earlier, when Raynaud's phenomenon and photosensitivity developed. One week before admission, edema appeared suddenly and proteinuria was pointed out on July 28, 1990. On admission, his legs, ankles, and eyelids were edematous. There was no sclerodactylia, although Raynaud's phenomenon was positive when his hand disclosed to the cold. Urinalysis showed heavy albuminuria (10.4g/day), but urinary sediment showed no abnormality. Immunological examination showed positive antinuclear antibody, determination at a titer of 1: 160 with a speckled pattern. Anti-RNP and anti-Sm antibody were positive. However, neither anti-DNA antibody nor hypocomplementemia was detected. There was high concentration of serum IgE (2564IU/ml). Renal biopsy was performed. Light microscopic study showed slight increase of mesangial cells and matrix. Immunofluorescence study showed mesangial localization of IgG and C3. Electron microscopic study showed electron dense deposits only in the mesangial area. The diagnosis of lupus nephritis associated with minimal change nephrotic syndrome (MCNS) was made and administration of PSL was started. Proteinuria disappeared after 3 weeks and nephrotic syndrome remitted completely. The case of lupus nephritis associated with MCNS is very rare. Therefore, the relationship between amounts of proteinuria and various histological types of 67 cases with lupus nephritis which we experienced at our hospital was evaluated. Results showed that nephrotic range proteinuria was not present in mesangial lupus nephritis. So, it was concluded that if heavy albuminuria was found in mesangial lupus nephritis, we should consider the possibility of lupus nephritis associated with MCNS.
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PMID:[A case report of lupus nephritis associated with minimal change nephrotic syndrome--comparison of various histological types of 67 cases with lupus nephritis]. 147 24

Although sunlight is known to induce skin lesions in patients with systemic lupus erythematosus (SLE) and to exacerbate systemic manifestations, the underlying mechanisms remain obscure. We report experiments that show enhanced binding of IgG autoantibodies to the cell surface membrane of ultraviolet-B (UVB) irradiated (200-1,600 J/m2) cultured SLE keratinocytes in 10 out of 12 such cell strains. The autoantibody probes showing increased binding were directed against the soluble intracellular antigens, Sm, RNP, SSA/Ro, SSB/La, whereas serum with anti-dsDNA activity did not demonstrate such binding. Control keratinocytes from several sources shared low level binding of autoantibodies after ultraviolet light exposure. In addition, 4/6 UVB-sensitive SLE strains showed increased autoantibody binding to the surface of SLE keratinocytes after UVA exposure (50-150 kJ/m2), but of lower magnitude. When UVB-sensitive nonirradiated SLE strains were exposed to autologous serum, 3/8 sera demonstrated a striking increase in IgG binding, which increased further after UVB exposure. Enhanced expression of saline-soluble intracellular antigens on the cell surface membrane of patient, but not control, keratinocytes may, in part, explain the photosensitivity of patients with SLE.
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PMID:Enhanced membrane binding of autoantibodies to cultured keratinocytes of systemic lupus erythematosus patients after ultraviolet B/ultraviolet A irradiation. 152 15

Serological features were investigated in 22 patients with polymorphous light eruption. The diagnosis was made from the history and clinical patterns and confirmed by phototesting and histological examinations. In 4 patients with polymorphous light eruption, anti-RNP antibodies were detected in the serum despite the fact that the antinuclear antibody tests were negative in all cases. The serum levels of anti-SSA/Ro antibodies in the patients were mildly but significantly elevated (p less than 0.001) as compared with healthy controls. No difference was found between the patients and controls regarding the serum concentrations of anti-SSB/La antibodies. The conclusion is drawn that it is necessary to examine patients with polymorphous light eruption with regard to a 'late autoimmune course' and it is worthwhile to study the relationship between the photosensitivity and the occurrence of anti-SSA/Ro antibodies.
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PMID:The occurrence of antinuclear, anti-SSA/Ro and anti-SSB/La antibodies in patients with polymorphous light eruption. 168 54

Autoantibodies to the non-histone nucleoprotein antigens SS-A/Ro, SS-B/La, and RNP are highly associated with photosensitive cutaneous lupus erythematosus (LE). In order to better understand the potential mechanisms of ultraviolet (UV) light on photosensitivity in patients with cutaneous LE, we designed immunopathologic in vitro and in vivo experiments to evaluate the effects of UV on the binding of such autoantibodies to the surface of human keratinocytes, one major target of immunologic damage in photosensitive LE. Short-term 2% paraformaldehyde fixation of suspensions of cultured human keratinocytes previously incubated with monospecific antiserum probes enabled the detection of ENA expression on the cell surface by flow-cytometry analysis. UVB light (280-320 nm) induced the binding of monospecific antibody probes for SS-A/Ro and SS-B/La on keratinocytes in a dose-dependent pattern with maximal induction observed at the dose of 200 mJ/cm2 UVB. Binding of SS-A/Ro, SS-B/La, and RNP antibody was augmented strongly, but binding of anti-Sm was very weak. In contrast, UVA (320-400 nm) light had no effect on the induction of binding of these antibody probes. Identical results were seen by standard immunofluorescence techniques. Hydroxyurea-treated keratinocytes showed similar induction of those antigens by UVB irradiation, which suggested that ENA expression on cultured keratinocytes by UVB were cell-cycle independent. Tunicamycin, an inhibitor of glycosylation of proteins, reduced UVB light effect on the SS-A/Ro and SS-B/La antigen's expression. These in vitro FACS analyses revealed that ENA augmentation on the keratinocyte cell surface was dose dependent, UVB dependent, glycosylation dependent, and cell-cycle independent. In vivo ENA augmentation on the keratinocyte surface was examined in suction blister epidermal roofs. Specific antibody probes for SS-A/Ro, SS-B/La, RNP, and Sm bound to human keratinocytes in intact suction blister epidermis following UVL irradiation in vivo. Using three different protocols, we have demonstrated that antibodies to SS-A/Ro, SS-B/La, and U1RNP bind to UVL-irradiated human keratinocytes. We speculate that this antibody binding is an important inducer of antibody dependent keratinocyte damage in photosensitive cutaneous lupus.
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PMID:Binding of antibodies to the extractable nuclear antigens SS-A/Ro and SS-B/La is induced on the surface of human keratinocytes by ultraviolet light (UVL): implications for the pathogenesis of photosensitive cutaneous lupus. 187 59

Homozygous C4A deficiency was found at a prevalence of 16% (13/80 patients) in systemic lupus erythematosus (SLE). The patients represented all diagnosed cases retrieved from a defined population in Southern Sweden, which minimizes the influence of patient selection. Photosensitivity was more common among C4A-deficient patients than among other SLE patients (p less than 0.05). Otherwise, clinical features were similar in the two groups. In addition, no differences were found with regard to presence of various autoantibodies (anti-dsDNA, anti-Sm, anti-RNP, anti-SSA, anti-SSB, rheumatoid factors and anti-cardiolipin). In patients expressing both C4A and C4B isotypes, C4B/C4A quotients were fairly stable in plasma irrespective of disease activity. This argues against preferential break-down of either isotype during complement activation in the disease. The increased photosensitivity of C4A-deficient patients partly resembles the findings in patients with complete deficiencies of classical pathway components.
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PMID:Homozygous C4A deficiency in systemic lupus erythematosus: analysis of patients from a defined population. 228 15

The identification of differences in the clinical manifestations of systemic lupus erythematosus (SLE) due to racial and socioeconomic factors has been hampered in previous studies by limitations in the numbers of black patients examined. We sought to define racial differences in the cumulative clinical manifestations of SLE in a large, racially balanced cohort (184 black patients and 174 white patients). Differences in the cumulative disease manifestations of SLE between black and white patients were evaluated by multivariate regression techniques, controlling for socioeconomic status and the potential confounding factors of age, gender, duration of follow-up, and treatments. Race was found to be an important factor influencing the prevalence of 9 of 24 clinical features of SLE. As a group, blacks more commonly manifested anti-Sm and anti-RNP antibodies, discoid skin lesions, and proteinuria, and less commonly manifested photosensitivity, than whites. Among specific age, gender, and socioeconomic subgroups, blacks were more likely than whites to have had psychosis, serositis, and urinary cellular casts, and less likely to have had sicca syndrome. Racial differences in the prevalence of renal failure were due to socioeconomic effects. These results suggest that race is under-recognized as a factor influencing the clinical heterogeneity of SLE.
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PMID:Clinical manifestations of systemic lupus erythematosus. Identification of racial and socioeconomic influences. 232 45

A clinicopathological study of antinuclear antibodies was performed in 43 patients with SLE. Autoantibodies to double-stranded DNA, measured by RIA, were found in 56% of subjects and were associated with the presence of mesangioproliferative glomerulonephritis, Raynaud's phenomenon and cryoglobulinaemia. Antibodies to extractable nuclear antigens (ENA), when assayed by ELISA, were found in 81% of subjects and were associated with photosensitivity and thrombocytopaenia. Antibodies to RNA-ase-resistant ENA were found in 42% and were associated with cryoglobulinaemia and sclerodactyly, while antibodies to RNA-ase sensitive ENA which had a 49% prevalence were associated with haemolysis and neutropaenia. RNP antibodies (detected by immunodiffusion in 40%) were also associated with photosensitivity. RNP antibody positive and negative sera differed from each other by virtue of their relationship with other autoantibodies, and because RNP-positive sera had significantly higher ENA antibody titres and affinities than did RNP-negative sera. We therefore concluded that various combinations of antinuclear antibodies may predispose to the development of specific clinicopathological lesions in SLE.
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PMID:Subsets of autoantibodies to extractable nuclear antigens (ENA) as assayed by ELISA correlate with specific clinical abnormalities in SLE. 242 99

15 cases of subacute cutaneous lupus erythematosus are reported. The diagnosis was based on the presence of the typical clinical features, on the histologic and immunpathologic examination of lesional skin and on the characteristic laboratory findings. 8 patients had annular type, 4 patients had papulosquamosus type of the characteristic skin signs of subacute cutaneous lupus erythematosus. In 3 patients both types of lesions existed simultaneously. 5 patients fulfilled the American Rheumatism Association criteria for systemic lupus erythematosus, however the systemic symptoms (arthritis, arthralgia, fever, myalgia, photosensitivity) were mild. 4 patients had positive ANA test, anti-Ro/SSA antibodies were determined in 5 patients, anti-RNP antibodies were detected in 8 patients. Anti-dsDNA antibodies were not detected. Subacute cutaneous lupus erythematosus is an intermediate subset in severity between discoid lupus erythematosus and severe systemic lupus erythematosus, therefore a milder form of therapy should be chosen.
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PMID:[Subacute cutaneous lupus erythematosus based on a study of 15 cases]. 268 50

Epidemiological, clinical and serological data of uranium miners with symptoms of connective tissue diseases (CTD) were collected during the control examinations for occupational lung diseases since 1975. Twenty eight definite (four or more ARA criteria) and 15 probable (2-3 ARA criteria) SLE were diagnosed. The estimated prevalence among heavily silica exposed uranium miners was up to 93 in 100,000. The only significant differences to nonexposed SLE patients were decreased frequency of arthritis and photosensitivity and the absence of anti-Sm and anti-U1-RNP antibodies. ANA were found in all definite SLE patients examined with the following specificities: anti-dsDNA (in 44.4%), & anti-Ro/SSA (in 55.6%, four cases together with anti-dsDNA) and anti-La/SSB (in 22.2%). The autoantibody profiles of patients with probable SLE were similar, but with a lower frequency of ANA, anti-dsDNA and anti-Ro/SSA. Middle to high-titred autoantibodies to dsDNA, Ro/SSA and La/SSB were detected in 3.2% uranium miners with no (N = 1229) and in 20.6% with some symptoms (one ARA criterion and/or two or more of other CTD typical symptoms, N = 68) of CTD development. We conclude, that the strong exposure to dust with a high content of silica may predispose to or initiate the development of SLE. The detection of SLE-typical antibodies in quartz dust-exposed miners may indicate a higher risk for the development of systemic autoimmune disease.
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PMID:Systemic lupus erythematosus after heavy exposure to quartz dust in uranium mines: clinical and serological characteristics. 864 29

Black patients with systemic lupus erythematosus (SLE) have a lower prevalence of photosensitivity rashes than white patients. The reasons for this are unknown, but some studies suggest a correlation between the presence of antinuclear antibodies and protection from photosensitivity. In our study, we determined serum antinuclear-antibody profiles, including anti-dsDNA, anti-Sm, anti-RNP, anti-Ro/SS-A, and anti-La/SS-B antibodies, in 91 black Jamaican patients with SLE. All 91 serum samples from SLE patients (100%) were positive in the fluorescent antinuclear-antibody test. Using the crithidia luciliae immunofluorescence test, anti-dsDNA was found in 27.5% of the samples. By a double immunodiffusion method, anti-Sm antibodies were found in 15.4%, anti-RNP in 18.7%, anti-Ro/SS-A in 9.9%, and anti-La/SS-B in 11.0%. However, no statistically significant differences were observed in the seroprevalence of these antinuclear antibodies when sera from patients of the following groups were compared: only photosensitivity rashes (n = 17), photosensitivity and other rashes (n = 23), other rashes without photosensitivity (n = 27), and patients with no skin rash of any type (n = 24). These results suggest that photosensitivity in black Jamaican patients with SLE is not associated with antinuclear-antibody specificity.
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PMID:Photosensitivity and antinuclear antibodies in black patients with systemic lupus erythematosus. 899 92


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