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Antibodies to cellular antigens in Sjögren's syndrome.

An extract of human lymphocytes from continous cell culture was used as the antigen source to detect antibodies in sera of patients with Sjögren's syndrome (SS). Using double diffusion in agarose, 85 per cent of a selected group of patients had precepating antibodies. Three precipitating antigen-antibody systems were detected and were shown to be different from those described previously in othe systemic rheumatic diseases. The SS precipitating antibodies were temporarily classified as precipitins, A, B, and C. SS patinets with sicca syndrome but without clinical rheumatoid arthritis had precipitin systems A and/or B, and SS patients with associated rheumatoid arthritis had precipitin system C. Serum reactants were demonstrated by immuno-electrophoresis to migrate in the gamma globulin region. The precipitating activity of the serum factors was not destroyed by treatment with 2-mercaptoethanol and was not removed by absorption of rheumatoid factor from the sera. The reactivity of the lympuocyte antigens was destroyed by treatment with trypsin but not by deoxyribonuclease or ribonuclease.

Absorption↗

LETS protein in normal and pathological human oral epithelium.

The distribution of LETS protein in human oral mucosa was studied by indirect immunofluorescence. Normal epithelium showed surface staining. Intracellular staining occurred in epithelial cell cytoplasm in lichen planus and pemphigoid. Surface staining was absent in discoid lupus erythematosus. In pemphigus, intercellular staining was seen near areas of acantholysis.

Acantholysis↗

Ethnic and geographical differences in systemic lupus erythematosus: an overview.

Systemic lupus erythematosus (SLE) is one of the most heterogeneous autoimmune disorders known. There is production of a variety of autoantibodies and patients present with a wide range of symptoms due to multiple organ involvement by the disease process. The underlying cause is not fully understood but it may involve genetic and environmental factors. It is interesting to note that while SLE is found worldwide, it is more commonly found in some countries, and within a country certain ethnic groups appear to be more susceptible to develop this condition than others. Additionally, the presentation and course of SLE appear highly variable between patients of different ethnic origins. For example, African-Americans and Orientals are believed to have a more severe disease than Caucasian whites. But are these ethnic and geographical differences real? If yes, they may provide investigators insight into the underlying pathoaetiology of this condition and pave the way to future research directions in lupus.

Anemia, Hemolytic, Autoimmune↗

The sequelae of chronic cutaneous lupus erythematosus.

Eighty-six patients with chronic cutaneous lupus erythematosus were examined. Twelve of these also suffered from systemic lupus erythematosus. The mean duration of the disease was 15.1 years. Fifty-seven percent of patients (49/86) had scarring of some kind producing destruction and deformity; 47% (41/86) had scarring of glabrous surfaces and 35% (30/86) had scarring alopecia; 35% (30/86) were also suffering from pigmentary disturbance. The details and treatment of these and other non-scarring sequelae are discussed.

Adult↗

The penetrating potential of autoantibodies into live cells in vitro coincides with the in vivo staining of epidermal nuclei.

We have previously demonstrated that IgG autoantibodies derived from SLE patients are capable of penetrating into nuclei of living COLO-16 cells, in vitro. To address the possible correlation in Lupus Erythematosus (LE) between the in vivo ANA binding to nuclei of epidermal cells and the presence of intranuclear penetrating antibodies in sera of those patients, 25 consecutive patients were studied. Out of 25 skin biopsies, 11 specimens (8 of SLE and 3 of DLE) showed by immunofluorescent microscopy extensive in vivo presence of IgG in epidermal nuclei, whereas all sera of these patients stained nuclei of living COLO-16 cells, in vitro. Such penetration was also observed in additional 6/25 sera of patients, but with in vivo negative biopsies. This in vitro nuclear binding, which was unrelated to clinical symptoms of patients or their serological autoantibody profile and titer, was reproduced following cross-linking of intracellular protein by PLP fixation. Likewise, western blotting (immunoblotting) analysis, demonstrated the intranuclear presence of IgG in all in vitro intranuclear IgG staining sera. Furthermore, this in vitro presence, which neither affects cell viability nor DNA synthesis, is time-dependent and of a transient nature: nuclear staining disappears within 48 h following removal of the penetrating sera from medium. In conclusion, since the COLO-16 in vitro assay mirrors exactly the in vivo situation, and because of its higher sensitivity, it provides an excellent tool for the study of non-degraded autoantibody penetration into the nuclei of living cells.

Adolescent↗

Cutaneous lupus erythematosus during the neonatal and childhood periods.

Cutaneous lupus erythematosus may be related to maternal autoantibody production in the neonatal lupus syndrome, or may occur later in childhood, with or without findings of systemic lupus erythematosus (SLE). In the first section, we will discuss the transient, passively transferred neonatal disease, and in the second, we will discuss the persistent, actively acquired forms of lupus erythematosus in childhood.

Child↗

Update on therapy--thalidomide in the treatment of lupus.

Thalidomide has been shown to be an effective treatment for cutaneous forms of lupus erythematous refractory to other therapies. Thalidomide has very serious side effects, including teratogenicity and neuropathy, which limit its clinical use in lupus to such severe refractory cases. Efficacy has been confirmed in several studies, although recurrence after discontinuation of treatment is frequent. More recent experience suggests that lower doses than originally used may be effective, which may result in a reduction in side effects. Much effort has been expended in studying the mechanisms of action of thalidomide, although as yet it is unclear which of the mechanisms identified to date contribute to its efficacy in treating cutaneous forms of lupus erythematosus. Identification of patients suitable for thalidomide therapy requires a rigorous selection process. Potential side effects should be clearly explained, particularly teratogenicity as many patients are young women. Written consent and a negative pregnancy test must be obtained prior to commencement of therapy. Reliable contraceptive measures should be strictly observed by patients taking thalidomide. Close clinical and neurophysiological supervision using nerve conduction studies should be undertaken.

Humans↗

MALToma and lupus.

We present two patients with low-grade non-Hodgkin MALT lymphoma (mucosa-associated lymphoid tissue) occurring years before clinical evidence of autoimmune disorders. While the strong association between autoimmune disorders, in particular Sjögren's syndrome and MALToma, is well described, the developement of MALToma years before the onset of an autoimmune disease is very unusual.

Adult↗

Direct immunofluorescence in lupus erythematosus (LE).

One hundred and twenty-six patients with LE were studied. They were distributed as follows: 84 with DLE, 13 with SALE and 29 with SLE. Biopsies from the skin lesions were performed and submitted to DIF. Positive results were equal to 69, 61.5 and 72.4 percent of the DLE, SALE and SLE cases, respectively. These data are in accordance with the literature. IgM was the most frequently found immunoglobulin, followed by the association IgM + C3.

Adolescent↗

Langerhans cells in connective tissue diseases.

We have conducted a quantitative analysis of Langerhans cells (LC) in skin biopsies of 20 patients with various connective tissue diseases. Clinically normal skin of SLE patients as well as lesional skin of DLE showed consistently normal LC densities as assessed using ATPase staining, anti-DR and anti-OKT6. Examination of LC in clinically involved skin of patients with scleroderma revealed an absolute or relative decrease in ATPase and OKT6 expression, while staining with anti-DR gave inconclusive results. Clinically normal skin of the same individuals showed basically normal LC density. These findings suggest that the perturbation of the LC population is probably an expression of a secondary local phenomenon, and does not reflect a more widespread derangement of the accessory cells in the skin.

Adenosine Triphosphatases↗