Effects of Cyclosporin on Bullous Pemphigoid and Pemphigus

Thivolet, J; Barthélémy, H; Rigot‐Muller, G.; Bendelac, Albert

doi:10.1016/s0140-6736(85)91099-2

Cited by 123 publications

(35 citation statements)

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“…Renal and hepatic cytotoxicity could be reduced by the use of lower dosages, such as 3-6 mg/kg/day, as now recommended in other autoimmune disorders [20][21][22], Such findings prompt us to propose the use ofciclosporin together with a moderate dose regimen of prednisone as primary thera peutic scheme inall clinical subsetsof EBA. However, fur ther studies are required to support such a hypothesis.…”

Section: Discussionmentioning

confidence: 99%

Intractable Epidermolysis bullosa acquisita: Efficacy of Ciclosporin A

et al. 1990

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We report the case of a 66-year-old man who presented with a spontaneous blistering involving both skin and mucous membranes diagnosed as an inflammatory form of epidermolysis bullosa acquisita. During a 5-year course the condition failed to respond to various therapies. Finally, only ciclosporin, together with a low dose regimen of prednisone, achieved a dramatic improvement with a total clearing within 6 months, persisting 1½ years after discontinuation. Such results prompt us to propose such an association as a primary therapeutic scheme in an otherwise barely tractable disease.

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Section: Discussionmentioning

confidence: 99%

Intractable Epidermolysis bullosa acquisita: Efficacy of Ciclosporin A

et al. 1990

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“…To date, very little is known on the effect of cyclosporin A on autoantibody production in human autoimmune diseases. Disappearance of anti-skin antibodies has been reported in patients suffering from bullous pemphigoid (48). By contrast, no inhibition of anti-double-stranded and antinuclear DNA antibodies has been reported in systemic lupus erythematosus over a 6-mo follow-up period (49).…”

Section: Discussionmentioning

confidence: 99%

Effect of cyclosporin A treatment on the production of antibody in insulin-dependent (type I) diabetic patients.

Boîtard

Feutren²,

Castano³

et al. 1987

J. Clin. Invest.

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Anti-islet cell and anti-insulin antibody production was studied over a 12-mo period in 82 recently diagnosed diabetics randomly receiving either cyclosporin or placebo. Cyclosporin had only minimal effects on the production of anti-islet cell antibodies whether directed to islet cytoplasmic (immunofluorescence) or membrane (cytotoxicity assay) antigens even in patients undergoing remission. These data suggest that these antibodies do not play a major role in the pathogenesis of the disease particularly since their (irregular) presence is not predictive of the clinical response to cyclosporin. Conversely, cyclosporin completely suppressed the synthesis of antibodies elicited by exogenous insulin irrespective of the insulin doses received, and decreased the autoantibody production against thyroid antigens, indicating that cyclosporin has variable effects on antibody production against various antigens.

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“…To further investigate the pathogenic role of CD4 + T cells in the Rag-2 2/2 /COL17-humanized recipients, we examined the efficacy of CsA (19)(20)(21)(22). Approximately 35 mg/kg of CsA dissolved in olive oil (n = 5) or a control vehicle (n = 5) was i.p.…”

Section: Col17-humanized Recipientsmentioning

confidence: 99%

A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen

Ujiie

Shibaki

Nishie

et al. 2010

The Journal of Immunology

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Bullous pemphigoid (BP), the most common autoimmune blistering disease, is caused by autoantibodies against type XVII collagen (COL17). To establish an active stable BP animal model that demonstrates the persistent inflammatory skin lesions initiated by the anti-human COL17 Abs, we used COL17-humanized (COL17m−/−,h+) mice that we recently produced. First, we generated immunodeficient Rag-2−/−/COL17–humanized mice by crossing Rag-2−/− mice with COL17-humanized mice. Then, splenocytes from wild-type mice that had been immunized by grafting of human COL17-transgenic mouse skin were transferred into Rag-2−/−/COL17–humanized mice. The recipient mice continuously produced anti-human COL17 IgG Abs in vivo and developed blisters and erosions corresponding to clinical, histological, and immunopathological features of BP, although eosinophil infiltration, one of the characteristic histological findings observed in BP patients, was not detected in the recipients. Although the depletion of CD8+ T cells from the immunized splenocytes was found to produce no effects in the recipients, the depletion of CD4+ T cells as well as CD45R+ B cells was found to inhibit the production of anti-human COL17 IgG Abs in the recipients, resulting in no apparent clinical phenotype. Furthermore, we demonstrated that cyclosporin A significantly suppressed the production of anti-human COL17 IgG Abs and prevented the development of the BP phenotype in the treated recipients. Although this model in an immunodeficient mouse does not exactly reproduce the induction mechanism of BP in human patients, this unique experimental system targeting humanized pathogenic Ag allows us to investigate ongoing autoimmune responses to human molecules in experimental animal models.

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Effects of Cyclosporin on Bullous Pemphigoid and Pemphigus

Cited by 123 publications

References 1 publication

Intractable Epidermolysis bullosa acquisita: Efficacy of Ciclosporin A

Intractable Epidermolysis bullosa acquisita: Efficacy of Ciclosporin A

Effect of cyclosporin A treatment on the production of antibody in insulin-dependent (type I) diabetic patients.

A Novel Active Mouse Model for Bullous Pemphigoid Targeting Humanized Pathogenic Antigen

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