Interventions for bullous pemphigoid

Kirtschig, Gudula; Middleton, Philippa; Bennett, Cathy; Murrell, Dédée F.; Wojnarowska, Fenella; Khumalo, Nonhlanhla P.

doi:10.1002/14651858.cd002292.pub3

Cited by 144 publications

(166 citation statements)

References 46 publications

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“…Another study suggested a correlation between the dosage of prednisone at discharge and the mortality rate [19]. In our study, prednisone dosage at start of therapy did not affect survival at 1 and at 5 years, most likely because patients were almost invariably treated with prednisone doses below 0.75 mg/kg daily, a range which appears to be better tolerated and associated with a mortality risk similar to that of potent topical steroids [23,38]. In contrast, we found that use of chlorambucil at an initial dose of 6 mg daily was a poor prognostic factor.…”

Section: Discussionmentioning

confidence: 49%

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

et al. 2012

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Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors. Methods: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil. Results: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis. Conclusion: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients.

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Section: Discussionmentioning

confidence: 49%

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

et al. 2012

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“…These drugs have been used successfully in various inflammatory disorders, including the autoimmune diseases rheumatoid arthritis and relapsing polychondritis (27). In fact, IL-1b inhibition would be the first therapeutic regimen without unspecific immunosuppressive/ immunomodulative action for AIBDs, such as EBA and bullous pemphigoid (1,28).…”

Section: Discussionmentioning

confidence: 99%

Caspase-1–Independent IL-1 Release Mediates Blister Formation in Autoantibody-Induced Tissue Injury through Modulation of Endothelial Adhesion Molecules

Sadeghi

Lockmann

Hund

et al. 2015

The Journal of Immunology

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Although reports documented aberrant cytokine expression in autoimmune bullous dermatoses (AIBDs), cytokine-targeting therapies have not been established in these disorders. We showed previously that IL-6 treatment protected against tissue destruction in experimental epidermolysis bullosa acquisita (EBA), an AIBD caused by autoantibodies to type VII collagen (COL7). The anti-inflammatory effects of IL-6 were mediated by induction of IL-1ra, and prophylactic IL-1ra administration prevented blistering. In this article, we demonstrate elevated serum concentrations of IL-1β in both mice with experimental EBA induced by injection of anti-COL7 IgG and in EBA patients. Increased IL-1α and IL-1β expression also was observed in the skin of anti-COL7 IgG-injected wild-type mice compared with the significantly less diseased IL-1R–deficient or wild-type mice treated with the IL-1R antagonist anakinra or anti–IL-1β. These findings suggested that IL-1 contributed to recruitment of inflammatory cells into the skin. Accordingly, the expression of ICAM-1 was decreased in IL-1R–deficient and anakinra-treated mice injected with anti-COL7. This effect appeared to be specifically attributable to IL-1 because anakinra blocked the upregulation of different endothelial adhesion molecules on IL-1–stimulated, but not on TNF-α–stimulated, cultured endothelial cells. Interestingly, injection of caspase-1/11–deficient mice with anti-COL7 IgG led to the same extent of skin lesions as in wild-type mice. Collectively, our data suggest that IL-1, independently of caspase-1, contributes to the pathogenesis of EBA. Because anti–IL-1β in a prophylactic setting and anakinra in a quasi-therapeutic setting (i.e., when skin lesions had already developed) improved experimental EBA, IL-1 appears to be a potential therapeutic target for EBA and related AIBDs.

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“…14 The results of several studies concerning the treatment of bullous pemphigoid with azathioprine are summarized in Table 1.…”

Section: Azathioprine In Bullous Pemphigoidmentioning

confidence: 99%