Management of pemphigus disease in pregnancy

Abstract

Pemphigus can cause complications during pregnancy and may cause serious harm to a fetus. For this study, a comprehensive review of common treatments of pemphigus and their adverse effects associated with pregnancy and male fertility was conducted. We concluded that a period of remission with minimal or no therapy before conception could significantly reduce the risk of the disease flaring up, at least in the first trimester. The period of remission causes a delay in the flare-up of the disease, which means lower cumulative doses and the prevention of possible congenital abnormalities caused by corticosteroid or immunosuppressant treatments. All common treatments of pemphigus-azathioprine, mycophenolate mofetil, and methotrexate-should be avoided during pregnancy. However, it appears that systemic corticosteroids in a safe dose with a topical form of corticosteroids may be used without serious risk. Due to the lack of data associated with rituximab therapy, it is recommended that this drug be avoided 12 months before conception. It appears that the safest treatment of pemphigus is intravenous immunoglobulin (IVIg), which may be more effective when used with topical corticosteroids. Due to the delayed effect of IVIg, it should be used some months prior to conception.

Emerging treatment options for the management of pemphigus vulgaris

“…Due to evidence of teratogenic risk, azathioprine use in pregnancy is not recommended; the same recommendation applies for breastfeeding because minimal concentrations of azathioprine and its metabolites have been found in breast milk. 29 , 40 …”

“… 31 , 35 The use of MMF during pregnancy is associated with increased risk of spontaneous abortion and congenital malformations. 40 …”

Current Therapy in Autoimmune Bullous Diseases

“…Severe disease can be treated with intravenous immunoglobulin in a conventional dose. The safety profiles of intravenous immunoglobulin in pregnant women were confirmed by several clinical studies in pregnant women with pemphigus [38,62]. Another choice of treating severe disease and unresponsive disease to conventional treatment is immunoadsorption [70].…”

“…Methotrexate is contraindicated in pregnancy due to its teratogenic and abortifacient properties. Moreover, it causes reproductive toxicity and decreases the sperm account [38]. Mucocutaneous toxicity occurs more commonly in patients without adequate folic acid supplementation.…”

“…The intravenous immunoglobulin preparations are safe and effective and can treat juvenile autoimmune bullous diseases including pemphigus [61]. Several studies reported good safe and efficacy profiles also in pregnancy [38,62].…”