Cyclosporine Treatment in Severe Gestational Pemphigoid

Huilaja, Laura; Mäkikallio, Kaarin; Hannula-Jouppi, Katariina; Väkevä, Liisa; Höök‐Nikanne, Johanna; Tasanen, Kaisa

doi:10.2340/00015555-2032

Cited by 19 publications

(12 citation statements)

References 27 publications

(42 reference statements)

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“…Azathioprine and dapsone were the second and third most commonly prescribed steroid-sparing medications, always given after delivery/abortion. On the other hand, cyclosporine, which was the fourth most frequently administered steroid-sparing drug, was successfully used also during pregnancy (14,15). Interestingly, rituximab was used in two persistent cases either during pregnancy to prevent disease flare (16) and after delivery (17).…”

Section: Evidence About Pemphigoid Gestationis Treatment Emerged Frommentioning

confidence: 99%

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

et al. 2020

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Background: Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series.Objectives: To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management.Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020.Results: In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality (n = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%).Limitations: The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias.Conclusion: Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.

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Section: Evidence About Pemphigoid Gestationis Treatment Emerged Frommentioning

confidence: 99%

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

et al. 2020

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“…In the mild form of PV disease, the topical steroid administration can be very a useful and safe therapeutic option. Even though the treatment of severe form of PV disease is reduced, it is very difficult because of immunosuppressive side effects medication on fetus and newborn [9].…”

Section: Discussionmentioning

confidence: 99%

Management of Pemphigus Vulgaris in Pregnancy - Case Report

2019

JGRM

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Pemphigus vulgaris is a rare autoimmune bullous disease caused by producing autoantibodies directed against transmembrane glycoproteins of desmosomes that can lead to steric hindrance to homophilic adhesion of desmogleins. The development of pemphigus during the pregnancy is rare. The disruption of the Th1:Th2 balance in the autoimmune disease during the pregnancy plays a crucial role and explains why some studies have reported the exacerbation of disease, while others observe improvements during the pregnancy. We present aclinical case of a 31-year-oldprimigravida, with dichorionic - diamniotic twin pregnancyassociatedwith pemphigus vulgaris, being discovered 2 years ago, who went under treatment with Medrol 64mg/day and Imuran 50mg/day. She was admitted to the hospital at 31 weeks of gestation with non-systemic contractions of moderate intensity. The clinical findings are multiple erosions and blisters which have been accentuated during the second semester and have been remitted after delivery. Nowadays, the therapeutic management of the pemphigus during the pregnancy is not yet established and all of these cases are individual evaluated with the primary target to control the disease with the safety of the fetus.

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“…Prenatal treatment with cyclosporine combined to prednisolone has been reported in four cases with good treatment response, and in one case, cyclosporine was used after intravenous immunoglobulin in persistent postnatal PG [6][7][8]. Our patients were treated with cyclosporine (300 mg/day) and prednisolone.…”

Section: Discussionmentioning

confidence: 99%

“…Most PG patients can be successfully treated with topical and oral glucocorticoids, which are considered relatively safe during pregnancy. Severe PG cases with immunosuppressive medication are sparse, and the benefi t to the mother needs to be weighed against possible risks to the fetus and the newborn [6].…”

Section: Discussionmentioning

confidence: 99%

A resistant case of pemphigus gestationis successfully treated with cyclosporine

Özdemir

Atalay

Asgarova

et al. 2016

IMAS

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Pemphigoid gestationis (PG) is a rare autoimmune blistering disease of pregnancy caused by antibasement membrane zone autoantibodies. The usual clinical fi ndings are multiple pruritic urticarial papules and plaques, target lesions, vesicles, and blisters that occur during the second and third trimesters of pregnancy or in the immediate postpartum period. The disease is often treated with topical corticosteroids and oral antihistaminics. In more severe cases, systemic corticosteroids are needed. Herein, we report a case of resistant PG that responded to treatment with cyclosporine.

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Cyclosporine Treatment in Severe Gestational Pemphigoid

Cited by 19 publications

References 27 publications

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

Management of Pemphigus Vulgaris in Pregnancy - Case Report

A resistant case of pemphigus gestationis successfully treated with cyclosporine

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