Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Lim, Yen Loo; Bohelay, G.; Hanakawa, Sho; Musette, Philippe; Janela, Baptiste

doi:10.3389/fmolb.2021.808536

Cited by 22 publications

(26 citation statements)

References 372 publications

(429 reference statements)

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“…Besides, a second cycle of RTX was systematically proposed 4 to 6 months after baseline in non-CR patients to achieve CR and 6 months after baseline in CR patients to consolidate the remission in the current series. These parameters might explain why the CR rate after a unique cycle was 68% in the previous study and only 26.6% in the current one. The proportion of responders (95.4%) is notably much higher than that reported in the study by Lamberts et al (57.1%) which applied a similar regimen with RTX injections at baseline, 6 and 12 months, but with lower dosage (500 mg) of RTX after baseline (28).…”

Section: Outcomes Of Patients With Mmp Treated With Rtx and Factors A...mentioning

confidence: 48%

“…RTX is a murine-human chimeric monoclonal antibody directed against CD20, a cell surface marker expressed by B cells after the late pre B-cell stage (except plasma cells), and is responsible for prolonged B-cell depletion followed by a 6-month delayed recovery period (19,20). Following clinical trials, metaanalyses, and a prospective multicenter randomized trial (21)(22)(23)(24), RTX is now recommended in first line therapy for moderate to severe pemphigus in combination with oral corticosteroids (25,26). RTX has also been used off-label, alone or in combination therapies, with success in a wide range of other refractory AIBDs, including MMP (10,11,27,28).…”

Section: Discussionmentioning

confidence: 99%

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Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients

et al. 2022

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Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, chronic, subepithelial autoimmune blistering diseases (AIBDs) with predominant involvement of mucous membranes that can be sight-threatening and life-threatening. Rituximab (RTX) has demonstrated its efficacy in severe MMP refractory to conventional immunosuppressants in small series that differed in RTX scheme, concomitant therapies, and outcome definitions. In a meta-analysis involving 112 patients with MMP treated with RTX, complete remission (CR) was reported in 70.5% of cases. Herein, we report the largest retrospective monocentric study on RTX efficacy in a series of 109 severe and/or refractory patients with MMP treated with RTX with a median follow-up period of 51.4 months. RTX was administered in association with immunomodulatory drugs (dapsone, salazopyrine) without any other systemic immunosuppressant in 104 patients. The RTX schedule comprised two injections (1 g, 2 weeks apart), repeated every 6 months until CR or failure, with a unique consolidation injection (1 g) after CR. The median survival times to disease control and to CR were 7.1 months and 12.2 months, respectively. The median number of RTX cycles required to achieve CR in 85.3% of patients was two. The larynx was the lesional site that took the longest time to achieve disease control. One year after RTX weaning, CR off RTX was obtained in 68.7% of cases. CR off RTX with only minimum doses of immunomodulatory drugs was achieved in 22.0% of patients. Further, 10.1% of patients were partial responders and 4.6% were non-responders to RTX. Relapse occurred in 38.7% of cases, of whom 91.7% had achieved CR again at the last follow-up. In MMP, CR was achieved in a longer time and after more rituximab cycles than in pemphigus, especially for patients with MMP with anti-type VII collagen reactivity. RTX with concomitant immunomodulatory drugs was not responsible for an unusual proportion of adverse events. This large study confirms that RTX is an effective therapy in patients with severe and/or refractory MMP, corroborating previous findings regarding the effects of RTX on AIBDs such as pemphigus.

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Section: Outcomes Of Patients With Mmp Treated With Rtx and Factors A...mentioning

confidence: 48%

Section: Discussionmentioning

confidence: 99%

Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients

et al. 2022

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“…Pemphigus is an autoimmune condition that causes blistering on the skin and in mucous membranes, with varying degrees of severity (9). Pemphigus can be triggered by a variety of factors including environmental, genetic, or a vaccination (10). Pemphigus foliaceus is the least severe subtype, with patients presenting with painless sores on the scalp that spread throughout the body (9).…”

Section: Discussion Conclusionmentioning

confidence: 99%

Lisinopril Associated with Non-Pemphigus Bullae Lesions

Callahan¹,

Sehmi

Hostoffer

2022

Preprint

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Background: Angiotensin-converting enzyme inhibitors are first-line drugs for patients suffering from hypertension. These drugs have common adverse effects including bronchospasm, cough, and angioedema. Drug-induced bullous eruptions have been reported previously with numerous drugs including lisinopril, an angiotensin-converting enzyme inhibitor. Three patients of note have had a form of biopsy-proven pemphigus induced by the introduction of lisinopril or an increase in dosage. We report a case of lisinopril-induced bullae that is not pemphigus foliaceus or bullous pemphigoid verified by biopsy.Case Presentation: A 65-year-old male presented with a blistering rash over his left flank, belly button and with a cluster on his ankle. His medical history is significant for hypertension, which was controlled by 20mg of lisinopril, which had been increased to 40mg in order to provide better control. A skin biopsy was done on one of the rashes with a histological report of a bullous reaction with a low possibility of bullous pemphigoid and probably caused by arthropod bite or drug hypersensitivity. Within a month of dosage change the patient started developing bullae across his body, with no relief from oral steroid administration. The patient was taken off lisinopril and within days the bullae started to heal and there were no new eruptions. Conclusion: Pemphigus is an autoimmune condition that causes blistering of the skin and mucous membranes and can be triggered by the environment, genetics or vaccination. Three patients have been reported to have a biopsy-proven pemphigus reaction caused by the introduction of lisinopril or an increase in dosage. These blisters were classified as pemphigus after biopsy and pathological analysis. These cases were resolved with removal of the medication from their regime along with topical creams to aid in healing. There is no known study published of a lisinopril-induced non-pemphigus bullae which our patient presented with after the doubling of his lisinopril dose and a pathology report stating a low likelihood of bullous pemphigoid.

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“…Pemphigus Foliaceus (PF) is a rare autoimmune disorder of the skin. 1 In PF, the production of autoantibodies (Abs) against desmoglein (Dsg) 1 leads to a clinical phenotype characterized by fragile blisters that involve the skin and spare the mucous membranes. 1 Several factors including genetic susceptibility, environment, malignant disorders, drugs intake and vaccines can provoke pemphigus.…”

Section: Figurementioning

confidence: 99%