Table A2.
Study | Type of Study | Number of Patients | Dose Used | Previous Treatments | Results Obtained | Side Effects | Observations | Level of Evidence |
---|---|---|---|---|---|---|---|---|
“Cao P et al. (2022)” [23] | Systematic review 75 studies included | 211 in total, 36 treated with dupilumab, 53 with omalizumab and 122 with RTX | NE | CTC, MTX, MMF, AZA, CLP, CFF | Total remission 66.7% (24/36) Partial remission 19.4% (7/36) at 4.5 months |
Recurrence 5.6% (2/36). No adverse effects | The rest of the patients included in the study (122) were treated with RTX, giving a higher number of recurrences, AEs and mortality | 2a |
“Russo R et al. (2022)” [47] | Literary review of 9 articles | 30 (16 M, 14 F), average age 69.85 years | NE | CTC, I.S. | Decrease in Th2 lymphocytes and improvement in pruritus | None, no interaction with other drugs | Some comorbidities: TB, melanoma, cancer, obesity, DM… | 2a |
“Zhao L et al. (2023)” [48] | Retrospective cohort study | 146, average age 73 years, 86% M | 300 mg every 2 weeks after an initial dose of 600 mg | NE | 127 (87%) BP control in 1 month | Injection site injuries did not require suspending dupilumab | 3 pctes pneumonia that improved with ATB without needing to suspend dupilumab (pneumonia associated with comorbidities) | 2b4 |
“Zhang Y et al. (2021)” [49] | Retrospective study | 24, average age 64.50 years (8 treatment with dupilumab + AZA + MTP and 16 AZA + MTP) | 600 mg initially, followed by 300 mg weekly | MTP, AZA | Complete remission 62.5%, partial remission 12.5% | Eosinophilia, recurrence 12.5% | Add dupilumab to MTP + AZA + effective than without dupilumab | 4 |
“Liang J et al. (2023)” [50] | Number of cases | 9, average age 68 years | NE | CTC | Total remission: 74.6% Partial remission 11.1% |
NE | NP | 4 |
“Abdat R et al. (2020)” [12] | Case series from 5 academic centers | 13, average age 76.8 years | NE | NE | Total remission: 53.8%, response to treatment 92.3% | None | NP | 4 |
“Yan T et al. (2023)” [18] | Retrospective cohorts | 40 (20 treated with dupilumab and another 20 with dupilumab + CTC) | 600 mg initially followed by 300 mg weekly | CTC | Of the 20 treated only with dupilumab: 12 had complete remission, 8 had partial remission after 6 months of treatment. | NE | dupilumab improves AP symptoms, but fails to reduce BP180 levels | 2b |
“Oren-Shabtai M et al. (2023)” [22] | Series of 9 cases | 9, 1 of them treated with dupilumab, 3 with omalizumab and 7 with RTX average age 60.4 years | 600 mg initially, followed by 300 mg weekly | CTC, BIO, RTX | 78% clinical improvement, 55% complete remission | None | NP | 4 |
“Learned C et al. (2023)” [51] | Retrospective study | 17 (10 M and 7 F), average age 72.2 years | 300 mg weekly | MMF, DOX, CTC IVIg | 14 patients had complete remission, 2 had partial remission and 1 had significant improvement. | None | The patients included had tried 4 lines of treatment prior to dupilumab | 4 |
“Seyed J et al. (2020)” [25] | Description of a case | 1, M 70 years | 600 mg initially, followed by 300 mg weekly | CTC, DAP, MTX, MMF, omalizumab | Disappearance of pruritus, VAS scale 0/10, complete remission in AOM association | NE | Complete remission with omalizumab + dupilumab after trying various treatments. Pcte with metabolic ds | 4 |
“Velin M et al. (2022)” [27] | Retrospective study | 112 (19 met inclusion criteria) | 300 mg every 2 weeks | CTC, MTX | 60% complete remission, 20% partial remission | Only 1 percent in treatment with dupilumab skin burning sensation, only lasted 1 month with dupilumab | Of the 19 patients, 12 received MTX, 7 omalizumab and 8 dupilumab | 4 |
“Hu L et al. (2023)” [52] | Retrospective study | 11, average age 76 years, 4M, 7F | 600 mg followed by 300 mg every 2 weeks | I.S., M.C. | In 2 weeks 10/11 patients control the disease | None | NP | 4 |
“Qi W et al. (2023)” [53] | Compare 2 groups | 27 (9 received MTP + dupilumab), 18 only MTP, mean age 72 years | NE | MTP | Improvement of the disease in patients treated with MTP + dupilumab | None with dupilumab | NP | 4 |
“Klepper EM et al. (2021)” [54] | Case report | 1, F 79 years | 600 mg initially, followed by 300 mg weekly | CTC, DAP, DOX | After 1 month of treatment with dupilumab, 100% reduction in itching | NE | dupilumab indicated in people over 6 years of age | 4 |
“Yang J et al. (2022)” [55] | Retrospective cohort study | 40 (20 MTP only, 20 MTP + dupilumab) | 600 mg initially, followed by 300 mg weekly | MTP | Greater control of BP, pruritus and quality of life in MTP + dupilumab | Eosinophilia, thrombosis in 2 patients (1 from each group), PE, gastritis, pneumonia, herpes zoster | NP | 4 |
“Foerster Y et al. (2023)” [56] | Report of 3 patients, only 1 treatment with dupilumab | 3 patients with BP + HIV-1, patients treated with dupilumab M aged 60 years | 600 mg initially, then 300 mg every 2 weeks | CTC, AZA, DAP, DOX + antiretroviral treatment | Disappearance of itching and blisters | NE | Of the 3 exposed cases, only 1 was treated with dupilumab | 4 |
“Zhang X et al. (2023)” [57] | Retrospective study | 7 | 600 mg initially, followed by 300 mg weekly for 16 weeks | CTC, OMZ, tofacinib, CLP | 6/7 complete remission 1/7 partial improvement | None | NP | 4 |
“Sanfilippo E et al. (2023)” [58] | Report of 1 case | 1, M 80 years | NE | CTC | Improvement of itching and disappearance of blisters | NE | Background: AF, HF, T2DM, HTN, prostate cancer, stroke | 4 |
“Takamura S et al. (2022)” [59] | Presentation of 1 case | 1, F 72 years | NE | NE | Improves pruritus, blisters and anti-BP180 negativity | NE | NP | 4 |
“Wang Q et al. (2023)” [60] | Presentation of 1 case | 1, M 60 years | 600 mg initially, followed by 300 mg weekly | CTC, MTX | Improvement of itching in 3 days and blisters in 2 weeks, disappearance of Ig in 6 weeks | NE | Tto for 10 weeks after induction: CTC + dupilumab without AP recurrences | 4 |
“Wang SH et al. (2023)” [61] | Number of cases | 10 (7M, 3F) mean age 72.7 | Initially 600 mg, then 300 mg every 2 weeks | MTP, MC, AH, IVIg | 90% improvement in pruritus, complete remission 70%, average duration 8.3 weeks | Eosinophilia in 2 cases that was resolved with IS | Multiple comorbidities: DM, allergic rhinitis, osteoporosis, CMV infection, pneumocystis pneumonia | 4 |
“Wang M et al. (2022)” [62] | Presentation of 2 cases | 2 | 1. 300 mg dupilumab twice 2. 300 mg dupilumab twice |
1. MTP + MTX 2. MTP |
1. pruritus improvement in 2 weeks 2. lesion remission in 2 weeks |
None | dupilumab prevents complications from other treatments such as RTX (infections and heart disease) | 4 |
“Bruni M et al. (2022)” [15] | Case study | 1, M 76 years | 300 mg dupilumab | MTP, DOX | Complete remission in 6 months | NE | BP triggered by nivolumab for treatment of lung metastases due to melanoma |
4 |
“Liu JH et al. (2023)” [63] | Case study | 1, M 73 years old | 600 mg subcutaneously, followed by 300 mg subcutaneously | DOX, CTC | Disappearance of PA lesions and psoriasis after 16 days of treatment with dupilumab. No relapses | NE | Effective treatment with dupilumab for psoriasis + BP | 4 |
“Manzo Margiotta F et al. (2023)” [64] | Presentation of a case | 1, M 74 years | 600 mg followed by 300 mg every 2 weeks | CTC, DOX, NT, DAP | Resolution of blisters and itching after 16 weeks of treatment | None | NP | 4 |
“Valenti M et al. (2022)” [65] | Case report | 1 | 600 mg followed by 300 mg every 2 weeks | MTP, AZA, DAP, CH | After 3 months anti BP230 normal levels | NE | NP | 4 |
“Savoldy MA et al. (2022)” [66] | Case study | 1, M 78 years old | 300 mg every 2 weeks | CTC, DOX, IS | Improvement after 6 weeks | None | AP triggered after COVID-19 vaccination | 4 |
“Zhou AE et al. (2022)” [67] | Presentation of a case | 1, F 17 years old | 300 mg | CTC, RTX, IVIg | Complete resolution after 4 weeks, improvement after 2 weeks of starting dupilumab | NE, no relapses | Young woman (17 years old) with BP, not AF | 4 |
“Pop SR et al. (2022)” [16] | Presentation of a case | 1, F 59 years | 300 mg dupilumab + CTC treatment |
CTC, DOX, NT, DAP, MMF | Improvement of blisters | NE, CTC could be suspended without regrowth, leaving only dupilumab | BP induced by pembrolizumab for cervical cancer treatment. | 4 |
“Riqueleme- Mc Loughlin et al. (2021)” [68] | Presentation of a case | 1, F 37 years | 600 mg at 30 weeks, followed by 300 mg at 2 weeks | CTC | Itching and blisters improve, fetus birth without incidents | PROM at 34.4 weeks, birth by cesarean section | Case of gestational AP + frequent in 2nd and 3rd trimester | 4 |
“Zhang Y et al. (2021)” [69] | Presentation of a case | 1, F 61 years | 600 mg followed by 300 mg | CTC, AZA | Disappearance of itching after a month, no formation of new blisters | NE | NP | 4 |
“Kaye A et al. (2018)” [70] | Presentation of a case | 1, M 80 years | 600 mg followed by 300 mg | CTC | Full resolution at 3 m and normalization levels BP180 and BP230 | NE | TB infection and HBsAg + contraindicated immunosuppressive treatment | 4 |
“Jendoubi F et al. (2022)” [71] | Presentation of a case | 1, F 76 years | 600 mg followed by 300 mg every 2 weeks | CTC | Complete resolution of pruritus and blisters, without recurrence after 6 months | None | Pcte with nodular pemphigus (BP variant) | 4 |
“Fournier C et al. (2023)” [72] | Presentation of 3 cases | 1, M 74 years old | 600 mg followed by 300 mg | CTC | Complete remission | NE | Development of BP following treatment with nivolumab for melanoma | 4 |
“Huand D et al. (2023)” [19] | Retrospective cohort study | 36 patients, 20 receive MTP, 16 dupilumab +MTP, average age 71 years | 600 mg of dupilumab followed by 300 mg athenext week | NE | Pruritus decrease and BPDAI scale improvement + effective with MTP + dupilumab at 2 weeks | 2 cases dermatitis at injection site, 3 transient hyperglycemia, 4 hypereosinophilia | MTP + dupilumab group, MTP is suspended and 300 mg 2/wk dupilumab is continued as monotherapy. | 4 |
“Chen J et al. (2023)” [73] | Presentation of 2 cases | 2, M, 66 and 79 years | 600 mg initially, followed by 300 mg weekly for 16 weeks | NE | Clinical improvement without relapses | One of them had erythema at the injection site, which resolved itself. | Comorbidities: DM, asthma, HTN | 4 |
AF: family history, HBsAg: hepatitis B surface antigen, AH: antihistamines, ATB: antibiotic, AZA: azathioprine, BIO: biological, BPDAI: bullous pemphigoid disease area index, CFF: cyclophosphamide, CH: colchicine, IC: contraindication, CLP: cyclosporine, CMV: cytomegalovirus, CTC: corticosteroids, DAP: dapsone, DM: diabetes mellitus, DOX: doxycycline, dupilumab dupilumab, CKD: chronic kidney disease, VAS: visual analog pain scale, F: woman, AF: atrial fibrillation, HTN: arterial hypertension, IC: heart failure, IDPP4: dipeptidyl peptidase 4 inhibitors, IF: immunofluorescence, Ig: immunoglobulins, IVIg: intravenous Ig, IM: immunomodulators, IR: renal failure, IS: immunosuppressants, PML: progressive multifocal leukoencephalopathy, M: male, MC: minocycline, MG: milligrams, MMF: mycophenolate mofetil, MTP: methylprednisolone, MTX: methotrexate, No.: number, NE: not specified, NP: not applicable, NT: nicotinamide, AOM: omalizumab, BP: bullous pemphigoid, PCTE: patient, QT: chemotherapy, PROM: premature rupture of membranes, RTX: rituximab, SB: subcutaneous, SD: syndrome, SEM: week, OS: week of gestation, TBC: tuberculosis, TC: tetracyclines, PET: pulmonary thromboembolism, TTO: treatment, HIV: human immunodeficiency virus.