Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

Higashihara, T; Kawase, Masaaki; Kobayashi, Motoyoshi; Hara, Mizuki; Matsuzaki, Hiroyuki; Uni, Rie; Matsumura, Mimiko; Etoh, Takafumi; Takano, Hideki

doi:10.1111/1744-9987.12557

Cited by 15 publications

(19 citation statements)

References 21 publications

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“…Double filtration plasmapheresis (DFPP) is a relatively new procedure that, similar to IA, removes selectively immunoglobulins, while minimizing the loss of albumin. In small case series and one retrospective study DFPP also demonstrated efficacy in drug resistant pemphigus (230–232).…”

Section: Treatmentmentioning

confidence: 98%

Pemphigus: Current and Future Therapeutic Strategies

et al. 2019

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Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients' quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon referred to as acantholysis. This in turn causes intra-epidermal blistering and the clinical appearance of flaccid blisters and erosions at involved sites. Since the advent of glucocorticoids, the overall prognosis of pemphigus has largely improved. However, mortality persists elevated, since long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants portend a high risk of serious adverse events, especially infections. Recently, rituximab, a chimeric anti CD20 monoclonal antibody which induces B-cell depletion, has been shown to improve patients' survival, as early rituximab use results in higher disease remission rates, long term clinical response and faster prednisone tapering compared to conventional immunosuppressive therapies, leading to its approval as a first line therapy in pemphigus. Other anti B-cell therapies targeting B-cell receptor or downstream molecules are currently tried in clinical studies. More intriguingly, a preliminary study in a preclinical mouse model of pemphigus has shown promise regarding future therapeutic application of Chimeric Autoantibody Receptor T-cells engineered using Dsg domains to selectively target autoreactive B-cells. Conversely, previous studies from our group have demonstrated that B-cell depletion in pemphigus resulted in secondary impairment of T-cell function; this may account for the observed long-term remission following B-cell recovery in rituximab treated patients. Likewise, our data support the critical role of Dsg-specific T-cell clones in orchestrating the inflammatory response and B-cell activation in pemphigus. Monitoring autoreactive T-cells in patients may indeed provide further information on the role of these cells, and would be the starting point for designating therapies aimed at restoring the lost immune tolerance against Dsg. The present review focuses on current advances, unmet challenges and future perspectives of pemphigus management.

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Section: Treatmentmentioning

confidence: 98%

Pemphigus: Current and Future Therapeutic Strategies

et al. 2019

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“…Nevertheless, there are other alternative methods of plasmapheresis, such as double filtration plasmapheresis (DFPP). DFPP is a semi‐selective method in which the patient's blood passes through a first filter (plasma separator), dissociating the formed elements of the blood and the plasma, which is then filtered by a second filter (plasma fractionator) permitting the extraction of the substance of interest according to its size 7 …”

mentioning

confidence: 99%

Use of double filtration plasmapheresis for the treatment of acquired thrombocytopenic thrombotic purpura

et al. 2020

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Double filtration plasmapheresis (DFPP) could be an alternative method to simple plasma exchange plasmapheresis in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP). In a retrospective single center case series, we studied clinical presentation, management care, and prognosis of aTTP patients from our academic center treated with DFPP and IV infusion of fresh frozen plasma (FFP) between 2009 and 2018. Nine patients were included for 11 episodes. Median age was 38 years old (IQR 26‐53) with 78% women. Six episodes (55%) required admission to the ICU, four of which required mechanical ventilation. Median FFP volume transfused was 35.2 mL/kg/d of session. Response was complete for nine episodes (82%). Four patients presented an early relapse, two a late relapse. Four patients died: one had an active untreated HCV infection, and two were over 80‐year‐old polymorbid patients. DFPP seems to be an efficient method of therapeutic plasmapheresis in TTP when combined with FFP transfusion and immunosuppressive treatments.

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“…Though several case reports or series also reported the capability of plasmapheresis in inducing remission in severe or refractory pemphigus patients, 19,20,22,[30][31][32] high quality evidence was still lacking. In the American Society for Apheresis guidelines updated in 2019, plasmapheresis was recommended as category III (optimum role of apheresis therapy is not established), Grade 2B (weak recommendation, low-quality or very low-quality evidence) with the indication of severe cases.…”

Section: Discussionmentioning

confidence: 99%

“…17 Since then, there have been quite a few researches in this field, but the results are controversial. [18][19][20] The utilized techniques of plasmapheresis are also different in these studies. 21,22 Double filtration plasmapheresis (DFPP) is superior to other techniques, because it can increase the selectivity of the molecular size.…”

Section: Introductionmentioning

confidence: 99%

Double‐filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China

Liang

Zhang

et al. 2020

J of Clinical Apheresis

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Background: Pemphigus is a group of rare and severe autoimmune blistering disease mediated by pathogenic autoantibodies against desmogleins. Plasmapheresis can directly remove autoantibodies from circulation, which has been applied to the treatment of pemphigus as an adjuvant therapy. But the results of the researches are controversial. This study aims to evaluate the efficacy and safety of double filtration plasmapheresis (DFPP) combined with immunosuppressive treatment for patients with severe pemphigus in our single center. Methods: We retrospectively analyzed 17 patients with severe pemphigus who were unresponsive to high-dose corticosteroid and received DFPP treatment between January 2010 and January 2020. The information on demographic characteristics, clinical and laboratory data, treatment regimens, and clinical outcomes were collected. Results: All the patients were diagnosed as severe pemphigus and had a period of at least 1 week of high-dose prednisone (1-1.5 mg/kg/day), but they were unresponsive to corticosteroid and immunosuppressants treatment. They received DFPP treatment as an adjuvant therapy. After DFPP treatment, the titers of desmogleins antibodies significantly decreased (P < .001), Nikolsky's sign became negative and no new blisters appeared. The dosage of corticosteroid could begin to taper down rapidly in 9 ± 4 days. On discharge, the dosage of prednisone decreased significantly (51 ± 3 mg/day, P < .001). No major adverse events happened that could lead to the termination of DFPP treatment. Conclusion: Double filtration plasmapheresis combined with immunosuppressive treatment is an effective and safe therapeutic regimen for severe pemphigus. DFPP can also contribute to the dosage reduction of steroid to avoid more drug-related side effect.

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Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

Cited by 15 publications

References 21 publications

Pemphigus: Current and Future Therapeutic Strategies

Pemphigus: Current and Future Therapeutic Strategies

Use of double filtration plasmapheresis for the treatment of acquired thrombocytopenic thrombotic purpura

Double‐filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China

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