{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T01:38:58Z","timestamp":1789609138034,"version":"build-2803163510"},"reference-count":33,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T00:00:00Z","timestamp":1597795200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Clinical Apheresis"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analyzed 17 patients with severe pemphigus who were unresponsive to high\u2010dose 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All the patients were diagnosed as severe pemphigus and had a period of at least 1 week of high\u2010dose prednisone (1\u20101.5 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 (<jats:italic>P<\/jats:italic>\u2009&lt;\u2009.001), Nikolsky's sign became negative and no new blisters appeared. The dosage of corticosteroid could begin to taper down rapidly in 9\u2009\u00b1\u20094\u2009days. On discharge, the dosage of prednisone decreased significantly (51\u2009\u00b1\u20093 mg\/day, <jats:italic>P<\/jats:italic>\u2009&lt;\u2009.001). No major adverse events happened that could lead to the termination of DFPP treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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\u2010related side effect.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jca.21829","type":"journal-article","created":{"date-parts":[[2020,8,27]],"date-time":"2020-08-27T21:20:21Z","timestamp":1598563221000},"page":"20-27","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Double\u2010filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10\u2009years' experience of a single center in China"],"prefix":"10.1002","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3767-9160","authenticated-orcid":false,"given":"Yan","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bingjie","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Dermatology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haiyun","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaohong","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ke","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Limeng","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuewang","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4729-6673","authenticated-orcid":false,"given":"Yan","family":"Qin","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuemei","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Nephrology Peking Union Medical College Hospital, Chinese Academy of Medical Sciences  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,8,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)66829-8"},{"issue":"10","key":"e_1_2_6_3_1","doi-asserted-by":"crossref","first-page":"844","DOI":"10.1111\/j.1610-0387.2011.07793.x","article-title":"Autoimmune bullous skin diseases. 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