{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T10:57:40Z","timestamp":1777114660218,"version":"3.51.4"},"reference-count":11,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T00:00:00Z","timestamp":1770249600000},"content-version":"vor","delay-in-days":4,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T00:00:00Z","timestamp":1769904000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Immunoglobulin replacement therapy (IRT) for primary immunodeficiency reduces infection risk and subsequent complications and can be lifesaving. However, IRT can cause severe systemic adverse events (AEs) that may limit adequate dosing. These AEs may be caused, in part, by activation and\/or consumption of complement proteins, thereby lowering C1 esterase inhibitor (C1\u2010INH) levels. Data suggest that C1\u2010INH administration prior to intravenous immunoglobulin (IVIG) may reduce IVIG\u2010related AEs. This case describes an adult with common variable immunodeficiency unable to tolerate IRT therapy (subcutaneous immunoglobulin [SCIG] 20% solution once weekly). She experienced AEs of severe neuropathy, described as burning and pins\u2010and\u2010needles sensation in the extremities and muscle twitching for several days post\u2010treatment. Dose decreases of SCIG to 0.5\u2009g did not improve the AE profile. Inability to tolerate IRT caused suboptimal dosing and inadequate primary immunodeficiency management, resulting in hospitalizations for pneumonia and sepsis. A trial of recombinant human C1\u2010INH 4200\u2009U was administered intravenously over approximately 5\u2009min, 1\u2009h prior to SCIG 1\u2009g (Day 1). This dose was well tolerated with minimal AEs reported. SCIG 3\u2009g was administered on Days 2 and 3 with no AEs reported. By continuing routine recombinant human C1\u2010INH 4200\u2009U prophylaxis, the patient was able to tolerate the recommended dose of SCIG 20\u2009g once weekly without the debilitating neuropathy and other AEs previously experienced with SCIG alone. This case suggests that a patient with IRT\u2010related AEs may benefit from C1\u2010INH replacement therapy prior to SCIG\/IVIG administration to improve tolerability.<\/jats:p>","DOI":"10.1002\/ccr3.71988","type":"journal-article","created":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T03:46:23Z","timestamp":1770349583000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Managing Primary Immunodeficiency Immunoglobulin Replacement Therapy\u2010Related Adverse Events With Recombinant Human\n                    <scp>C1<\/scp>\n                    Esterase Inhibitor Prophylaxis: A Case Report"],"prefix":"10.1002","volume":"14","author":[{"given":"Douglas H.","family":"Jones","sequence":"first","affiliation":[{"name":"Rocky Mountain Allergy, Asthma, and Immunology  Salt Lake City Utah USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heidi","family":"Memmott","sequence":"additional","affiliation":[{"name":"Pharming Healthcare, Inc  Warren New Jersey USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,2,5]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2016.09.023"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.anai.2015.04.023"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.2147\/BTT.S25188"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.632744"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41577\u2010023\u201000926\u20101"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.00794"},{"key":"e_1_2_10_8_1","volume-title":"Ruconest (C1 Esterase Inhibitor [Recombinant]) for Intravenous Use, Lyophilized Powder for Reconstitution. Package Insert","year":"2020"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.15214"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.3389\/falgy.2022.952233"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(17)31963\u20103"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.09.002"}],"container-title":["Clinical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.71988","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ccr3.71988","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.71988","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T17:24:27Z","timestamp":1772990667000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ccr3.71988"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2]]},"references-count":11,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1002\/ccr3.71988"],"URL":"https:\/\/doi.org\/10.1002\/ccr3.71988","archive":["Portico"],"relation":{},"ISSN":["2050-0904","2050-0904"],"issn-type":[{"value":"2050-0904","type":"print"},{"value":"2050-0904","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2]]},"assertion":[{"value":"2025-08-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-01-17","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-05","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71988"}}