{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T05:41:25Z","timestamp":1764481285047,"version":"3.46.0"},"reference-count":3,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,4,12]],"date-time":"2017-04-12T00:00:00Z","timestamp":1491955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"CSL Behring Institutional Grant"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2017,6]]},"abstract":"Key Clinical Message<\/jats:title>\n \n A 71\u2010year\u2010old female patient with no major history of infection had presented with recurrent chronic purulent sinusitis over the past 3\u00a0years. These recurrent infections started in 2000 with otolaryngologists\u2019 support before diagnosis of IgG4 deficiency be asked. The patient was treated with increasingly extensive courses of antibiotics and underwent several maxillary and sphenoidal sinus washouts. She continued to present with purulent nasal discharge containing\n Staphylococcus epidermidis<\/jats:italic>\n . The blood and immune work\u2010ups were normal. Her antinuclear antibody count was 1\/320, with no unusual types. The total immunoglobulin (Ig)E, serology,\n CD<\/jats:styled-content>\n 4 count, and lymphocyte B phenotype results were all normal. No humoral immune deficiency was detected. The analyses confirmed an underlying specific IgG4 deficiency with values of 3\u20134\u00a0mg\/L over 4\u00a0months. The patient was treated in March 2011 with prophylactic antibiotic therapy, sinus drainage, and\n IV<\/jats:styled-content>\n infusions of enriched immunoglobulins (\n IVI<\/jats:styled-content>\n g 10%) administered at the outpatient clinic every 4\u00a0weeks for 3\u00a0months. The Ig\n IV<\/jats:styled-content>\n treatment was not interrupted. Her general condition improved within a few months, with her IgG4 levels rising to 44\u00a0mg\/L. The\n IVI<\/jats:styled-content>\n g infusions were well tolerated. The purulent nasal discharge was controlled, and the antibiotics were stopped. The follow\u2010up visits at 2 and 9\u00a0months after introduction of\n IVI<\/jats:styled-content>\n g showed that her IgG4 level had improved, rising to 15 and 11\u00a0mg\/L, respectively, although it had not yet returned to normal. The infusions were then given every 3\u00a0weeks. At her last visit, the patient's clinical condition had substantially improved. She was able to start using the subcutaneous Ig concentrate form (20%\n SCI<\/jats:styled-content>\n g), 15\u00a0g every 2\u00a0weeks, leading to a clear improvement in her clinical condition, with stabilization of her otolaryngologists\u2019 symptoms and signs. The complete blood count was normal, IgG4 were stable at 40\u00a0mg\/L, and the other immunoglobulins and IgG subclasses were normal. It was then possible to reduce the\n SCI<\/jats:styled-content>\n g dose to 10\u00a0g every 3\u00a0weeks, while continuing to monitor her clinical condition and laboratory test results. This is one of the rare cases of selective IgG4 subclass deficiency treated with immunoglobulins. Treatment resulted in a significant improvement in IgG4 levels versus pretreatment levels. The first improvement noted was the stabilization otolaryngologists\u2019 infections particularly purulent nasal discharge.\n <\/jats:p>","DOI":"10.1002\/ccr3.936","type":"journal-article","created":{"date-parts":[[2017,4,12]],"date-time":"2017-04-12T03:52:54Z","timestamp":1491969174000},"page":"792-794","source":"Crossref","is-referenced-by-count":3,"title":["Chronic sinusitis in a patient with selective IgG4 subclass deficiency controlled with enriched immunoglobulins"],"prefix":"10.1002","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4123-305X","authenticated-orcid":false,"given":"G\u00e9rard","family":"Dine","sequence":"first","affiliation":[{"name":"H\u00e9matologie clinique et biologique H\u00f4pital des Hauts Clos 101 Avenue Anatole France Troyes 10000 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nadia","family":"Ali\u2010Ammar","sequence":"additional","affiliation":[{"name":"H\u00e9matologie clinique et biologique H\u00f4pital des Hauts Clos 101 Avenue Anatole France Troyes 10000 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Said","family":"Brahimi","sequence":"additional","affiliation":[{"name":"H\u00e9matologie clinique et biologique H\u00f4pital des Hauts Clos 101 Avenue Anatole France Troyes 10000 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yves","family":"Rehn","sequence":"additional","affiliation":[{"name":"H\u00e9matologie clinique et biologique H\u00f4pital des Hauts Clos 101 Avenue Anatole France Troyes 10000 France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,4,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1016\/j.jaci.2009.10.013","article-title":"Primary immunodeficiencies: 2009 update","volume":"124","author":"Notarangelo L. D.","journal-title":"J. Allergy Clin. Immunol."},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-011-9557-z"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0025-6196(12)64949-0"}],"container-title":["Clinical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fccr3.936","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ccr3.936","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T05:39:28Z","timestamp":1764481168000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ccr3.936"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4,12]]},"references-count":3,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2017,6]]}},"alternative-id":["10.1002\/ccr3.936"],"URL":"https:\/\/doi.org\/10.1002\/ccr3.936","archive":["Portico"],"relation":{},"ISSN":["2050-0904","2050-0904"],"issn-type":[{"type":"print","value":"2050-0904"},{"type":"electronic","value":"2050-0904"}],"subject":[],"published":{"date-parts":[[2017,4,12]]}}}