Yuxue Nie, Nianyi Zhang, Jingna Li, Di Wu, Yunjiao Yang, Li Zhang, Wei Bai, Nan Jiang, Lin Qiao, Can Huang, Shuang Zhou, Xinping Tian, Mengtao Li, Xiaofeng Zeng, Linyi Peng, Wen Zhang
{"title":"Hypogammaglobulinemia and Infection Events in Patients with Autoimmune Diseases Treated with Rituximab: 10 Years Real-Life Experience.","authors":"Yuxue Nie, Nianyi Zhang, Jingna Li, Di Wu, Yunjiao Yang, Li Zhang, Wei Bai, Nan Jiang, Lin Qiao, Can Huang, Shuang Zhou, Xinping Tian, Mengtao Li, Xiaofeng Zeng, Linyi Peng, Wen Zhang","doi":"10.1007/s10875-024-01773-y","DOIUrl":"10.1007/s10875-024-01773-y","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate predictors of hypogammaglobulinemia (HGG) and severe infection event (SIE) in patients with autoimmune disease (AID) receiving rituximab (RTX) therapy.</p><p><strong>Methods: </strong>This was a retrospective study conducted in a tertiary medical center in China. Predictors of HGG or SIE were assessed using Cox analysis. Restricted cubic spline (RCS) analysis was applied to examine the correlation between glucocorticoid (GC) maintenance dose and SIE.</p><p><strong>Results: </strong>A total of 219 patients were included in this study, with a cumulative follow-up time of 698.28 person-years. Within the study population, 117 patients were diagnosed with connective tissue disease, 75 patients presented with ANCA-associated vasculitis, and 27 patients exhibited IgG4-related disease. HGG was reported in 63.3% of the patients, where an obvious decline in IgG and IgM was shown three months after RTX initiation. The rate of SIE was 7.2 per 100 person-years. An increase in the GC maintenance dose was an independent risk factor for both hypo-IgG (HR 1.07, 95% CI 1.02-1.12, p = 0.003) and SIE (HR 1.06, 95% CI 1.02-1.1, p = 0.004). Further RCS analysis identified 7.48 mg/d prednisone as a safe threshold dose for patients who underwent RTX treatment to avoid a significantly increased risk for SIE.</p><p><strong>Conclusion: </strong>HGG was relatively common in RTX-treated AID patients. Patients with chronic lung disease or who were taking ≥ 7.5 mg/d prednisone during RTX treatment were at increased risk for SIE and warrant attention from physicians.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"179"},"PeriodicalIF":7.2,"publicationDate":"2024-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141988043","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jennifer G Chester, Alani M Estrella, Douglas B Kuhns, Christine K Garcia, Ramsay L Fuleihan
{"title":"Expanding the Clinical Phenotype of Autosomal Recessive Chronic Granulomatous Disease.","authors":"Jennifer G Chester, Alani M Estrella, Douglas B Kuhns, Christine K Garcia, Ramsay L Fuleihan","doi":"10.1007/s10875-024-01782-x","DOIUrl":"10.1007/s10875-024-01782-x","url":null,"abstract":"","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"177"},"PeriodicalIF":7.2,"publicationDate":"2024-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141982458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Pathogenic Role of Anti-Granulocyte-Macrophage Colony-Stimulating Factor Autoantibodies in the Nocardiosis with the Central Nervous System Involvement.","authors":"Yu-Fang Lo, Shang-Yu Wang, Yi-Hui Wu, Mao-Wang Ho, Chun-Fu Yeh, Tsai-Yi Wu, Jhan-Jie Peng, You-Ning Lin, Jing-Ya Ding, Han-Po Shih, Chia-Chi Lo, Yu-Pei Chan, Cheng-Shyuan Rau, Chen-Yen Kuo, Kun-Hua Tu, Wei-Te Lei, Yi-Chun Chen, Cheng-Lung Ku","doi":"10.1007/s10875-024-01775-w","DOIUrl":"10.1007/s10875-024-01775-w","url":null,"abstract":"<p><strong>Purpose: </strong>Anti-granulocyte-macrophage colony-stimulating factor autoantibodies (anti-GM-CSF Abs) are implicated in the pathogenesis of Cryptococcus gattii (C. gattii) infection and pulmonary alveolar proteinosis (PAP). Their presence has also been noted in nocardiosis cases, particularly those with disseminated disease. This study delineates a case series characterizing clinical features and specificity of anti-GM-CSF Abs in nocardiosis patients.</p><p><strong>Methods: </strong>In this study, eight patients were recruited to determine the presence or absence of anti-GM-CSF Abs. In addition to the detailed description of the clinical course, we thoroughly investigated the autoantibodies regarding the characteristics, isotypes, subclasses, titers, and neutralizing capacities by utilizing the plasma samples from patients.</p><p><strong>Results: </strong>Of eight patients, five tested positive for anti-GM-CSF Abs, all with central nervous system (CNS) involvement; patients negative for these antibodies did not develop CNS nocardiosis. Distinct from previously documented cases, none of our patients with anti-GM-CSF Abs exhibited PAP symptoms. The titer and neutralizing activity of anti-GM-CSF Abs in our cohort did not significantly deviate from those found in C. gattii cryptococcosis and PAP patients. Uniquely, one individual (Patient 3) showed a minimal titer and neutralizing action of anti-GM-CSF Abs, with no relation to disease severity. Moreover, IgM autoantibodies were notably present in all CNS nocardiosis cases investigated.</p><p><strong>Conclusion: </strong>The presence of anti-GM-CSF Abs suggests an intrinsic immunodeficiency predisposing individuals toward CNS nocardiosis. The presence of anti-GM-CSF Abs helps to elucidate vulnerability to CNS nocardiosis, even with low titer of autoantibodies. Consequently, systematic screening for anti-GM-CSF Abs should be considered a crucial diagnostic step for nocardiosis patients.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"176"},"PeriodicalIF":7.2,"publicationDate":"2024-08-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zehra Busra Azizoglu, Royala Babayeva, Zehra Sule Haskologlu, Mustafa Burak Acar, Serife Ayaz-Guner, Fatma Zehra Okus, Mohammad Bilal Alsavaf, Salim Can, Kemal Erdem Basaran, Mehmed Fatih Canatan, Alper Ozcan, Hasret Erkmen, Can Berk Leblebici, Ebru Yilmaz, Musa Karakukcu, Mehmet Kose, Ozlem Canoz, Ahmet Özen, Elif Karakoc-Aydiner, Serdar Ceylaner, Gülsüm Gümüş, Huseyin Per, Hakan Gumus, Halit Canatan, Servet Ozcan, Figen Dogu, Aydan Ikinciogullari, Ekrem Unal, Safa Baris, Ahmet Eken
{"title":"DIAPH1-Deficiency is Associated with Major T, NK and ILC Defects in Humans.","authors":"Zehra Busra Azizoglu, Royala Babayeva, Zehra Sule Haskologlu, Mustafa Burak Acar, Serife Ayaz-Guner, Fatma Zehra Okus, Mohammad Bilal Alsavaf, Salim Can, Kemal Erdem Basaran, Mehmed Fatih Canatan, Alper Ozcan, Hasret Erkmen, Can Berk Leblebici, Ebru Yilmaz, Musa Karakukcu, Mehmet Kose, Ozlem Canoz, Ahmet Özen, Elif Karakoc-Aydiner, Serdar Ceylaner, Gülsüm Gümüş, Huseyin Per, Hakan Gumus, Halit Canatan, Servet Ozcan, Figen Dogu, Aydan Ikinciogullari, Ekrem Unal, Safa Baris, Ahmet Eken","doi":"10.1007/s10875-024-01777-8","DOIUrl":"10.1007/s10875-024-01777-8","url":null,"abstract":"<p><p>Loss of function mutations in Diaphanous related formin 1 (DIAPH1) are associated with seizures, cortical blindness, and microcephaly syndrome (SCBMS) and are recently linked to combined immunodeficiency. However, the extent of defects in T and innate lymphoid cells (ILCs) remain unexplored. Herein, we characterized the primary T, natural killer (NK) and helper ILCs of six patients carrying two novel loss of function mutation in DIAPH1 and Jurkat cells after DIAPH1 knockdown. Mutations were identified by whole exome sequencing. T-cell immunophenotyping, proliferation, migration, cytokine signaling, survival, and NK cell cytotoxicity were studied via flow cytometry-based assays, confocal microscopy, and real-time qPCR. CD4<sup>+</sup> T cell proteome was analyzed by mass spectrometry. p.R351* and p.R322*variants led to a significant reduction in the DIAPH1 mRNA and protein levels. DIAPH1-deficient T cells showed proliferation, activation, as well as TCR-mediated signaling defects. DIAPH1-deficient PBMCs also displayed impaired transwell migration, defective STAT5 phosphorylation in response to IL-2, IL-7 and IL-15. In vitro generation/expansion of Treg cells from naïve T cells was significantly reduced. shRNA-mediated silencing of DIAPH1 in Jurkat cells reduced DIAPH1 protein level and inhibited T cell proliferation and IL-2/STAT5 axis. Additionally, NK cells from patients had diminished cytotoxic activity, function and IL-2/STAT5 axis. Lastly, DIAPH1-deficient patients' peripheral blood contained dramatically reduced numbers of all helper ILC subsets. DIAPH1 deficiency results in major functional defects in T, NK cells and helper ILCs underlining the critical role of formin DIAPH1 in the biology of those cell subsets.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"175"},"PeriodicalIF":4.4,"publicationDate":"2024-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11315734/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141906767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Wenjing Ying, Xin Long, Travis Vandergriff, Hemanth Karnati, Meghan Heberton, Mingyi Chen, Xiaochuan Wang, Christian Wysocki, Xiao-Fei Kong
{"title":"Epidermodysplasia Verruciformis and Vδ2 γδ T-cell Expansion in STK4 Deficiency.","authors":"Wenjing Ying, Xin Long, Travis Vandergriff, Hemanth Karnati, Meghan Heberton, Mingyi Chen, Xiaochuan Wang, Christian Wysocki, Xiao-Fei Kong","doi":"10.1007/s10875-024-01780-z","DOIUrl":"10.1007/s10875-024-01780-z","url":null,"abstract":"<p><p>The clinical penetrance of infectious diseases varies considerably among patients with inborn errors of immunity (IEI), even for identical genetic defects. This variability is influenced by pathogen exposure, healthcare access and host-environment interactions. We describe here a patient in his thirties who presented with epidermodysplasia verruciformis (EV) due to infection with a weakly virulent beta-papillomavirus (HPV38) and CD4<sup>+</sup> T-cell lymphopenia. The patient was born to consanguineous parents living in the United States. Exome sequencing identified a previously unknown biallelic STK4 stop-gain mutation (p.Trp425X). The patient had no relevant history of infectious disease during childhood other than mild wart-like lesion on the skin, but he developed diffuse large B-cell lymphoma (DLBCL) and EBV viremia with a low viral load in his thirties. Despite his low CD4<sup>+</sup> T-cell count, the patient had normal counts of CD3<sup>+</sup> cells, predominantly double-negative T cells (67.4%), which turned out to be Vδ2<sup>+</sup> γδ T cells. γδ T-cell expansion has frequently been observed in the 33 reported cases with STK4 deficiency. The Vδ2 γδ T cells of this STK4-deficient patient are mostly CD45RA<sup>-</sup>CD27<sup>+</sup>CCR7<sup>+</sup> central memory γδT cells, and their ability to proliferate in response to T-cell activation was impaired, as was that of CD4<sup>+</sup> T cells. In conclusion, γδ T-cell expansion may act as a compensatory mechanism to combat viral infection, providing immune protection in immunocompromised individuals.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"172"},"PeriodicalIF":7.2,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11306306/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141897570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmed Elmoursi, Baijun Zhou, Mei-Sing Ong, Joseph S Hong, Andrew Pak, Megha Tandon, Natalia Sutherland, Daniel V DiGiacomo, Jocelyn R Farmer, Sara Barmettler
{"title":"A Cross-Sectional Study of Health-Related Quality of Life in Patients with Predominantly Antibody Deficiency.","authors":"Ahmed Elmoursi, Baijun Zhou, Mei-Sing Ong, Joseph S Hong, Andrew Pak, Megha Tandon, Natalia Sutherland, Daniel V DiGiacomo, Jocelyn R Farmer, Sara Barmettler","doi":"10.1007/s10875-024-01781-y","DOIUrl":"10.1007/s10875-024-01781-y","url":null,"abstract":"<p><p>Health-related quality of life (HRQoL) measures individual well-being across physical, psychological, and social domains. Patients with predominantly antibody deficiency (PAD) are at risk for morbidity and mortality, however, the effect of these complications on HRQoL requires additional study. Patients with PAD were asked to voluntarily complete the Centers for Disease Control (CDC) HRQoL-14 Healthy Days Measure questionnaire. These results were compared to data from the CDC-initiated Behavioral Risk Factor Surveillance System (BRFSS), a cross-sectional questionnaire including questions from CDC-HRQOL-14. Statistical analyses included two-proportion Z-test, t-tests, and analysis of variance. 83 patients with PAD completed the survey. Patients were sub-stratified into mild (23.7%), moderate (35.5%), severe (40.8%), and secondary (8.4%) PAD. \"Fair or poor\" health status was reported in 52.6% of PAD patients. Mental health challenges ≥ 14 days/month occurred in 25% of patients. Physical health issues ≥ 14 days/month was reported in 44.7% of patients. Activity limitations were noted by 80.3% of patients. There were no statistically significant differences by PAD severity. Patients with autoimmune and inflammatory disease co-morbidities reported more mental health challenges compared to those without (78% vs. 54.3%, p = 0.02). Compared to the CDC-BRFSS data, significantly more patients with PAD reported \"fair or poor\" health status (53% vs 12.0%; p < 0.0001), mental health challenges (24.1% vs 14.7%; p = 0.02), and poor physical health (44.6% vs 8.0%; p < 0.0001). Patients with PAD had significantly reduced HRQoL compared to CDC-BRFSS respondents from a similar geographical region. Decreased HRQoL was prevalent across all PAD severity levels. Additional research is needed to improve HRQoL for patients with PAD.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"173"},"PeriodicalIF":7.2,"publicationDate":"2024-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141897569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ximena León-Lara, Uriel Pérez-Blanco, Marco A Yamazaki-Nakashimada, Juan Carlos Bustamante-Ogando, Nancy Aguilar-Gómez, Hernán Cristerna-Tarrasa, Aidé-Tamara Staines-Boone, Omar J Saucedo-Ramírez, Eunice Fregoso-Zuñiga, Ana-Paola Macías-Robles, María R Canseco-Raymundo, Marco Venancio-Hernández, Cristina Moctezuma-Trejo, Berenise Gámez-González, Carmen Zarate-Hernández, Roselia Ramírez-Rivera, Selma Scheffler-Mendoza, Nancy Jiménez-Polvo, Leticia Hernández-Nieto, Jocelyn Carmona-Vargas, María L García-Cruz, Óscar Zavaleta-Martínez, Carla M Román-Montes, Victoria Cervantes-Parra, Anelena González-Reynoso, Rogelio Guzmán-Cotaya, Francisco Espinosa-Rosales, Patricia Saltigeral-Simental, Sara Espinosa-Padilla, Lizbeth Blancas Galicia
{"title":"Description of BCG and Tuberculosis Disease in a Cohort of 79 Patients with Chronic Granulomatous Disease.","authors":"Ximena León-Lara, Uriel Pérez-Blanco, Marco A Yamazaki-Nakashimada, Juan Carlos Bustamante-Ogando, Nancy Aguilar-Gómez, Hernán Cristerna-Tarrasa, Aidé-Tamara Staines-Boone, Omar J Saucedo-Ramírez, Eunice Fregoso-Zuñiga, Ana-Paola Macías-Robles, María R Canseco-Raymundo, Marco Venancio-Hernández, Cristina Moctezuma-Trejo, Berenise Gámez-González, Carmen Zarate-Hernández, Roselia Ramírez-Rivera, Selma Scheffler-Mendoza, Nancy Jiménez-Polvo, Leticia Hernández-Nieto, Jocelyn Carmona-Vargas, María L García-Cruz, Óscar Zavaleta-Martínez, Carla M Román-Montes, Victoria Cervantes-Parra, Anelena González-Reynoso, Rogelio Guzmán-Cotaya, Francisco Espinosa-Rosales, Patricia Saltigeral-Simental, Sara Espinosa-Padilla, Lizbeth Blancas Galicia","doi":"10.1007/s10875-024-01778-7","DOIUrl":"10.1007/s10875-024-01778-7","url":null,"abstract":"<p><strong>Purpose: </strong>Chronic granulomatous disease (CGD) is an inherited immunodeficiency caused by pathogenic variants of genes encoding the enzyme complex NADPH oxidase. In countries where tuberculosis (TB) is endemic and the Bacillus Calmette-Guérin (BCG) vaccine is routinely administered, mycobacteria are major disease-causing pathogens in CGD. However, information on the clinical evolution and treatment of mycobacterial diseases in patients with CGD is limited. The present study describes the adverse reactions to BCG and TB in Mexican patients with CGD.</p><p><strong>Methods: </strong>Patients with CGD who were evaluated at the Immunodeficiency Laboratory of the National Institute of Pediatrics between 2013 and 2024 were included. Medical records were reviewed to determine the clinical course and treatment of adverse reactions to BCG and TB disease.</p><p><strong>Results: </strong>A total of 79 patients with CGD were included in this study. Adverse reactions to BCG were reported in 55 (72%) of 76 patients who received the vaccine. Tuberculosis was diagnosed in 19 (24%) patients. Relapse was documented in three (10%) of 31 patients with BGC-osis and six (32%) of 19 patients with TB, despite antituberculosis treatment. There was no difference in the frequency of BCG and TB disease between patients with pathogenic variants of the X-linked CYBB gene versus recessive variants.</p><p><strong>Conclusions: </strong>This report highlights the importance of considering TB in endemic areas and BCG complications in children with CGD to enable appropriate diagnostic and therapeutic approaches to improve prognosis and reduce the risk of relapse.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"171"},"PeriodicalIF":7.2,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889395","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Insights into Patient Experiences with Facilitated Subcutaneous Immunoglobulin Therapy in Primary Immune Deficiency: A Prospective Observational Cohort.","authors":"Ezgi Yalcin Gungoren, Melek Yorgun Altunbas, Ummugulsum Dikici, Zeynep Meric, Isil Eser Simsek, Ayca Kiykim, Salim Can, Esra Karabiber, Nalan Yakici, Fazil Orhan, Haluk Cokugras, Metin Aydogan, Oner Ozdemir, Sevgi Bilgic Eltan, Safa Baris, Ahmet Ozen, Elif Karakoc-Aydiner","doi":"10.1007/s10875-024-01771-0","DOIUrl":"10.1007/s10875-024-01771-0","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin G replacement therapy (IgRT), intravenous (IV) and subcutaneous (SC) routes, is pivotal in treatment of primary immunodeficiencies (PID). In recent years, facilitated subcutaneous immunoglobulin (fSCIG), a combination of rHuPH20 and 10% IgG has emerged as a delivery method to combine advantages of both IV and SC.</p><p><strong>Method: </strong>In an observational prospective cohort, we investigated patient experience with fSCIG in PID patients from 5 PID centers for up to 12 months. We assessed the efficacy and safety of this treatment with patient/caregiver- and physician-reported indicators. Additionally, we analyzed patient treatment satisfaction (TSQM-9) and quality of life (QoL).</p><p><strong>Results: </strong>We enrolled 29 patients (22 pediatric and 7 adults; 14 females and 15 males; (median: 15, min-max: 2-40.9 years) who initiated fSCIG as IgRT-naive (n = 1), switched from conventional rapid-push 10% SCIG (n = 6) or IVIG (n = 22). Among the participants, 19 (65%) exhibited antibody deficiencies, 8 (27%) combined immunodeficiencies, and 2 (7%) immune dysregulations. Remarkably, targeted trough immunoglobulin G levels were achieved under all previous IgRTs as well as fSCIG. No severe systemic adverse drug reactions were documented, despite prevalent local (%86.45) and mild systemic (%26.45) adverse reactions were noted with fSCIG. Due to mild systemic symptoms, 2 patients switched from fSCIG to 10% SCIG. The patient satisfaction survey revealed a notable increase at 2-4th (p = 0.102); 5-8th (p = 0.006) and 9-12th (p < 0.001) months compared to the baseline. No significant trends were observed in QoL surveys.</p><p><strong>Conclusion: </strong>fSCIG demonstrates admissable tolerability and efficacy in managing PIDs in addition to notable increase of patients' drug satisfaction with IgRT. The identified benefits support the continuation of this therapy despite the local reactions.</p>","PeriodicalId":15531,"journal":{"name":"Journal of Clinical Immunology","volume":"44 8","pages":"169"},"PeriodicalIF":7.2,"publicationDate":"2024-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11298503/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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