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Eur J Gastroenterol Hepatol. 2023 Feb 1;35(2):167-173. doi: 10.1097/MEG.0000000000002484. Epub 2022 Nov 29.

ABSTRACT

OBJECTIVES: Data are lacking on the immunogenicity of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines in patients affected by coeliac disease, Whipple’s disease and other noncoeliac enteropathies (NCE), characterised by primary or drug-related immunosuppression. We aimed to assess humoral response to SARS-CoV-2 vaccination in these patients compared to controls.

METHODS: Between December 2021 and January 2022, IgG anti-SARS-CoV-2 spike protein antibodies were measured in serum samples of coeliac disease, Whipple’s disease and NCE patients attending our gastroenterology outpatient clinic for follow-up, who had received their first SARS-CoV-2 vaccination dose 3-6-9 (±1) months prior. Humoral response was compared with healthy controls (vaccinated healthcare workers undergoing serological screening), matched for gender, age, and time from first vaccine dose at sample collection.

RESULTS: A total of 120 patients [107 coeliac disease; 10 Whipple’s disease; 2 common-variable immunodeficiency (CVID); 1 idiopathic villous atrophy; 77 F, 42 ± 16 years] and 240 matched controls (154 F, 43 ± 14 years) were enrolled. At 3, 6 and 9 months, humoral response in coeliac patients was not impaired compared to controls. Inadequate humoral response to vaccination was significantly more common among Whipple’s disease patients than controls (P < 0.001). Patients on immunosuppressive therapy had markedly lower IgG anti-SARS-CoV-2 antibody titres (median 14 vs. 520 BAU/mL, P < 0.001). As expected, patients with CVID showed no humoral response to vaccination.

CONCLUSIONS: Humoral immunogenicity of SARS-CoV-2 vaccines was not reduced in coeliac disease patients compared to controls, although it was in Whipple’s disease and CVID patients. Post-vaccination humoral response should be monitored in patients with Whipple’s disease and chronic enteropathies on immunosuppressive therapy in order to schedule vaccine booster doses.

PMID:36574307 | DOI:10.1097/MEG.0000000000002484

Clin Exp Immunol. 2022 Dec 26:uxac121. doi: 10.1093/cei/uxac121. Online ahead of print.

ABSTRACT

Common variable immunodeficiency (CVID) is a “late-onset” primary immunodeficiency characterized by variable manifestations and genetic heterogeneity. A monogenic cause of CVID has been reported in 10% of patients. In this study, we identified two novel pathogenic variants implicated in monogenic CVID by whole exome sequencing (WES) analysis: a heterozygous nuclear factor κB subunit 1 (NFKB1) p.G686fs mutation and a homozygous inducible T-cell co-stimulator (ICOS) p.L96Sfs mutation. The predicted crystal models indicated premature truncation of the two mutated proteins. Both variants were demonstrated as loss-of-function mutations and were associated with overlapped manifestations of respiratory fungal infection and splenomegaly. We further performed a detailed assessment of immunologic phenotypes and impaired lymphocyte functions in patients. Moreover, we discovered an association between monoclonal T-large granular lymphocyte proliferation and ICOS-deficient CVID for the first time. These observations lead to a new perspective on the underlying genetic heterogeneity of CVID.

PMID:36571238 | DOI:10.1093/cei/uxac121

Allergy Asthma Clin Immunol. 2022 Dec 24;18(1):110. doi: 10.1186/s13223-022-00746-3.

ABSTRACT

BACKGROUND: Understanding the impact of different immunoglobulin (Ig) infusion methods (intravenous [IVIg] and subcutaneous [SCIg]) upon treatment experience can potentially facilitate optimization of patient outcomes. Here, the perspective of patients with primary and secondary immunodeficiency diseases (PID and SID, respectively) receiving IVIg and SCIg was evaluated, in terms of treatment satisfaction, accounting for treatment history, using Association des Patients Immunodéficients du Québec (APIQ) survey data.

METHODS: The online APIQ survey (shared October 2020-March 2021) of patients with immunodeficiencies in Canada contained 101 questions on: Ig use, history, and detailed infusion characteristics; as well as structured patient-reported outcomes such as treatment satisfaction (via TSQM-9), symptom state (via PASS), general health perception (via GHP), and physical and mental function (via PROMIS). Adult respondents (≥ 18 years old) currently using Ig were compared by their current Ig infusion method (IVIg or SCIg cohort) overall, and in a sub-analysis, the IVIg cohort was compared with the SCIg cohort after stratification by respondents who started SCIg when naïve to Ig (‘SCIg naïve’) or with previous IVIg experience (‘SCIg switch’).

RESULTS: In total, 54 respondents currently used IVIg and 242 used SCIg. The average duration per infusion of a weekly SCIg infusion was significantly shorter compared with the average duration of a 3-4 weekly IVIg infusion (p < 0.001). The SCIg cohort was associated with significantly higher scores for the TSQM-9 effectiveness domain compared with the IVIg cohort. The scores for TSQM-9 convenience and global satisfaction domains were similar in the two cohorts. The SCIg cohort was also associated with a significantly higher proportion of respondents who were in an acceptable symptom state and a lower proportion who reported very poor or poor perception of health compared with the IVIg cohort. Further, the SCIg naïve subgroup was associated with significantly higher TSQM-9 effectiveness and convenience domain scores compared with the IVIg cohort, while there was no significant difference between the SCIg switch subgroup and the IVIg cohort in terms of convenience.

CONCLUSIONS: A better understanding of how different IgRT administration methods impact treatment experience and satisfaction may assist with informed treatment decision making and ultimately further improvements in patient outcomes.

PMID:36566213 | DOI:10.1186/s13223-022-00746-3

Immunol Med. 2022 Dec 23:1-3. doi: 10.1080/25785826.2022.2151170. Online ahead of print.

ABSTRACT

Primary immunodeficiency (PI) patients may still experience persistent viral and bacterial respiratory infections with ongoing treatments. We report a challenging case of a PI patient who experienced recurrent viral respiratory infections despite receiving standard immunoglobulin replacement therapy. The patient was subsequently managed with immune globulin intravenous, human-slra (ASCENIV™) that contains elevated antibodies against multiple respiratory pathogens. The patient demonstrated significant clinical improvement with a resolution of persistent and debilitating viral respiratory infections and associated sequela.

PMID:36562473 | DOI:10.1080/25785826.2022.2151170

Pathogens. 2022 Dec 10;11(12):1515. doi: 10.3390/pathogens11121515.

NO ABSTRACT

PMID:36558849 | DOI:10.3390/pathogens11121515

Genes (Basel). 2022 Dec 13;13(12):2348. doi: 10.3390/genes13122348.

NO ABSTRACT

PMID:36553615 | DOI:10.3390/genes13122348

Medicine (Baltimore). 2022 Dec 16;101(50):e32347. doi: 10.1097/MD.0000000000032347.

NO ABSTRACT

PMID:36550896 | DOI:10.1097/MD.0000000000032347

Med Sci (Basel). 2022 Dec 4;10(4):67. doi: 10.3390/medsci10040067.

NO ABSTRACT

PMID:36548002 | DOI:10.3390/medsci10040067

Front Immunol. 2022 Dec 5;13:1023127. doi: 10.3389/fimmu.2022.1023127. eCollection 2022.

NO ABSTRACT

PMID:36544766 | PMC:PMC9760934 | DOI:10.3389/fimmu.2022.1023127

Sci Transl Med. 2022 Dec 21;14(676):eabp9675. doi: 10.1126/scitranslmed.abp9675. Epub 2022 Dec 21.

NO ABSTRACT

PMID:36542690 | DOI:10.1126/scitranslmed.abp9675