Clinical and Phenotypic Characterization of Common Variable Immunodeficiency Diagnosed in Younger and Older Adults

Fortier, Julia C; Haltigan, Emily; Cavero-Chavez, Vanessa; Gomez-Manjarres, Diana; Squire, Jacqueline D.; Reeves, Westley H.; Cuervo‐Pardo, Lyda

doi:10.1007/s10875-022-01290-w

Cited by 3 publications

(5 citation statements)

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“…The incidence of bronchiectasis was determined to be similar in the different age groups in the Fortier study [ 9 ], but in the current study it was found to be lower in older adults. Malignancy was observed at an equal frequency in the two age groups of the current study, and the absence of disease-related comorbidity was more common in older adults.…”

Section: Discussionmentioning

confidence: 49%

“…Due to the heterogeneous nature of CVID, the patients in the current study were separated into subgroups of similar clinical and phenotypic characteristics (lymphoproliferation, autoimmunity, GI/liver disease, malignancy). The only previous study in this area is the study by Fortier et al [ 9 ], in which CVID patients aged >40 years were evaluated, whereas in the current study, evaluations were made of older adult patients aged ≥65 years. Consistent with the previous study, the incidence of splenomegaly and autoimmunity was found to be lower in the older adults of the current study population.…”

Section: Discussionmentioning

confidence: 97%

“…The records of the European Society for Immunodeficiency (ESID) state that PID patients over the age of 65 constitute 8% of all PID patients and CVID has been determined to be the most commonly seen PID in this age group [ 8 ]. However, very little is known about the clinical characteristics of older adult CVID patients [ 9 ]. The aim of this study was to determine the immunological findings and clinical characteristics of older adult CVID patients and to examine the similarities and differences with adult patients.…”

Section: Highlight Key Pointsmentioning

confidence: 99%

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The clinical and immunological characteristics of common variable immunodeficiency in adults and older adults

Yildiz

2024

North Clin Istanbul

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OBJECTIVE The aim of this study was to determine the clinical and immunological characteristics of older adults with common variable immunodeficiency (CVID). METHODS Patients aged ≥18 years who were followed up with the diagnosis of CVID between 2015 and 2020 were included in the study. The patients were separated into two age groups according to the age at diagnosis: the adult group, aged 18-65 years (n=49) and the older adult group, aged ≥65 years (n=11). RESULTS Splenomegaly (55.1% vs. 9.1%, p=0.006), bronchiectasis (53.0% vs. 9.1%, p=0.008), and autoimmunity (42.8% vs. 9.1%, p=0.036) were determined to be more common in the adult group than in the older adults. A similar frequency of malignancy was seen in both groups (6.1% vs. 9.1%, p=0.721). There were significantly more patients with no comorbidity in the older adult group than in the adult group (45.5% vs. 16.3%, p=0.034). Serum IgG and IgA levels were determined to be significantly higher in the older adult group than in the adult group (p=0.001 for all). The CD19+ B-cell count at the time of diagnosis was determined to be lower and the CD19+CD27+IgD- switched memory B-cells and CD16+CD56+ natural killer cell counts were higher in the older adults than in the adult group (p=0.016, p=0.032, p=0.044, respectively). CONCLUSION Knowledge of clinical and immunological differences in older adult CVID patients may be of benefit in polyclinic follow-up and in respect of changes to be made to the treatment plan.

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Section: Discussionmentioning

confidence: 49%

Section: Discussionmentioning

confidence: 97%

Section: Highlight Key Pointsmentioning

confidence: 99%

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The clinical and immunological characteristics of common variable immunodeficiency in adults and older adults

Yildiz

2024

North Clin Istanbul

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“…Previous cross-sectional studies investigated the differences in CVID manifestations and complications according to age at onset or at diagnosis (14,(27)(28)(29)(30). They reported that pediatriconset CVID patients were more affected by infections and autoimmunity than adult-onset patients.…”

Section: Age At Cvid Onsetmentioning

confidence: 99%

Long-term follow-up in common variable immunodeficiency: the pediatric-onset and adult-onset landscape

et al. 2023

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IntroductionThe primary aim of this study is to investigate the evolution of the clinical and laboratory characteristics during the time in a longitudinal cohort of pediatric-onset and adult-onset Common Variable Immunodeficiency (CVID) patients in order to identify early predictive features of the disease and immune dysregulation complications.MethodsThis is a retrospective-prospective monocentric longitudinal study spanning from 1984 to the end of 2021. The data of pediatric-onset vs. adult-onset patients have been compared for immunological features and for infectious and non-infectious complications assessed at diagnosis and follow-up.ResultsSeventy-three CVID patients have been enrolled, with a mean of 10.0 years (SD ± 8.17) of prospective follow-up. At diagnosis, infections were observed in 89.0% of patients and immune dysregulation in 42.5% of patients. At diagnosis, 38.6% of pediatric-onset and 20.7% of adult-onset patients presented with only infections. Polyclonal lymphoid proliferation (62.1%) and autoimmunity (51.7%) were more prevalent in the adult-onset than in the pediatric-onset group (polyclonal lymphoid proliferation 52.3% and autoimmunity 31.8%, respectively). Enteropathy was present in 9.1% of pediatric-onset and 17.2% of adult-onset patients. The prevalence of polyclonal lymphoid proliferation increased during follow-up more in pediatric-onset patients (diagnosis 52.3%—follow-up 72.7%) than in adult-onset patients (diagnosis 62.1%—follow-up 72.7%). The cumulative risk to develop immune dysregulation increases according to the time of disease and the time of diagnostic delay. At the same age, pediatric-onset patients have roughly double the risk of having a complication due to immune dysregulation than adult-onset patients, and it increases with diagnostic delay. The analysis of lymphocyte subsets in the pediatric-onset group showed that CD21 low B cells at diagnosis may be a reliable prognostic marker for the development of immune dysregulation during follow-up, as the ROC curve analysis showed (AUC = 0.796). In the adult-onset group, the percentage of transitional B cells measured at diagnosis showed a significant accuracy (ROC AUC = 0.625) in identifying patients at risk of developing immune dysregulation.DiscussionThe longitudinal evaluation of lymphocyte subsets combined with clinical phenotype can improve the prediction of lymphoid proliferation and allow experts to achieve early detection and better management of such complex disorder.

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“…Risk factors for GLILD include history of cytopenias, female gender, age between 20 and 50 years, concomitant hypersplenism and polyarthritis ( 17 , 18 ). It is associated with increased risk for non-Hodgkin's lymphoma ( 19 ). Dysregulated B cells, with increased expression of B cell activating factor (BAFF), which upregulates IFN- γ signaling, have been reported in CVID with interstitial lung diseases ( 20 ).…”

Section: Introductionmentioning

confidence: 99%

Granulomatous inflammation in inborn errors of immunity

Sacco

Gazzin²,

Notarangelo³

et al. 2023

Front. Pediatr.

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Granulomas have been defined as inflammatory infiltrates formed by recruitment of macrophages and T cells. The three-dimensional spherical structure typically consists of a central core of tissue resident macrophages which may merge into multinucleated giant cells surrounded by T cells at the periphery. Granulomas may be triggered by infectious and non-infectious antigens. Cutaneous and visceral granulomas are common in inborn errors of immunity (IEI), particularly among patients with chronic granulomatous disease (CGD), combined immunodeficiency (CID), and common variable immunodeficiency (CVID). The estimated prevalence of granulomas in IEI ranges from 1%–4%. Infectious agents causing granulomas such Mycobacteria and Coccidioides presenting atypically may be ‘sentinel’ presentations for possible underlying immunodeficiency. Deep sequencing of granulomas in IEI has revealed non-classical antigens such as wild-type and RA27/3 vaccine-strain Rubella virus. Granulomas in IEI are associated with significant morbidity and mortality. The heterogeneity of granuloma presentation in IEI presents challenges for mechanistic approaches to treatment. In this review, we discuss the main infectious triggers for granulomas in IEI and the major forms of IEI presenting with ‘idiopathic’ non-infectious granulomas. We also discuss models to study granulomatous inflammation and the impact of deep-sequencing technology while searching for infectious triggers of granulomatous inflammation. We summarize the overarching goals of management and highlight the therapeutic options reported for specific granuloma presentations in IEI.

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Clinical and Phenotypic Characterization of Common Variable Immunodeficiency Diagnosed in Younger and Older Adults

Cited by 3 publications

References 47 publications

The clinical and immunological characteristics of common variable immunodeficiency in adults and older adults

The clinical and immunological characteristics of common variable immunodeficiency in adults and older adults

Long-term follow-up in common variable immunodeficiency: the pediatric-onset and adult-onset landscape

Granulomatous inflammation in inborn errors of immunity

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