Long-term evaluation of a historical cohort of Iranian common variable immunodeficiency patients

Aghamohammadi, Asghar; Abolhassani, Hassan; Latif, AmirHossein; Tabassomi, Firouzeh; Shokuhfar, Tahaamin; Sagvand, Babak Torabi; Shahinpour, Shervin; Moeini, Babak; Parvaneh, Nima; Movahedi, Masoud; Gharagozlou, Mohammad; Sherkat, Roya; Amin, Reza; Aleyasin, Soheila; Faridhosseini, Reza; Jabbari‐Azad, Farahzad; Cheraghi, Taher; Eslamian, Mohammad Hossein; Khalili, Abbas; Kalantari, Najmoddin; Shafiei, Alireza; Dabbaghzade, Abbas; Khayatzadeh, Alireza; Ebrahimi, Mohsen; Razavinejad, Davood; Bazregari, Saeid; Ebrahimi, Milad; Ghaffari, Javad; Bemanian, Mohammad Hassan; Behniafard, Nasrin; Kashef, Sara; Mohammadzadeh, Iraj; Hammarström, Lennart; Rezaei, Nima

doi:10.1586/1744666x.2014.958469

Cited by 23 publications

(11 citation statements)

References 93 publications

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“…Although this was the second most common infectious disease among Iranian XLA patients, chronic otitis media was the most common complication among chronic manifestations [35]. We found a lower rate of otitis media in CVID patients with hypogammaglobulinemia than in those with agammaglobulinemia [17,36]. This rate was higher in other studies [37][38][39], although this difference could be due to differences in the number of patients in each study (high in our study).…”

Section: Otitis Mediacontrasting

confidence: 57%

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

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Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs. Key words: Primary immunodeficiency disorders. Pulmonary complications. Upper respiratory tract. Lower respiratory tract. ResumenLas inmunodeficiencias primarias (PIDs) son enfermedades causadas por uno o más defectos del sistema inmunológico. Estos pacientes presentan con frecuencia infecciones recidivantes y/o severas así como otro tipo de complicaciones. Las patologías respiratorias son la principal y más frecuente manifestación y complicación de las PIDs. Las complicaciones de estas patologías pulmonares constituyen una de las principales causas de morbimortalidad entre los pacientes que sufren PIDs. El diagnóstico temprano y el tratamiento adecuado pueden prevenir, o al menos retrasar, la aparición de las complicaciones respiratorias en estos pacientes. Dado que el espectro de las enfermedades pulmonares es muy amplio, hemos dividido estas complicaciones entre aquellas que afectan a las vías aéreas superiores (sinusitis, otitis media y angioedema laríngeo, etc.) y las que afectan a las vías aéreas bajas (neumonía, bronquitis, bronquiectasias, enfermedades pulmonares intersticiales, neumonía organizada, adenopatías pulmonares y neoplasias, hiperreactividad bronquial, disgenesia pulmonar y las debidas a los efectos secundarios del tratamiento instaurado). Este artículo revisa las manifestaciones respiratorias que se observan más frecuentemente en los pacientes con PIDs. Palabras clave: Inmunodeficiencias primarias. Complicaciones pulmonares. Vías respiratorias altas. Vías respiratorias bajas.

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Section: Otitis Mediacontrasting

confidence: 57%

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

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“…+ T cells and that there is a strong correlation between naive CD4 + T-cell counts and clinical features [12,13,39,40]. In this regard, when CVID patients were classified on the basis of clinical phenotypes (infection only, polyclonal lymphoid proliferation, cytopenias, organ-specific autoimmunity, lymphoid malignancy, and enteropathy), the autoimmune cytopenia and organ-specific autoimmunity groups had the most significantly reduced naive CD4…”

Section: Naive Cd4 + T Cellsmentioning

confidence: 99%

T-Cell Abnormalities in Common Variable Immunodeficiency

Azizi

Rezaei

Kiaee

et al. 2016

J Investig Allergol Clin Immunol

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Common variable immunodeficiency (CVID) is the most common clinical primary immunodeficiency. It is characterized by a defect in B-cell differentiation to plasma and memory B cells. Moreover, numerous T-cell abnormalities have been reported and include decreased T-cell count and proliferative response, increased T-cell activation and apoptosis, and abnormalities in cytokine production. The aims of this review are to describe phenotypic and functional defects in T cells in CVID patients and to review the literature with respect to the effects of immunoglobulin replacement on the T-cell component in CVID patients. Key words: Common variable immunodeficiency. T cell. Helper T cells. Regulatory T cells. ResumenLa inmunodeficiencia común variable (CVID) es la inmunodeficiencia primaria más frecuente. Se caracteriza por un defecto en la diferenciación de linfocitos B hacia células plasmáticas y linfocitos B memoria. Se han descrito numerosas alteraciones en los linfocitos T de estos pacientes, tales como disminución en el número de linfocitos T y en sus respuestas proliferativas, aumento en la activación de células T y en la apoptosis, así como alteraciones en la producción de citokinas. El objetivo de esta revisión es describir las alteraciones funcionales y fenotípicas de los linfocitos T en los pacientes con CVID y revisar la bibliografía en relación a los efectos que la administración de inmunoglobulinas produce en los linfocitos T de los pacientes con CVID. Palabras clave: Inmunodeficiencia común variable. Células T. Células T helper. Células T reguladoras.

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“…In Iran, parental consanguinity is reported in 72% of patients suffering from CVID, while the mean proportion of consanguineous marriages in the country is 38% [35]. Previously, we also showed that the rate of parental consanguinity was significantly higher in children with early onset disease than in adults [9]. Furthermore, we have shown that the rate of multiple cases in a consanguineous cohort of CVID patients is more than 30% if all first relatives without severe clinical manifestations are evaluated [36].…”

Section: Discussionmentioning

confidence: 65%

A Comparison of Clinical and Immunologic Phenotypes in Familial and Sporadic Forms of Common Variable Immunodeficiency

et al. 2017

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Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency disease, and its prevalence varies significantly among different population. Minority of CVID patients present a familial aggregation suggesting a higher probability of heritable genetic defects. A total of 235 registered CVID patients were evaluated in this cohort study. Familial and sporadic patients were stratified, and demographic information, clinical records, laboratory and molecular data were compared among these two groups of patients. Multiple cases were identified in 12 families (30 patients) and sporadic presentation in 120 cases. The rate of parental consanguinity (83.3%) and clinical presentation of lymphoid malignancy (20.7%) were predominant in familial CVID patients, whereas significantly increased recurrent upper respiratory infections were recorded in sporadic patients (0.3 infections per year). Probands of familial group were presented with a higher severity score resulting in a profound mortality rate (41.7% after 30-year follow-up) comparing to the non-proband CVID patients in the same families with a lowered diagnostic delay. Familial CVID patients had a specific signature in clinical presentation and immunologic profile, and a high consanguinity in this group of patients suggests a Mendelian trait with an autosomal recessive inheritance pattern. Diagnosis of an index patient within a multiple case families significantly improves the diagnostic process and outcomes of the yet asymptomatic patients.

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Long-term evaluation of a historical cohort of Iranian common variable immunodeficiency patients

Abstract: Despite current efforts, more comprehensive studies are needed, especially for classification and investigation of the genetic background and prognostic factors for patients with CVID in order to better managment and followup of patinets.

Cited by 23 publications

References 93 publications

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

T-Cell Abnormalities in Common Variable Immunodeficiency

A Comparison of Clinical and Immunologic Phenotypes in Familial and Sporadic Forms of Common Variable Immunodeficiency

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