Comparison of pulmonary diseases in common variable immunodeficiency and X‐linked agammaglobulinaemia

Aghamohammadi, Asghar; Allahverdi, Abdolreza; Abolhassani, Hassan; Moazzami, Kasra; Alizadeh, Hooman; Gharagozlou, Mohammad; Kalantari, Najmoddin; Sajedi, Vahid; Shafiei, Alireza; Parvaneh, Nima; Mohammadpour, Masoud; Karimi, Nasser; Sadaghiani, Mohammad Salehi; Rezaei, Nima

doi:10.1111/j.1440-1843.2009.01679.x

Cited by 63 publications

(45 citation statements)

References 63 publications

(139 reference statements)

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“…The incidence of bronchiectasis in patients with XLA is almost 32%, although XLA has been associated with up to 3% of cases of childhood bronchiectasis [73][74][75] and is rare in adults. We indicated that the rate and severity of bronchiectasis were greater in CVID patients than in those with XLA, probably because of the earlier diagnosis and treatment of XLA patients [76]. Moreover, CVID patients had a greater likelihood of developing lung disease, possibly owing to delayed diagnosis and immune dysregulation, as compared with XLA patients [76].…”

Section: Bronchiectasismentioning

confidence: 77%

“…We indicated that the rate and severity of bronchiectasis were greater in CVID patients than in those with XLA, probably because of the earlier diagnosis and treatment of XLA patients [76]. Moreover, CVID patients had a greater likelihood of developing lung disease, possibly owing to delayed diagnosis and immune dysregulation, as compared with XLA patients [76]. Mild to severe vitamin D deficiency in CVID patients is considered to be associated with a risk of bronchiectasis.…”

Section: Bronchiectasismentioning

confidence: 82%

See 1 more Smart Citation

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: Bronchiectasismentioning

confidence: 77%

Section: Bronchiectasismentioning

confidence: 82%

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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“…Hypogammaglobulinemia or antibody deficiency is the major hallmark of PAD patients. Affected individuals share a clinical phenotype with common features, such as chronic and recurrent infections, chronic inflammation, and autoimmunity [2], leading to recurrent hospitalization and a decreased quality of life [3,4,5]. …”

Section: Introductionmentioning

confidence: 99%

Autoimmunity in Primary Antibody Deficiencies

Azizi¹,

Ahmadi²,

Abolhassani³

et al. 2016

Int Arch Allergy Immunol

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Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients. Immune thrombocytopenic purpura and autoimmune hemolytic anemia are the most common autoimmune disorders in this group of patients. The aim of this review is to describe the proposed mechanisms for autoimmunity and to review the literature with respect to the reported autoimmune disorders in each type of PAD.

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“…98 Clinically, patients present with recurrent pyogenic sinopulmonary infections in the second or third decades of life; this later presentation could account for the higher incidence of lung disease seen with CVID when compared with XLA. 99 Bronchiectasis and lymphoid interstitial pneumonitis may develop and there is also an increased risk of autoimmune disease and malignancy. The development of bronchiectasis is aided by unregulated inflammation intrinsic to the disease as well as a low number of IgM memory B cells and reduced IgM anti-pneumococcal polysaccharide antibodies seen in a subset of these patients noted to be at higher risk for recurrent encapsulated bacterial infections.…”

Section: Disorders Of Humoral Immunitymentioning

confidence: 99%