Clinical picture and treatment of 2212 patients with common variable immunodeficiency

Gathmann, Benjamin; Mahlaoui, Nizar; Gérard, Laurence; Oksenhendler, Éric; Warnatz, Klaus; Schulze, Ilka; Kindle, Gerhard; Kuijpers, Taco W.; Beem, Rachel T. van; Guzmán, David Sánchez-Migallón; Workman, Sarita; Soler‐Palacín, Pere; Gracia, Javier de; Witte, Torsten; Schmidt, Reinhold; Litzman, Jiří; Hlaváčková, Eva; Thon, Vojtěch; Borte, Michael; Borte, Stephan; Kumararatne, Dinakantha; Feighery, Conleth; Longhurst, Hilary; Helbert, Matthew; Szaflarska, Anna; Šedivá, Anna; Belohradsky, Bernd H.; Jones, Alison; Baumann, Ulrich; Meyts, Isabelle; Necil, Kutukculer; Wågström, Per; Galal, Nermeen; Roesler, Joachim; Farmaki, Evangelia; Zinovieva, Natalia; Čižnár, Peter; Papadopoulou‐Alataki, Efimia; Bienemann, Kirsten; Velbri, S; Panahloo, Zoya; Grimbacher, Bodo

doi:10.1016/j.jaci.2013.12.1077

Cited by 504 publications

(532 citation statements)

References 22 publications

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“…Almost two-thirds of CVID patients have experienced at least 1 episode of pneumonia before diagnosis, and many experienced multiple prior episodes [49][50][51]. A recent large-scale study found that pneumonia was the most frequent manifestation in 2212 CVID patients [52]. The authors demonstrated that levels of IgG were lower in patients with recurrent pneumonia than in patients without pneumonia, indicating that the risk of pneumonia increases if patients have a low serum IgG level.…”

Section: Pneumoniamentioning

confidence: 99%

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: Pneumoniamentioning

confidence: 99%

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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“…Common variable immunodeficiency (CVID) is a mysterious primary immunodeficiency disorder (PID) characterized by impaired immunoglobulin production and an unpredictable clinical course (1). Two peaks in childhood (before the age of 10 years) and adulthood (between 30 and 40 years of age) are seen for this disorder.…”

Section: Introductionmentioning

confidence: 99%

Adults with common variable immunodeficiency: a single-center experience*

Muşabak¹,

YEŞİLLİK²,

Baysan³

et al. 2017

Turk J Med Sci

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Background/aim: In this study, we aimed to assess the clinical and immunological findings of our patients with common variable immunodeficiency (CVID). Materials and methods:We analyzed the records of 31 adult patients with CVID (12 females, 19 males). The patients were classified into clinical and immunophenotypic subgroups for statistical comparisons.Results: Our patients had some clinical signs in considerable frequencies, such as low body weight (45.2%), urinary tract infections (41.9%), various dermatoses (35.5%), and oral aphthae (32.3%). The histological findings in the biopsy specimens of the gastrointestinal tract (nodular lymphoid hyperplasia, villous atrophy, and lymphocytic infiltrates at mucosa) were significantly associated with splenomegaly, hepatomegaly, or low body weight (P = 0.005, 0.045, and 0.007, respectively). The patients with low CD4/CD8 ratios had lower IgG levels and a lower percentage of CD19+ B cells, but a higher percentage of activated T cells (P = 0.023, 0.011, and 0.028, respectively). Conclusion:In adults with CVID, there existed some clinical signs at considerable frequencies, but these are not overemphasized in the literature. The CD4/CD8 ratio is an important factor in antibody production and the clinical presentation of CVID. It seems that the adaptive immune system is on alert and subclinical immune activation insidiously continues in patients with CVID.

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“…A higher prevalence has been reported from countries with consanguine marriages (8). CVID is the most common phenotype of PIDs and embraces a heterogeneous group of patients which either suffer from infections only or in addition from inflammation, granuloma, autoimmunity, colitis, cytopenia and malignancy (9)(10)(11)(12). Ten to twenty percent of the CVID phenotype have a familial trait and in about 3% of CVID 10 different gene defects or polymorphisms have been identified in the last 10 years (deficiencies of BAFF-R, CD19, CD20, CD21, CD81, ICOS, LRBA, MSH5, NF-κB2, PI3Kδ, and TACI) (13;14).…”

Section: Progress In Understanding the Physiopathology Of Primary Immmentioning

confidence: 99%

A glance on recent progresses in diagnosis and treatment of primary immunodeficiencies/ Progrese recente în diagnosticul şi tratamentul imunodeficienţelor primare

Späth¹

2014

Romanian Review of Laboratory Medicine

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Clinical picture and treatment of 2212 patients with common variable immunodeficiency

Cited by 504 publications

References 22 publications

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Adults with common variable immunodeficiency: a single-center experience*

A glance on recent progresses in diagnosis and treatment of primary immunodeficiencies/ Progrese recente în diagnosticul şi tratamentul imunodeficienţelor primare

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