Pulmonary complications in primary hypogammaglobulinemia: a survey by high resolution CT scan

Gharagozlou, M; Ebrahimi, F.A.; Farhoudi, A.; Aghamohammadi, A; Bemanian, Mohammad Hasan; Chavoshzadeh, Zahra; Heidarzadeh, Mohammad; Mehdizadeh, Mehrzad; Moin, Mostafa; Movahedi, Masoud; Nabavi, Mohammad; Pourpak, Zahra; Rezaei, Narges

doi:10.4081/monaldi.2006.567

Cited by 15 publications

(11 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A reduced rate of carbon monoxide uptake and restrictive ventilatory pattern is typically found in CVID patients with ILD (63). A significant correlation between the HRCT score and the predicted values in pulmonary function test has been confirmed in several studies (46), while the others did not find such associations (49, 87). There is a negative correlation between a number of episodes of pneumonia and lung function tests.…”

Section: Respiratory Manifestations Of Primary Immunodeficienciesmentioning

confidence: 72%

“…The bronchiectasis is usually in the lower or middle lobes. It seems that the majority of the hypogammaglobulinemic patients suffer from the mild type of bronchiectasis (46). Bronchiectasis and bronchial wall thickening occurs in 17–76% of antibody deficiency patients and are well recognized complication in these patients (17).…”

Section: Respiratory Manifestations Of Primary Immunodeficienciesmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

et al. 2014

View full text Add to dashboard Cite

Primary immunodeficiencies (PIDs) are inherited disorders in which one or several components of immune system are decreased, missing, or of non-appropriate function. These diseases affect the development, function, or morphology of the immune system. The group of PID comprises more than 200 different disorders and syndromes and the number of newly recognized and revealed deficiencies is still increasing. Their clinical presentation and complications depend on the type of defects and there is a great variability in the relationship between genotypes and phenotypes. A variation of clinical presentation across various age categories is also presented and children could widely differ from adult patients with PID. Respiratory symptoms and complications present a significant cause of morbidity and also mortality among patients suffering from different forms of PIDs and they are observed both in children and adults. They can affect primarily either upper airways (e.g., sinusitis and otitis media) or lower respiratory tract [e.g., pneumonia, bronchitis, bronchiectasis, and interstitial lung diseases (ILDs)]. The complications from lower respiratory tract are usually considered to be more important and also more specific for PIDs and they determinate patients’ prognosis. The spectrum of the causal pathogens usually demonstrates typical pattern characteristic for each PID category. The respiratory signs of PIDs can be divided into infectious (upper and lower respiratory tract infections and complications) and non-infectious (ILDs, bronchial abnormalities – especially bronchiectasis, malignancies, and benign lymphoproliferation). Early diagnosis and appropriate therapy can prevent or at least slow down the development and course of respiratory complications of PIDs.

show abstract

Section: Respiratory Manifestations Of Primary Immunodeficienciesmentioning

confidence: 72%

Section: Respiratory Manifestations Of Primary Immunodeficienciesmentioning

confidence: 99%

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Most patients with hypogammaglobulinemia experience the mild type of bronchiectasis [67]. Patients with PADs are at a significantly increased risk for developing bronchiectasis [65,68,69], as we indicated in a study in which 37.5% of patients with bronchiectasis were diagnosed with defects in antibody-mediated immunity [70].…”

Section: Bronchiectasismentioning

confidence: 99%

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

View full text Add to dashboard Cite

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.

show abstract

“…Longitudinal data in children with bronchiectasis suggest that early diagnosis and intensive treatment slows deterioration of lung function (9,10). Gharagozlou showed that in patients with primary hypogammaglobulinemia, there had been a longer delay in diagnosis among those who developed bronchiectasis compared to those who did not (21).…”

Section: A B a B Discussionmentioning

confidence: 99%

Chronic suppurative lung disease in adults

Metersky

Mangardich

2016

J. Thorac. Dis.

View full text Add to dashboard Cite

Chronic suppurative lung disease (CSLD), characterized by a bronchiectasis-like syndrome in the absence of bronchial dilatation, is well described in the pediatric literature. In some patients, it may be a precursor of bronchiectasis. In adults, this syndrome has not been well described. We present four adult patients without obvious causative exposures who presented with prolonged cough and purulent sputum.Sputum cultures revealed a variety of Gram negative bacteria, fungi and mycobacteria. High resolution CT scanning did not reveal bronchiectasis. Evaluation revealed underlying causes including immunodeficiency in two, and Mycobacterium avium infection. One patient subsequently developed bronchiectasis. All patients improved with therapy. CSLD occurs in adults and has characteristics that distinguish it from typical chronic bronchitis. These include the lack of causative environmental exposures and infection with unusual pathogens. Evaluation and treatment of these patients similar to bronchiectasis patients may lead to clinical improvement. J Thorac Dis 2016;8(9):E974-E978 jtd.amegroups.com level were normal. He was now producing a half cup of yellow-green sputum daily, thus it was felt that he was a good candidate for azithromycin at 250 mg 3 times a week, as would be indicated for bronchiectasis patients. Three months later, he reported an "80% improvement" in his cough, which was then productive of only scant clear sputum and he has continued to do well with azithromycin and an airway clearance regimen. Case 2A 66-year-old male with a history of life-long asthma which was reasonably well controlled without systemic steroids, was referred for a several year history of daily cough of approximately a tablespoon of yellow sputum. His respiratory symptoms periodically worsened, and he experienced six exacerbations requiring antibiotics in the year prior to his referral. He had a 12-pack-year smoking history, discontinued more than 20 years previously. Physical examination was significant for a wet cough, rhinitis and diminished breath sounds at the lung bases. Spirometry testing showed borderline low FEV 1 /FVC ratio of 71% and sagging of the expiratory loop, but an FEV 1 of 111% of predicted and no bronchodilator response. Blood work showed an elevated IgE level at 590 kU/L. Other immunoglobulins, alpha-1 antitrypsin level and a cystic fibrosis (CF) mutation screen were normal. Sputum cultures showed Aspergillus fumigatus, Bordetella bronchiseptica, Pseudomonas aer uginosa and Mycobacterium avium complex (MAC). Aspergillus IgG and IgE were elevated, although the IgE elevation was borderline (0.47 kU/L, upper limit of normal 0.32 kU/L), suggesting the possibility of allergic bronchopulmonary aspergillosis. An HRCT from approximately 3 years prior to his referral, but after the onset of symptoms, demonstrated some areas of bronchial wall thickening but no bronchiectasis (Figure 2A). A HRCT after his referral demonstrated bronchiectasis, bronchial wall thickening and mucus impaction in his left lower lo...

show abstract

Pulmonary complications in primary hypogammaglobulinemia: a survey by high resolution CT scan

Abstract: It seems that the majority of hypogammaglobulinaemic patients suffer from the mild type of bronchiectasis, which is mostly observed in the proximal bronchi of the lower lobe segments. The delay of diagnosis plays an important role in the occurrence of this complication in these patients.

Cited by 15 publications

References 13 publications

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

Pulmonary Manifestations of Primary Immunodeficiency Disorders in Children

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Chronic suppurative lung disease in adults

Contact Info

Product

Resources

About