Progression of cystic fibrosis lung disease as a function of serum immunoglobulin G levels: A 5-year longitudinal study

Wheeler, William Bruce; Williams, Mary H.; Matthews, Wallace J.; Colten, Harvey R.

doi:10.1016/s0022-3476(84)80946-4

Cited by 108 publications

(68 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Reactive hypergammaglobulinaemia is typical in CF, but hypogammaglobulinaemia is found in a minority of children with CF, 9 although it is not necessarily a poor prognosis feature. 10,11 This child was also shown to be IgG2 deficient, as is seen in a significant minority of patients with CF. 9 Since the time of the original analysis the patient's immunoglobulins, including IgE, have steadily improved.…”

Section: Discussionmentioning

confidence: 69%

Immunoglobulin E deficiency: a forgotten clue pointing to possible immunodeficiency?

2011

View full text Add to dashboard Cite

Background: Patients with primary antibody deficiency often have delayed diagnosis. Very low IgE, found during investigations for allergy, may be a marker for other immunodeficiency. Methods: We introduced a new laboratory policy of testing cases with very low IgE levels for possible linked antibody deficiency. The data represent an audit of routine results collected over two years. Results: Very low IgE ( 2 IU/mL) was identified in 85/2622 (3.2%) routine patient samples. Two children and four adult patients were found to have one or more classes of immunoglobulin below the reference range for age. In 2/6, the initiative of the laboratory led to a new unsuspected diagnosis of antibody immunodeficiency. Conclusions: Common variable immunodeficiency continues to be overlooked as a primary cause of lung disease in adults. Very low serum IgE should trigger appropriate investigation (immunoglobulin quantification and serum electrophoresis).

show abstract

Section: Discussionmentioning

confidence: 69%

Immunoglobulin E deficiency: a forgotten clue pointing to possible immunodeficiency?

2011

View full text Add to dashboard Cite

show abstract

“…FEV1 is the best surrogate outcome measure in CF, with little within-subject variability and high relative precision (Cooper et al, 1990;Kerem et al, 1992). Serum total IgG levels represent a marker of the chronic inflammatory process in CF and correlate inversely with the patients' lung function and long-term prognosis (Wheeler et al, 1984;Levy et al, 2007). Therefore, data were analyzed using the same MLR model for both IgG and FEV1 as dependent variables.…”

Section: Inverse Relation Between Vitamin D and Serum Igg T Pincikovamentioning

confidence: 99%

“…Progressive pulmonary disease is the major cause of morbidity and mortality in CF patients. Both the severity of lung disease and long-term prognosis are closely correlated with total immunoglobulin G (IgG) levels in children and adults with CF (Wheeler et al, 1984;Levy et al, 2007). Bacterial infection and chronic colonization of the lungs are the primary stimuli for inflammation in the CF airway.…”

Section: Introductionmentioning

confidence: 99%

Inverse relation between vitamin D and serum total immunoglobulin G in the Scandinavian Cystic Fibrosis Nutritional Study

Pincikova

Nilsson²,

Moen³

et al. 2010

Eur J Clin Nutr

View full text Add to dashboard Cite

Background/Objectives: The hallmark of cystic fibrosis (CF) is chronic lung inflammation. The severity of lung disease is closely correlated with immunoglobulin G (IgG) levels. Beyond its contribution to the bone health, the importance of vitamin D has not been fully recognized owing to the lack of human studies providing evidence of its benefit. In the context of the recently described immunomodulatory functions of vitamin D, we aimed to assess the relationship between vitamin D and IgG levels. Subjects/Methods: Eight hundred and ninety-six CF patients were included (0.53-65.9 years) from seven centers in Denmark, Norway and Sweden. Serum 25-hydroxyvitamin D (25OHD) and total IgG were measured, spirometry was carried out and vitamin D intake data were gathered using a 7-day dietary food record. Multiple linear regression analyses were performed for IgG and forced expiratory volume in 1ls (FEV1) as dependent variables, and serum 25OHD, daily food and supplemented vitamin D sources of intake as independent variables. The model was controlled for age, gender, genotype, CF-related diabetes, season, infection/colonization status, long-term oral corticosteroid treatment, long-term treatment with macrolide antibiotics, pancreatic insufficient phenotype and body mass index z-score. Results: Serum total IgG levels were negatively associated with serum 25OHD (adjusted R 2 ¼ 0.376; beta ¼ À0.02; Po0.001), supplemented vitamin D intake per kg bodyweight (adjusted R 2 ¼ 0.375; beta ¼ À0.82; Po0.001) and total vitamin D intake per kg bodyweight (adjusted R 2 ¼ 0.398; beta ¼ À0.60; P ¼ 0.002). Serum 25OHD was positively associated with FEV1 (adjusted R 2 ¼ 0.308; beta ¼ 0.0007; P ¼ 0.025). Conclusions: Increasing vitamin D intake may positively modulate inflammation in CF. This study supports the proposed role of vitamin D in the immune system during infection and substantiates prospective studies.

show abstract

“…Early in the disease course the bacterial pathogens frequently found in the bronchial secretions are Staphylococcus aureus and Haemophilus influenzae. Later the airways of these subjects are invariably colonized with Pseudomonas aeruginosa, an event that initiates an acclerated deterioration of lung functions [3] as wellas an excessive systemic immune response [6]. These pathogens cannot be eliminated from the airways by antimicrobial therapy, and because the deleterious effect of P. aeruginosa infection in individuals with cystic fibrosis is well established, the identification of factors favoring colonization of the airways with P. aeruginosa is important.…”

mentioning

confidence: 99%

Fibronectin-Cleaving Activity in Bronchial Secretions of Patients with Cystic Fibrosis

Suter

Schaad²,

Morgenthaler³

et al. 1988

Journal of Infectious Diseases

View full text Add to dashboard Cite

In cystic fibrosis, colonization of the airways with Pseudomonas aeruginosa follows colonization with Staphylococcus aureus and is related to accelerated deterioration of pulmonary function. Because P. aeruginosa adheres better to cell surfaces devoid of fibronectin, we searched for fibronectin-cleaving activity in bronchial secretions and saliva from 24 patients with cystic fibrosis who were followed up for 4.5 y and from two control groups. Proteolytic activity against 12sI-labeled fibronectin was continuously present in cysticfibrosis bronchial secretions; significantly higher fibronectin-cleaving activity was found in older vs. younger patients, in patients with advanced disease stages determined by a five-stage scoring system, and in those colonized with P. aeruginosa. The fibronectin-cleaving activity was due to neutrophil elastase and cathepsin G. Cystic fibrosis bronchial secretions had proteolytic activity against surface fibronectin of airway mucosal cells. Thus fibronectin-cleaving activity of bronchial secretions rather than of saliva may favor P. aeruginosa colonization of the upper respiratory tract in individuals with cystic fibrosis.Most individuals with cystic fibrosis who survive beyond the neonatal period die from respiratory failure, which is the consequence of chronic, progressively destructive bronchitis [1][2][3]. Clinical and pathological observations show extensive tissue destruction in the airways, which first causes obstruction and obliteration of small airways and thereafter destruction of the walls of large airways [4,5]. Early in the disease course the bacterial pathogens frequently found in the bronchial secretions are Staphylococcus aureus and Haemophilus influenzae. Later the airways of these subjects are invariably colonized with Pseudomonas aeruginosa, an event that initiates an acclerated deterioration of lung functions [3] as wellas an excessive systemic immune response [6]. These pathogens cannot be eliminated from the airways by antimicrobial therapy, and because the deleterious effect of P. aeruginosa infection in individuals with cystic fibrosis is well established, the identification of factors favoring colonization of the airways with P. aeruginosa is important. Coloniza-

show abstract

Progression of cystic fibrosis lung disease as a function of serum immunoglobulin G levels: A 5-year longitudinal study

Cited by 108 publications

References 18 publications

Immunoglobulin E deficiency: a forgotten clue pointing to possible immunodeficiency?

Immunoglobulin E deficiency: a forgotten clue pointing to possible immunodeficiency?

Inverse relation between vitamin D and serum total immunoglobulin G in the Scandinavian Cystic Fibrosis Nutritional Study

Fibronectin-Cleaving Activity in Bronchial Secretions of Patients with Cystic Fibrosis

Contact Info

Product

Resources

About