Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia

Sweinberg, Sharon K.; Wodell, Ruthven A.; Grodofsky, Marshall P.; Greene, Jeremy A.; Conley, Mary Ellen

doi:10.1016/0091-6749(91)90306-9

Cited by 82 publications

(70 citation statements)

References 16 publications

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“…However, the effectiveness in reducing pulmonary complications was demonstrated in a retrospective analysis when treatment was used in conjunction with intramuscular immunoglobulin prior to the availability of the currently used i.v. or s.c. preparations (130).…”

Section: Prophylactic Antibioticsmentioning

confidence: 99%

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

2009

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SUMMARY Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.

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Section: Prophylactic Antibioticsmentioning

confidence: 99%

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

2009

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“…While encapsulated organisms such as Streptococcus pneumoniae or atypicals such as Mycobacteria are the most commonly identified pathogens, patients may also present with infections typically associated with T cell defects such as Pneumocystis jiroveci [14,19]. As might be expected, it has long been recognized that chronic inflammation and recurrent infections associated with CVID may result in severe bronchiectasis and pulmonary fibrosis [19,35,38,39,40,41,42,43,44,45,46,47,48], possibly influenced by mannose-binding lectin polymorphisms [42]. Further, patients with CVID are at risk for granulomatous infiltration and interstitial pneumonia in a pattern termed GLILD (granulomatous/lymphocytic interstitial lung disease) [19,44,48,49,50,51].…”

Section: Cvid Clinical Featuresmentioning

confidence: 99%

Common Variable Immunodeficiency: Etiological and Treatment Issues

Deane

Selmi

Naguwa

et al. 2009

Int Arch Allergy Immunol

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One of the great advances in clinical medicine was the recognition of the pleomorphism of the immune response and the multiple afferent and efferent limbs of antigen processing and responsiveness. A significant contribution to this understanding was derived from studies of human immunodeficiency states, including both inherited and acquired syndromes. Amongst these syndromes, one of the most common, and least understood, is common variable immune deficiency (CVID). CVID is a syndrome that leads to a reduction in serum immunoglobulins and complications including recurrent infections. Management includes immunoglobulin replacement therapy; however, patients with CVID are at risk for complications of exogenous immunoglobulin administration as well as CVID-associated diseases such as autoimmune processes and malignancies. To assess the current state of knowledge in the field, we performed a literature review of a total of 753 publications covering the period of 1968 until 2008. From this list, 189 publications were selected for discussion. In this review, we demonstrate that while the molecular basis of CVID in many cases remains incompletely understood, significant strides have been made and it is now clear that there is involvement of several pathways of immune activation, with contributions from both T and B cells. Furthermore, despite the current gaps in our knowledge of the molecular pathogenesis of the syndrome, there have been dramatic advances in management that have led to improved survival and significantly reduced morbidity in affected patients.

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“…Central nervous system infection did not occur in this study. For comparison, the number of infections for normal Australian children calculated from the data of 5 Spirometric evidence of obstructive lung disease, reported to be the most common abnormality seen in subjects with hypogammaglobulinaemia, 5 was not seen in any of our subjects. One child had radiographic evidence of bronchiectasis before institution of treatment and two developed permanent radiological changes indicative of bronchiectasis.This may be contrasted with findings in a group of eight boys with agammaglobulinaemia treated at our institution from 1958 to 1972 with intramuscular therapy where on review all had permanent radiological abnormalities on chest radiography.…”

Section: Infectionsmentioning

confidence: 90%