Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Aug 2007
ReviewPulmonary manifestations of systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) can affect the lung in multiple ways. All components of the respiratory system, including the pleura, pulmonary parenchyma, airways, vessels, and respiratory muscles can be involved in various degrees at some time in the disease course and contribute to its morbidity and mortality. This article reviews the clinical symptoms, imaging techniques, histopathology, prognosis, and treatment of pulmonary manifestations of SLE and the related disorder mixed connective tissue disease (MCTD), from a historical perspective and with regard to new insights into pathogenesis and therapy.
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Sjögren's syndrome (SS) is a chronic, slowly progressive, inflammatory, autoimmune disease characterized by (1) lymphocytic infiltration of the exocrine glands leading to diminished or absent glandular secretion, and (2) marked B-lymphocytic cell hyperreactivity manifested initially by a variety of serum autoantibodies, including those against the Ro(SSA) and La(SSB) ribonucleoproteins, ending in the development of B cell non-Hodgkin's lymphoma in a substantial number of patients. Most patients with SS present only with keratoconjunctivitis sicca and xerostomia. ⋯ Pulmonary manifestations develop in some patients and may present as (1) bronchitis sicca; (2) a wide spectrum of lymphoproliferative diseases, ranging from bronchus-associated lymphoid tissue (BALT) hyperplasia, lymphoid interstitial pneumonia, and B cell non-Hodgkin's lymphoma mainly of the extranodal marginal zone B-cell lymphoma of BALT-type or rarely of higher-grade malignancy; and (3) other interstitial pneumonias. Pleuritis can be seen in SS patients with associated systemic lupus erythematosus or rheumatoid arthritis.
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Semin Respir Crit Care Med · Jun 2007
ReviewEvaluating infants and children with interstitial lung disease.
The field of children's interstitial lung disease (chILD) has been confusing for clinicians and families. It is fraught with imperfect pediatric definitions and classification systems, limited understanding of underlying molecular mechanisms, pathophysiology and natural history, and inadequate clinical networks to improve care. To address these issues two large efforts have focused on chILD: a European Respiratory Society (ERS) Task Force on chronic interstitial lung disease in children and the National Institute of Health (NIH)-sponsored Rare Lung Disease Consortium (RLDC). ⋯ Both efforts have yielded new concepts and approaches to the evaluation of pediatric patients with ILD. Diagnostic techniques have also evolved to provide more advanced testing in children. This article reviews the current evaluation for children with ILD and highlights areas of controversy.
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Semin Respir Crit Care Med · Jun 2007
ReviewEmerging and unusual gram-negative infections in cystic fibrosis.
People with cystic fibrosis (CF) have chronic airway infection and frequent exposure to antibiotics, which often leads to the emergence of resistant organisms. In addition to the development of multiresistance in common CF pathogens such as Pseudomonas aeruginosa, several newer, inherently resistant gram-negative species are becoming more common, including Burkholderia cepacia complex, Stenotrophomonas maltophilia, Achromobacter (Alcaligenes) xylosoxidans, certain Ralstonia species, and those within the new genus Pandoraea. Many of these are closely related and have similar phenotypes, making accurate laboratory identification challenging. Although their role in contributing to pulmonary disease in CF is not clear, some, such as those of the B. cepacia complex, are clearly linked to an adverse prognosis, and both treatment and infection control issues can pose a real challenge.
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Semin Respir Crit Care Med · Jun 2007
ReviewShould preschool wheezers ever be treated with inhaled corticosteroids?
The syndrome of preschool wheeze commonly regresses completely in the preschool years, but it may lead to prolonged symptoms and established asthma. Although epidemiological studies have established that there are several different phenotypes, it is currently impossible to assign the majority of wheezing preschool children to a phenotype prospectively. Bronchoalveolar lavage studies have shown an increase in total cellular inflammation in the youngest, symptomatic children, and that in older preschool children the neutrophil is the predominant inflammatory cell in the airway. ⋯ Unfortunately, neither corticosteroids nor any other currently available therapy modifies the long-term outcome of preschool wheeze. In conclusion, corticosteroid treatment may have a small role in preschool wheeze, in particular for those thought to have early asthma, but the uncritical application of recommendations that are appropriate for older children and adults with asthma has led to widespread overuse of these medications. There is an urgent need for better treatment of preschool wheeze.