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Idiopathic pulmonary fibrosis.

Semin Respir Crit Care Med. 2012 Oct;33(5):433-9

Authors: Adkins JM, Collard HR

Abstract
Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive, chronic fibrosing interstitial lung disease occurring primarily in older adults and limited to the lungs. The prognosis is dire, with half of all patients progressing to death from respiratory failure within 3 to 5 years from initial diagnosis. The pathogenesis of IPF is complex and incompletely understood, and the natural history of this disease is variable and unpredictable. There is no widely accepted treatment for IPF, except for lung transplantation. Nevertheless, these are important and exciting times for the diagnosis and management of patients with IPF. Novel diagnostic approaches (e.g., biomarker-based), improved prognostic models, and clinical trials of novel drug agents may alter disease management substantially over the next few years. For now, clinicians should practice comprehensive management, including symptom-based management, aggressive management of comorbidities, and patient education and support.

PMID: 23001798 [PubMed - in process]

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Pulmonary alveolar proteinosis.

Semin Respir Crit Care Med. 2012 Oct;33(5):498-508

Authors: Wang T, Lazar CA, Fishbein MC, Lynch JP

Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant lipids and protein in the alveolar spaces, with resultant impairment in gas exchange. The clinical course can be variable, ranging from spontaneous resolution to respiratory failure and death. PAP in all forms is caused by excessive accumulation of surfactant within the alveolar spaces. Autoimmune PAP accounts for the vast majority of cases in humans and is caused by autoantibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF), which results in impaired catabolism and clearance of surfactant lipids and proteins. Inherited or congenital forms of PAP are exceptionally rare and caused by mutations of genes encoding for surfactant proteins. Secondary forms of PAP are associated with diverse clinical disorders and are caused by reduced alveolar macrophage numbers or function with resultant reduced pulmonary clearance of surfactant. PAP is characterized by progressive exertional dyspnea and nonproductive cough with hypoxemia. Bilateral infiltrates are typically present on chest radiograph, and high-resolution computed tomography reveals diffuse ground-glass opacities and airspace consolidation with interlobular septal thickening in a characteristic "crazy paving" pattern. Although surgical lung biopsy will provide a definitive diagnosis, a combination of typical clinical and imaging features with periodic acid-Schiff (PAS)-positive material on bronchoalveolar lavage and transbronchial biopsies is usually sufficient. The standard of care for treatment of PAP remains whole lung lavage, but treatment is not required in all patients. Autoimmune PAP has also been successfully treated with GM-CSF, both inhaled and systemic, but the optimal dose, duration, and route of administration of GM-CSF have not been elucidated.

PMID: 23001804 [PubMed - in process]

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Obliterative (constrictive) bronchiolitis.

Semin Respir Crit Care Med. 2012 Oct;33(5):509-32

Authors: Lynch JP, Weigt SS, Derhovanessian A, Fishbein MC, Gutierrez A, Belperio JA

Abstract
Obliterative bronchiolitis (OB) (formerly termed bronchiolitis obliterans), is a rare fibrotic disorder involving terminal and respiratory bronchioles. The term constrictive bronchiolitis is synonymous with OB. Clinically, OB is characterized by progressive (often fatal) airflow obstruction, the absence of parenchymal infiltrates on chest radiographs, a mosaic pattern of perfusion on high-resolution computed tomographic scan, poor responsiveness to therapy, and high mortality rates. Most cases of OB occur in the context of a specific risk factor. Currently, most cases of OB occur in lung transplant recipients with chronic allograft rejection or hematopoietic stem cell transplant (HSCT) recipients with graft versus host disease (GVHD). Other causes of OB include connective tissue disease (CTD) (particularly rheumatoid arthritis); lower respiratory tract infections; inhalation injury; exposure or inhalation of toxic fumes, metals, dusts, particulate matter, or pollutants; occupational exposures; drug reactions; consumption of uncooked leaves of Sauropus androgynus; chronic hypersensitivity pneumonia; diffuse neuroendocrine cell hyperplasia; miscellaneous. When no cause is identified, the term cryptogenic obliterative bronchiolitis is used. This review discusses the salient clinical, radiographic, and histological features of OB and presents a management approach.

PMID: 23001805 [PubMed - in process]

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Diffuse lung diseases in cigarette smokers.

Semin Respir Crit Care Med. 2012 Oct;33(5):533-42

Authors: Vassallo R

Abstract
Cigarette smoking is a recognized causative agent or precipitant of specific diffuse lung diseases characterized by bronchiolar and interstitial lung inflammation. Respiratory bronchiolitis-associated interstitial lung disease and pulmonary Langerhans cell histiocytosis are now considered smoking-induced diffuse lung diseases. Desquamative interstitial pneumonia is also recognized as a smoking-induced interstitial pneumonia in most cases. These disorders affect relatively young adult smokers and may be progressive. Although distinguishable by histopathological and radiographic features, significant overlap occurs in many cases with chest radiography and lung histology showing overlapping features of smoking-related bronchiolar and interstitial lung injury. Cigarette smoking is also recognized as an important precipitant of many acute eosinophilic pneumonia cases. Smokers are at higher risk of developing fibrotic interstitial lung diseases such as idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial lung disease. Certain smokers also develop combined emphysema and lung fibrosis. The avoidance of primary and second-hand cigarette smoke is a critical component of management for patients afflicted with these smoking-induced diffuse lung diseases. The role of corticosteroids and other immunosuppressive treatments in the management of smoking-related interstitial lung diseases remains poorly defined and should be reserved for individuals with progressive disease despite smoking cessation. Understanding mechanisms by which tobacco induces diffuse lung pathology is critical in the pursuit of novel therapeutic approaches for these diseases.

PMID: 23001806 [PubMed - in process]