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Diagnosis and management of interstitial lung disease.

Meyer KC - Transl Respir Med (2014)

Bottom Line: When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life.If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies.Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin USA.

ABSTRACT
The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life. A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies. However, ILD accompanied by extensive fibrosis may be difficult to treat, and the identification of an effective pharmacologic therapy for idiopathic pulmonary fibrosis (IPF) has remained elusive despite the completion of many phase 3 clinical trials over the past decade. Nonetheless, patients with IPF or advanced forms of non-IPF ILD can benefit significantly from detection and treatment of various co-morbid conditions that are often found in patients (especially the elderly patient), and supportive care (oxygen therapy, pulmonary rehabilitation) can have a beneficial impact on quality of life and symptom palliation. Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation.

No MeSH data available.


Related in: MedlinePlus

Suggested approach to the diagnosis of ILD. Abbreviations: BAL = bronchoalveolar lavage fluid; HRCT-high-resolution computed tomography; ILD = interstitial lung disease; VATS = video-assisted thorascopic surgery.
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Fig2: Suggested approach to the diagnosis of ILD. Abbreviations: BAL = bronchoalveolar lavage fluid; HRCT-high-resolution computed tomography; ILD = interstitial lung disease; VATS = video-assisted thorascopic surgery.

Mentions: Bronchoscopy and/or surgical lung biopsy may be required to make a confident diagnosis of a specific ILD (FigureĀ 2). Bronchoscopy is generally a very safe procedure if performed by an experienced bronchoscopist [6], and the most serious potential complications are pneumothorax or excessive bleeding that may occur as a consequence of transbronchial biopsy (TBLBx). Bronchoalveolar lavage (BAL) can be readily performed, and the recently published, ATS Task Force Report on BAL for the diagnosis of ILD recommends using recently obtained HRCT imaging to choose an appropriate segment of the lung in which to perform BAL from a wedge position [7]. The right middle lobe or lingula of the left upper lobe are likely the best regions to perform lavage when diffuse disease is present, and areas with ground-glass opacification or profuse nodular change are more likely to provide useful diagnostic information (e.g. differential cell count of nucleated immune cells) than areas with extensive fibrosis. In addition to total and differential BAL cell counts, BAL fluid and sediment can be analyzed for infection or the presence of malignant cells, and the gross appearance of freshly retrieved BAL fluid may provide diagnosistic information (e.g. progressively increasing blood in sequential aliquots that is seen with diffuse alveolar hemorrhage or white-tan discoloration of the BAL fluid with rapidly settling tan sediment [due to gravity] that can be seen with pulmonary alveolar proteinosis). Significant BAL lymphocytosis or eosinophilia may provide strong support for a specific diagnosis when combined with imaging and clinical data, but routine determination of BAL lymphocyte subsets is unlikely to provide additional useful information [7, 8].Figure 2


Diagnosis and management of interstitial lung disease.

Meyer KC - Transl Respir Med (2014)

Suggested approach to the diagnosis of ILD. Abbreviations: BAL = bronchoalveolar lavage fluid; HRCT-high-resolution computed tomography; ILD = interstitial lung disease; VATS = video-assisted thorascopic surgery.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4215823&req=5

Fig2: Suggested approach to the diagnosis of ILD. Abbreviations: BAL = bronchoalveolar lavage fluid; HRCT-high-resolution computed tomography; ILD = interstitial lung disease; VATS = video-assisted thorascopic surgery.
Mentions: Bronchoscopy and/or surgical lung biopsy may be required to make a confident diagnosis of a specific ILD (FigureĀ 2). Bronchoscopy is generally a very safe procedure if performed by an experienced bronchoscopist [6], and the most serious potential complications are pneumothorax or excessive bleeding that may occur as a consequence of transbronchial biopsy (TBLBx). Bronchoalveolar lavage (BAL) can be readily performed, and the recently published, ATS Task Force Report on BAL for the diagnosis of ILD recommends using recently obtained HRCT imaging to choose an appropriate segment of the lung in which to perform BAL from a wedge position [7]. The right middle lobe or lingula of the left upper lobe are likely the best regions to perform lavage when diffuse disease is present, and areas with ground-glass opacification or profuse nodular change are more likely to provide useful diagnostic information (e.g. differential cell count of nucleated immune cells) than areas with extensive fibrosis. In addition to total and differential BAL cell counts, BAL fluid and sediment can be analyzed for infection or the presence of malignant cells, and the gross appearance of freshly retrieved BAL fluid may provide diagnosistic information (e.g. progressively increasing blood in sequential aliquots that is seen with diffuse alveolar hemorrhage or white-tan discoloration of the BAL fluid with rapidly settling tan sediment [due to gravity] that can be seen with pulmonary alveolar proteinosis). Significant BAL lymphocytosis or eosinophilia may provide strong support for a specific diagnosis when combined with imaging and clinical data, but routine determination of BAL lymphocyte subsets is unlikely to provide additional useful information [7, 8].Figure 2

Bottom Line: When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life.If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies.Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin USA.

ABSTRACT
The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life. A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies. However, ILD accompanied by extensive fibrosis may be difficult to treat, and the identification of an effective pharmacologic therapy for idiopathic pulmonary fibrosis (IPF) has remained elusive despite the completion of many phase 3 clinical trials over the past decade. Nonetheless, patients with IPF or advanced forms of non-IPF ILD can benefit significantly from detection and treatment of various co-morbid conditions that are often found in patients (especially the elderly patient), and supportive care (oxygen therapy, pulmonary rehabilitation) can have a beneficial impact on quality of life and symptom palliation. Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation.

No MeSH data available.


Related in: MedlinePlus