Biomaterial Database

[Histopathology of idiopathic diffuse interstitial lung diseases]. 2010

Maud Rabeyrin, and Sylvie Lantuéjoul

Département de pathologie, Pôle de biologie, CHU A. Michallon, BP 217, F-38043 Grenoble Cedex 09, France.

The diagnosis of interstitial disease requires a multidisciplinary approach and interaction between clinicians, radiologists, and pathologists. Pathologists must identify a model orpatternof histologic lesions that suggests a given interstitial disease, without prejudice to its idiopathic nature. Usual interstitial pneumonia (UIP), the designation used by pathologists for the clinical equivalent, idiopathic pulmonary fibrosis, is characterized histologically by fibrosis with a temporal and spatial heterogeneity, by the presence of fibroblastic foci, and by patches of subpleural and paraseptal honeycomb remodeling. Nonspecific interstitial pneumonia (NSIP) is the principal differential diagnosis for UIP, but contrary to UIP, has a good prognosis. The histologic appearance of NSIP is non-specific, and collagenoses, drug toxicity, immunologic lung diseases, and some infectious processes can present in this form. The diagnosis of NSIP is therefore a provisional diagnosis proposed by pathologists, while awaiting additional clinical and laboratory investigations to identify the cause; it is considered idiopathic only by default.

UI	MeSH Term	Description	Entries
D008168	Lung	Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.	Lungs
D011658	Pulmonary Fibrosis	A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.	Alveolitis, Fibrosing,Idiopathic Diffuse Interstitial Pulmonary Fibrosis,Fibroses, Pulmonary,Fibrosis, Pulmonary,Pulmonary Fibroses,Alveolitides, Fibrosing,Fibrosing Alveolitides,Fibrosing Alveolitis
D003663	Decision Trees	A graphic device used in decision analysis, series of decision options are represented as branches (hierarchical).	Decision Tree,Tree, Decision,Trees, Decision
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208	Acute Disease	Disease having a short and relatively severe course.	Acute Diseases,Disease, Acute,Diseases, Acute
D017563	Lung Diseases, Interstitial	A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.	Diffuse Parenchymal Lung Disease,Diffuse Parenchymal Lung Diseases,Interstitial Lung Disease,Interstitial Lung Diseases,Pneumonia, Interstitial,Pneumonitis, Interstitial,Interstitial Pneumonia,Interstitial Pneumonias,Interstitial Pneumonitides,Interstitial Pneumonitis,Lung Disease, Interstitial,Pneumonias, Interstitial,Pneumonitides, Interstitial
D054988	Idiopathic Interstitial Pneumonias	A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.	Idiopathic Interstitial Pneumonia,Interstitial Pneumonia, Idiopathic,Interstitial Pneumonias, Idiopathic,Pneumonia, Idiopathic Interstitial,Pneumonias, Idiopathic Interstitial
D054990	Idiopathic Pulmonary Fibrosis	A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.	Cryptogenic Fibrosing Alveolitis,Familial Idiopathic Pulmonary Fibrosis,Fibrocystic Pulmonary Dysplasia,Fibrosing Alveolitis, Cryptogenic,Idiopathic Fibrosing Alveolitis, Chronic Form,Idiopathic Pulmonary Fibrosis, Familial,Interstitial Pneumonitis, Usual,Pulmonary Fibrosis, Idiopathic,Usual Interstitial Pneumonia,Cryptogenic Fibrosing Alveolitides,Dysplasia, Fibrocystic Pulmonary,Fibrocystic Pulmonary Dysplasias,Fibrosing Alveolitides, Cryptogenic,Idiopathic Pulmonary Fibroses,Interstitial Pneumonia, Usual,Pneumonitides, Usual Interstitial,Pneumonitis, Usual Interstitial,Pulmonary Dysplasia, Fibrocystic,Pulmonary Fibroses, Idiopathic,Usual Interstitial Pneumonias,Usual Interstitial Pneumonitides,Usual Interstitial Pneumonitis
D018549	Cryptogenic Organizing Pneumonia	An interstitial lung disease of unknown etiology, occurring between 21-80 years of age. It is characterized by a dramatic onset of a "pneumonia-like" illness with cough, fever, malaise, fatigue, and weight loss. Pathological features include prominent interstitial inflammation without collagen fibrosis, diffuse fibroblastic foci, and no microscopic honeycomb change. There is excessive proliferation of granulation tissue within small airways and alveolar ducts.	BOOP Bronchiolitis Obliterans Organizing Pneumonia,Bronchiolitis Obliterans Organizing Pneumonia,Primary Organizing Pneumonia,Cryptogenic Organizing Pneumonias,Organizing Pneumonia, Cryptogenic,Organizing Pneumonia, Primary,Organizing Pneumonias, Primary,Pneumonia, Cryptogenic Organizing,Pneumonia, Primary Organizing,Primary Organizing Pneumonias