Idiopathic interstitial pneumonia | |
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Other names | Noninfectious pneumonia |
Micrograph of usual interstitial pneumonia (UIP). UIP is the most common pattern of idiopathic interstitial pneumonia and usually represents idiopathic pulmonary fibrosis. H&E stain. Autopsy specimen. | |
Specialty | Respirology |
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia [1] are a class of diffuse lung diseases. These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis). There are seven recognized distinct subtypes of IIP. [2]
Classification can be complex, [3] and the combined efforts of clinicians, radiologists, and pathologists can help in the generation of a more specific diagnosis. [4] [5]
Idiopathic interstitial pneumonia can be subclassified based on histologic appearance into the following patterns: [6] [7]
Histology | Clinical Correlates |
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Desquamative interstitial pneumonia (DIP) | DIP |
Diffuse alveolar damage (DAD) | ARDS, AIP, TRALI |
Nonspecific interstitial pneumonia (NSIP) | NSIP |
Respiratory bronchiolitis | RB-ILD |
Usual interstitial pneumonia (UIP) | CVD, IPF, drug toxicity, pneumoconiosis |
Organizing pneumonia | Cryptogenic organizing pneumonia |
Lymphoid interstitial pneumonia (LIP) | LIP |
Usual interstitial pneumonia is the most common type. [8]
Table 1: Development of the (histologic) idiopathic interstitial pneumonia classification
Leibow et al. (1969) | Katzenstein (1998) [9] | ATS/ERS (2002) [7] | |||||||||||||||||||||||||||
UIP | UIP | ||||||||||||||||||||||||||||
UIP | DAD | DAD | |||||||||||||||||||||||||||
NSIP | NSIP | ||||||||||||||||||||||||||||
DIP | DIP/RB | DIP | |||||||||||||||||||||||||||
RB | |||||||||||||||||||||||||||||
BIP | OP | OP | |||||||||||||||||||||||||||
LIP | (LPD) | LIP | |||||||||||||||||||||||||||
GIP | (HMF) | (HMF) | |||||||||||||||||||||||||||
UIP=usual interstitial pneumonia; DAD=diffuse alveolar damage; NSIP=non-specific interstitial pneumonia; DIP=desquamative interstitial pneumonia; RB=respiratory bronchiolitis; BIP=bronchiolitis obliterans interstitial pneumonia; OP=organizing pneumonia; LIP=lymphoid interstitial pneumonia; LPD=lymphoproliferative disease (not considered a diffuse lung disease); GIP=giant cell interstitial pneumonia; HMF=heavy metal fibrosis, no longer grouped with diffuse lung disease
Lymphoid interstitial pneumonia was originally included in this category, then excluded, then included again. [10]
Diffusing capacity of the lung (DL) measures the transfer of gas from air in the lung, to the red blood cells in lung blood vessels. It is part of a comprehensive series of pulmonary function tests to determine the overall ability of the lung to transport gas into and out of the blood. DL, especially DLCO, is reduced in certain diseases of the lung and heart. DLCO measurement has been standardized according to a position paper by a task force of the European Respiratory and American Thoracic Societies.
Asbestosis is long term inflammation and scarring of the lungs due to asbestos fibers. Symptoms may include shortness of breath, cough, wheezing, and chest tightness. Complications may include lung cancer, mesothelioma, and pulmonary heart disease.
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases affecting the interstitium (the tissue and space around the alveoli. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process goes awry and the tissue around the air sacs becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The disease presents itself with the following symptoms: shortness of breath, nonproductive coughing, fatigue, and weight loss, which tend to develop slowly, over several months. The average rate of survival for someone with this disease is between three and five years. The term ILD is used to distinguish these diseases from obstructive airways diseases.
Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.
Hypersensitivity pneumonitis (HP) or extrinsic allergic alveolitis (EAA) is a rare immune system disorder that affects the lungs. It is an inflammation of the alveoli (airspaces) within the lung caused by hypersensitivity to inhaled organic dusts. Sufferers are commonly exposed to the dust by their occupation or hobbies.
Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans organizing pneumonia (BOOP), is an inflammation of the bronchioles (bronchiolitis) and surrounding tissue in the lungs. It is a form of idiopathic interstitial pneumonia.
Bronchopulmonary dysplasia is a chronic lung disease in which premature infants, usually those who were treated with supplemental oxygen, require long-term oxygen. The alveoli that are present tend to not be mature enough to function normally. It is more common in infants with low birth weight (LBW) and those who receive prolonged mechanical ventilation to treat respiratory distress syndrome (RDS). It results in significant morbidity and mortality. The definition of BPD has continued to evolve primarily due to changes in the population, such as more survivors at earlier gestational ages, and improved neonatal management including surfactant, antenatal glucocorticoid therapy, and less aggressive mechanical ventilation.
Idiopathic pulmonary fibrosis (IPF) is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. The tissue in the lungs becomes thick and stiff, which affects the tissue that surrounds the air sacs in the lungs. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism.
Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the supporting framework (interstitium) of the lung. UIP is thus classified as a form of interstitial lung disease.
Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.
Pulmonary function testing (PFT) is a complete evaluation of the respiratory system including patient history, physical examinations, and tests of pulmonary function. The primary purpose of pulmonary function testing is to identify the severity of pulmonary impairment. Pulmonary function testing has diagnostic and therapeutic roles and helps clinicians answer some general questions about patients with lung disease. PFTs are normally performed by a respiratory therapist, physiotherapist, pulmonologist, and/or general practitioner.
Ventilator-associated lung injury (VALI) is an acute lung injury that develops during mechanical ventilation and is termed ventilator-induced lung injury (VILI) if it can be proven that the mechanical ventilation caused the acute lung injury. In contrast, ventilator-associated lung injury (VALI) exists if the cause cannot be proven. VALI is the appropriate term in most situations because it is virtually impossible to prove what actually caused the lung injury in the hospital.
Pulmonary hygiene, formerly referred to as pulmonary toilet, is a set of methods used to clear mucus and secretions from the airways. The word pulmonary refers to the lungs. The word toilet, related to the French toilette, refers to body care and hygiene; this root is used in words such as toiletry that also relate to cleansing.
Desquamative interstitial pneumonia (DIP) is a form of idiopathic interstitial pneumonia featuring elevated numbers of macrophages within the alveoli of the lung. The alveolar macrophages have a characteristic light brown pigmentation and accumulate in the alveolar lumen and septa regions of the lower lobes of the lungs. The typical effects of the macrophage accumulation are inflammation and later fibrosis of the lung tissue.
Respiratory bronchiolitis interstitial lung disease refers to a form of idiopathic interstitial pneumonia associated with smoking.
Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia.
Pulmonary rehabilitation, also known as respiratory rehabilitation, is an important part of the management and health maintenance of people with chronic respiratory disease who remain symptomatic or continue to have decreased function despite standard medical treatment. It is a broad therapeutic concept. It is defined by the American Thoracic Society and the European Respiratory Society as an evidence-based, multidisciplinary, and comprehensive intervention for patients with chronic respiratory diseases who are symptomatic and often have decreased daily life activities. In general, pulmonary rehabilitation refers to a series of services that are administered to patients of respiratory disease and their families, typically to attempt to improve the quality of life for the patient. Pulmonary rehabilitation may be carried out in a variety of settings, depending on the patient's needs, and may or may not include pharmacologic intervention.
Inverse ratio ventilation (IRV) is not necessarily a mode of mechanical ventilation though it may be referred to as such. IRV is a strategy of ventilating the lungs in such a way that the amount of time the lungs are in inhalation is greater than the amount of time they are in exhalation, allowing for a constant inflation of the lungs, ensuring they remain "recruited". The primary goal for IRV is improved oxygenation by forcing inspiratory time to be greater than expiratory time increasing the mean airway pressure and potentially improving oxygenation. Normal I:E ratio is 5:6, so forcing the I:E to be 2:1, 3:1, 4:1, is the source of the term for the strategy.
Ground-glass opacity (GGO) is a finding seen on chest x-ray (radiograph) or computed tomography (CT) imaging of the lungs. It is typically defined as an area of hazy opacification (x-ray) or increased attenuation (CT) due to air displacement by fluid, airway collapse, fibrosis, or a neoplastic process. When a substance other than air fills an area of the lung it increases that area's density. On both x-ray and CT, this appears more grey or hazy as opposed to the normally dark-appearing lungs. Although it can sometimes be seen in normal lungs, common pathologic causes include infections, interstitial lung disease, and pulmonary edema.
William N. Rom is the Sol and Judith Bergstein Professor of Medicine and Environmental Medicine, Emeritus at New York University School of Medicine and former Director of the Division of Pulmonary, Critical Care and Sleep Medicine at New York University and Chief of the Chest Service at Bellevue Hospital Center, 1989-2014. He is Research Scientist at the School of Global Public Health at New York University and Adjunct Professor at the NYU Robert F. Wagner Graduate School of Public Service. He teaches Climate Change and Global Public Health.
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