Desquamative interstitial pneumonia | |
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Specialty | Pulmonology |
Desquamative interstitial pneumonia (DIP) is a form of idiopathic interstitial pneumonia featuring elevated numbers of macrophages within the alveoli (air sacs) 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. [1] The typical effects of the macrophage accumulation are inflammation and later fibrosis (thickening and stiffness) of the lung tissue. [2]
The term DIP is a misnomer. [3] Its name is derived from the former belief that these macrophages were pneumocytes that had desquamated. [4] It has been suggested that a more accurate term for cases of DIP that occur in smokers and feature "ropey" alveolar septal collagen is Smoking-related interstitial fibrosis (SRIF).
It is associated with patients with a history of smoking. [5] Since more than 80% of cases occur in smokers, it has been suggested that the term DIP should be discarded and the subset occurring in smokers should be replaced with more accurate terms such as smoking-related interstitial fibrosis (SRIF). [3] Although smoking is the most common cause, studies have shown a relationship between occupational exposures and the development of DIP, including occupational dust, fire-extinguisher powder, diesel fumes, nylon filaments and beryllium and copper dust. [6] Additionally, DIP has been observed in children where it typically presents as a result of surfactant protein gene mutations, indicating that the disease is not always acquired in adulthood. [7]
Smoking cessation and avoidance of secondhand smoke exposure are both crucial to preventing disease progression, however, treatment with corticosteroids and immunosuppressive therapy has been reported to be effective pharmacologic intervention. [8] Treatment with methylprednisolone has been reported. [9]
Pneumoconiosis is the general term for a class of interstitial lung disease where inhalation of dust has caused interstitial fibrosis. The three most common types are asbestosis, silicosis, and coal miner's lung. Pneumoconiosis often causes restrictive impairment, although diagnosable pneumoconiosis can occur without measurable impairment of lung function. Depending on extent and severity, it may cause death within months or years, or it may never produce symptoms. It is usually an occupational lung disease, typically from years of dust exposure during work in mining; textile milling; shipbuilding, ship repairing, and/or shipbreaking; sandblasting; industrial tasks; rock drilling ; or agriculture. It is one of the most common occupational diseases in the world.
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by an abnormal accumulation of surfactant-derived lipoprotein compounds within the alveoli of the lung. The accumulated substances interfere with the normal gas exchange and expansion of the lungs, ultimately leading to difficulty breathing and a predisposition to developing lung infections. The causes of PAP may be grouped into primary, secondary, and congenital causes, although the most common cause is a primary autoimmune condition in an individual.
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of respiratory diseases affecting the interstitium (the tissue and space around the alveoli of the lungs. 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 is disrupted, 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 syndrome caused by the repetitive inhalation of antigens from the environment in susceptible or sensitized people. Common antigens include molds, bacteria, bird droppings, bird feathers, agricultural dusts, bioaerosols and chemicals from paints or plastics. People affected by this type of lung inflammation (pneumonitis) are commonly exposed to the antigens by their occupations, hobbies, the environment and animals. The inhaled antigens produce a hypersensitivity immune reaction causing inflammation of the airspaces (alveoli) and small airways (bronchioles) within the lung. Hypersensitivity pneumonitis may eventually lead to interstitial lung disease.
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.
Acute interstitial pneumonitis is a rare, severe lung disease that usually affects otherwise healthy individuals. There is no known cause or cure.
Alveolar lung diseases, are a group of diseases that mainly affect the alveoli of the lungs.
Occupational lung diseases are work-related, lung conditions that have been caused or made worse by the materials a person is exposed to within the workplace. It includes a broad group of diseases, including occupational asthma, industrial bronchitis, chronic obstructive pulmonary disease (COPD), bronchiolitis obliterans, inhalation injury, interstitial lung diseases, infections, lung cancer and mesothelioma. These diseases can be caused directly or due to immunological response to an exposure to a variety of dusts, chemicals, proteins or organisms.
Idiopathic pulmonary fibrosis (IPF), or (formerly) fibrosing alveolitis, is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It 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 pulmonary interstitium. UIP is thus classified as a form of interstitial lung disease.
High-resolution computed tomography (HRCT) is a type of computed tomography (CT) with specific techniques to enhance image resolution. It is used in the diagnosis of various health problems, though most commonly for lung disease, by assessing the lung parenchyma. On the other hand, HRCT of the temporal bone is used to diagnose various middle ear diseases such as otitis media, cholesteatoma, and evaluations after ear operations.
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.
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung diseases. These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways. There are seven recognized distinct subtypes of IIP.
Respiratory bronchiolitis is a lung disease associated with tobacco smoking. In pathology, it is defined by the presence of "smoker's macrophages". When manifesting significant clinical symptoms it is referred to as respiratory bronchiolitis interstitial lung disease (RB-ILD).
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.
Flock worker's lung is an occupational lung disease caused by exposure to flock, small fibers that are glued to a backing in order to create a specific texture. People who work in flocking are at risk of inhaling small pieces of the flock fibers, which causes interstitial lung disease. The disease was initially described in 1998, when a group of workers at a flocking plant developed interstitial lung disease of unknown cause.
Emphysema is any air-filled enlargement in the body's tissues. Most commonly emphysema refers to the enlargement of air spaces (alveoli) in the lungs, and is also known as pulmonary emphysema.
Smoker’s macrophages are alveolar macrophages whose characteristics, including appearance, cellularity, phenotypes, immune response, and other functions, have been affected upon the exposure to cigarettes. These altered immune cells are derived from several signaling pathways and are able to induce numerous respiratory diseases. They are involved in asthma, chronic obstructive pulmonary diseases (COPD), pulmonary fibrosis, and lung cancer. Smoker’s macrophages are observed in both firsthand and secondhand smokers, so anyone exposed to cigarette contents, or cigarette smoke extract (CSE), would be susceptible to these macrophages, thus in turns leading to future complications.
Smoking-related interstitial fibrosis (SRIF) is an abnormality in the lungs characterized by excessive collagen deposition within the walls of the air sacs. This abnormality can be seen with a microscope and diagnosed by pathologists. It is caused by cigarette smoking.