All interstitial lung diseases affect the interstitium, a part of your lungs. The diagnosis and management of interstitial lung diseases. The american thoracic societyeuropean respiratory society classification of idiopathic interstitial pneumonias iips, published in 2002, defines the morphologic patterns on which clinicalradiologicpathologic diagnosis of iips is based. All interstitial lung diseases affect the interstitium, a part of the lungs anatomic structure. Plain film and hrct diagnosis of interstitial lung disease. Among the 300plus interstitial lung diseases, the most important are sarcoidosis, idiopathic pulmonary fibrosis, extrinsic allergic alveolitis, interstitial lung disease as a feature of connectivetissuedisease,druginduced interstitial lung disease and pneumoconiosis. Secondary pulmonary lobule is a fundamental unit of lung structure and the key to understanding the distribution of diffuse lung disease 2. Pdf radiologic diagnosis of interstitial lung diseases. The most common symptoms are a dry cough and shortness of breath. Correspondence the new england journal of medicine 1486 n engl j med 381.
Objectives despite many shared risk factors and pathophysiological pathways, the risk of ischaemic heart disease ihd and myocardial infarction mi in interstitial lung disease ild remains poorly understood. Pdf diagnosis and management of interstitial lung disease. This lack of data could be preventing patients who may benefit from screening for these cardiovascular diseases. Treatment and prognosis depends upon the type of lung disease. Pdf interstitial lung diseases comprise more than 100 entities in which the lung is more or less broadly involved by a diffuse disease, mostly. Exclusion of other known causes of interstitial lung disease eg, toxic effects of certain drugs, environmental ex.
Interstitial lung disease ild is a group of many lung conditions. Nonspecific interstitial pneumonia came to be recognized as a distinct entity, with a variable, heterogeneous clinical course. American thoracic societyeuropean respiratory society. The chronic interstitial lung diseases ilds have variable prognoses, ranging from death within a few weeks of diagnosis to recovery, depending on their rapidity of progression. 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 air sacs of the lungs. Rbild and dip are smokingrelated diseases characterized by centrilobular. The idiopathic interstitial pneumonias radiologic clinics. Interstitial lung disease is the name for a group of diseases that affect the lungs, for example, interstitial pneumonitis, black lung, farmers lung, mold, grasses, fumes, and autoimmune diseases. Ryu, md interstitial lung disease ild comprises an array of heterogeneous parenchymal lung diseases. Respiratory bronchiolitisassociated interstitial lung disease could now. Interstitial lung disease is a risk factor for ischaemic.
Some known causes of interstitial lung disease listed in. Mechanical ventilation in interstitial lung disease chest. Interstitial lung disease is believed to be caused by an. Radiologic pleuroparenchymal fibroelastosislike lesion in connective tissue disease related interstitial lung disease article pdf available in plos one 126. In advanced disease, the chest radiograph shows decreased lung. For ex ample, in patients with the entity of usual interstitial pneumonia, the appearance of the disease on hrct is characteristic enough for an accurate. Idiopathic interstitial pneumonia interstitial lung disease multidisciplinary. Unlike systemic high blood pressure, this condition affects only the arteries in your lungs.
Only about one in three cases of interstitial lung disease. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream. Interstitial lung diseases national heart, lung, and. Interstitial lung disease diagnostic histopathology. Pdf the complex tasks of making a confident diagnosis of a specific form of interstitial. Interstitial lung disease ild describes a large group of conditions that cause inflammation and scar tissue in the lungs. Sjogrens syndrome presenting with interstitial lung disease. Pleural effusion is uncommon in primary ss and is seen more often in secondary ss. Highresolution ct in diffuse interstitial lung disease. The research we fund today will help improve our future health. What every radiologist should know about idiopathic. Other interstitial lung diseases, such as respira tory bronchiolitis associated interstitial lung disease, are self limiting and potentially reversible.
Interstitial lung disease symptoms and causes mayo clinic. Approximately 4050% patients with dermatomyositis suffer from its pulmonary complications, which include interstitial lung diseases, pneumonia, drug induced lung diseases. The american thoracic societyeuropean respiratory society classification of idiopathic interstitial pneumonias iips, published in 2002, defines the morphologic patterns on which clinicalradiologicpathologic. In patients with unclassifiable disease, representing around 10% of the interstitial lung disease. Secondary pulmonary lobule represents a polygonal shaped. Interstitial lung disease 10 interstitial lung disease is a term that broadly describes a diverse collection of more than 200 lung disorders.
An overview justin oldham, md ms assistant professor of medicine director, interstitial lung disease program. The interstitium is a lacelike network of tissue that goes throughout both lungs. The role of infection in interstitial lung diseases. Classification of diffuse lung diseases rsna publications online. Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality.
The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. The dilds have been subcategorized into a dilds that have a known etiology, b the idiopathic interstitial pneumonias, c the granulomatous dilds, and d a group of diffuse lung diseases. Interstitial lung disease can lead to a series of lifethreatening complications, including. Depending on the specific disease, other compartments of the lung. Interstitial lung disease resulting from exposure to known causing irritants is sometimes referred to as hypersensitivity pneumonitis table 2. My approach to interstitial lung disease using clinical. Classification of idiopathic interstitial pneumonias. Childhood interstitial lung disease child represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality. In these subjects with ct findings of uip due to either ipf or ctdild, three. What every radiologist should know about idiopathic interstitial.
Interstitial lung disease ild is an umbrella term that encompasses a large number of disorders that are characterized by diffuse cellular infiltrates in a periacinar location. Fibrotic ilds peripheral fibrosis common in idiopathic pulmonary fibrosis. The interstitial lung disease that develops with these entities may show a histologic pattern of uip or nsip, with uip being more common in rheumatoid arthritis, and nsip more common in progressive systemic sclerosis. The interstitial pneumonias are a group of heterogeneous nonneoplastic lung diseases that may be idiopathic or associated with an underlying abnormality. Pdf radiologic pleuroparenchymal fibroelastosislike. A usual interstitial pneumonia uip pattern on chest ct scans is highly suggestive of uip pathologic findings. These diseases are classified together because they all affect the tissue and space around the alveoli air sacs, called the interstitium.
Search the nih reporter to learn about research the nhlbi is funding on interstitial lung diseases. The interstitium is a lacelike network of tissue that extends throughout both lungs. Interstitial lung disease in children younger than 2 years. Although they share some features in common, they also exhibit diverse pulmonary manifestations. The interstitial pneumonias are a group of heterogeneous nonneoplastic lung diseases that. The lung is the most common extramuscular organ to be affected in dermatomyositis. Our division of lung diseases oversees much of the research on interstitial lung diseases we fund, helping us understand and manage the conditions. Knowledge of both, the radiological and clinical appearance of these more common interstitial lung diseases. Interstitial lung disease is a term that broadly describes a diverse collection of. Part 2, the interstitial pneumoniasclinical, histologic. Under current guidelines, a uip pattern on ct images is specific for ipf after a thorough clinical and serologic workup has excluded other causes of interstitial lung disease.
The first systematic studies of computed tomography in diffuse lung disease began in the mid1980s and the development of hrct in the early 1990s was followed by more than 10 years of hrctpathology correlative studies that bridged the gap between the microscopic world of interstitial lung disease. The term interstitial lung disease ild comprises more than 200 separate disease entities, each having its separate and often unique radiological. Interstitial lung disease in children made easier rsna. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial. Interstitial lung disease is a general category that includes many different lung conditions. Respiratory bronchiolitisassociated interstitial lung disease. Functional ageing in fibrotic interstitial lung disease. Interstitial lung disease european respiratory society. Interstitial lung disease radiology reference article. Diffuse interstitial lung diseases dilds comprise a huge number of diseases which diffusely involve the lung parenchyma. Interstitial lung disease ild is an umbrella term used for a large group of diseases that cause scarring fibrosis of the lungs. A number of interstitial lung diseases are associated with such a characteristic pattern on hrct, that diagnosis is strongly promoted by image findings, e. Radiological diagnosis of interstitial lung disease. Ct features of the usual interstitial pneumonia pattern.
High blood pressure in your lungs pulmonary hypertension. Background accelerated biological and functional ageing is common in fibrotic interstitial lung disease ild. Interstitial lung disease refers to inflammation in the interstitial tissue of the lungs, the spaces that surround and separate the air sacs. There are numerous interstitial lung diseases, but in clinical practice only about ten diseases account for approximately 90% of cases. A practical approach to highresolution ct of diffuse lung. Diagnosis and treatment of diffuse interstitial lung diseases.
682 927 1165 343 148 778 624 105 613 1060 1349 1033 778 171 850 1122 822 1168 755 687 1015 82 20 431 583 1483 70 750 1408 554 770 179 282 149 2 1502 683 1442 1423 756 1157 63 334 25 1349 897 134