Usual interstitial pneumonia an overview sciencedirect. We aimed to develop a molecular test that distinguishes usual interstitial pneumonia from other interstitial lung diseases in surgical lung biopsy samples. The scarring involves the supporting framework of the lung. Idiopathic interstitial pneumonias iips are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic patterns on lung biopsy. The diagnosis of uip can be established by surgical lung biopsy or by highresolution thinsection computed tomographic ct scans provided the radiographic features are classical. Some patients with idiopathic nsip subsequently develop collagen vascular diseases kono m et al. Interstitial pneumonia msd manual consumer version. On imaging, uip usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing. Nonspecific interstitial pneumonia cleveland clinic. It can occur in children, usually those infected with the human immunodeficiency virus hiv. Idiopathic pulmonary fibrosis ipfusual interstitial pneumonia uip is not wellunderstood. Usual interstitial pneumonia surgical pathology criteria. Desquamative interstitial pneumonia desquamative interstitial pneumonia diffuse alveolar damage acute interstitial pneumonia organising pneumonia cryptogenic organising pneumonia lymphoid interstitial pneumonia lymphoid interstitial pneumonia atsers subdivision of nsip cellular fibrotic atsers workshop ajrccm 2008. The findings of hrct are compatible with usual interstitial pneumonia uip, which refers to a morphological pattern of interstitial lung disease.
The brazilian thoracic association guidelines for interstitial lung diseases have recently been published. Case discussion uip is a histological term adopted to describe a pattern of interstitial fibrosis characterized by peripheral and basal interstitial fibrosis and presence of honeycombing cysts. Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular. 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. On imaging, the most common features are relatively symmetric. Specifically, the disease involves damage to the interstitium, which renders protection to the air sacs. Usual interstitial pneumonia uip is a histologic pattern characterized by nonuniform, lower zone, subpleural, and paraseptal predominant lung injury defined by geographic heterogeneity and architectural distortion. Since the publication of the first bts guidelines for diffuse lung disease nearly 10years ago,1 the. In addition to the pathologic findings in the morphology of the lung, uip may be from a known or unknown cause. It may occur when an injury to the lungs triggers an abnormal healing. In conformity with the official 2011 american thoracic society statement, idiopathic pulmonary fibrosis ipf is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in. Routine chest radiographs are not sensitive, and the reported rate based on chest xrays was 15%. Acute interstitial pneumonia genetic and rare diseases.
It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Usual interstitial pneumonia uip and idiopathic pulmonary. Nonspecific interstitial pneumonia nsip is a rare disorder that affects the tissue that surrounds and separates the tiny air sacs of the lungs. Fibroblastic foci adjacent to mature collagen, covered by epithelium. Lymphoid interstitial pneumonia can also occur in adults, often those with autoimmune disorders such as plasma cell disorders, sjogren syndrome, hashimoto thyroiditis. An official american thoracic societyeuropean respiratory society. Idiopathic pulmonary fibrosis ipfusual interstitial pneumonia uip, previously known as cryptogenic fibrosing alveolitis cfa in europe, is the most common type of idiopathic interstitial pneumonia iip.
Usual interstitial pneumonia, abbreviated uip, is a relatively common pattern in diffuse lung diseases. Nonspecific interstitial pneumonia and usual interstitial. Pulmonology a condition of middleaged individuals, often associated with connective tissue disease, characterized by insidious deterioration of respiratory function with dyspnea, tachypnea, rightsided heart failure, v lung capacity, v residual volume imaging early, groundglass, linear or nodular markings. Rosane duarte achcar md, in idiopathic pulmonary fibrosis, 2019. Foci of fibroblastic activity and honeycomb change are hallmarks of the uip pattern. Acute and subacute idiopathic interstitial pneumonias taniguchi. Uip is thus classified as a form of interstitial lung disease. The condition tends to progress rapidly, causing irreparable lung damage in as little as one year after symptoms begin and ultimately leading to respiratory failure. Nonspecific interstitial pneumonia nsip is the second most common morphological and pathological pattern of interstitial lung diseases. When groundglass attenuation is seen in other conditions, such as nonspecific interstitial pneumonia nsip or desquamative interstitial pneumonia dip, it is often indicative of a reversible process.
Lymphoid interstitial pneumonia is a form of idiopathic interstitial pneumonia. Most people who have nonspecific interstitial pneumonia are women between the ages of 40 and 50. Interstitial lung disease ild is a group of many lung conditions. Diagnosis of idiopathic pulmonary fibrosis american thoracic. Respiratory bronchiolitisinterstitial lung disease is now com monly diagnosed without. One of the most common and most lethal lung diseases.
Nonspecific interstitial pneumonia nsip 0142549 nsip. Iips are spontaneously occurring ie, idiopathic diffuse parenchymal lung diseases. Idiopathic pulmonary fibrosisusual interstitial pneumonia. Nonspecific interstitial pneumonitis statpearls ncbi bookshelf. Usual interstitial pneumonia desquamative interstitial pneumonia. However, a similar process can develop in people with connective tissue disorders in particular, systemic sclerosis, polymyositis, or dermatomyositis, in some forms of druginduced lung injury, and in. Korean guidelines for diagnosis and management of interstitial. Interstitial lung diseases ilds including connective tissue disease ctd associated ild, hypersensitivity pneumonitis, acute eosinophilic. Usual interstitial pneumonia uip refers to a morphologic entity defined by a combination of 1 patchy interstitial fibrosis with alternating areas of normal lung, 2 temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci in the background of dense acellular collagen, and 3 architectural alteration due to chronic scarring or honeycomb change. Classified into 8 histologic subtypes, all are characterized by varying degrees of inflammation and fibrosis and all cause dyspnea. Acute interstitial pneumonia merck manuals professional edition. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy. Idiopathic nonspecific interstitial pneumonia pulmonary fibrosis. The results showed that the pathological appearance of nonspecific interstitial pneumonia nsip and uip coexisted in his upper lobe.
Differential diagnosis usual interstitial pneumonia. Pathology outlines usual interstitial pneumonia uip. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and ongoing research continues to investigate multiple hypotheses. Idiopathic pulmonary fibrosis ipf is a specific form of chronic interstitial lung disease of unknown cause, associated with histologic and radiologic pattern of usual interstitial pneumonia uip essential features. The fibrosis involves the interstitium of the lung and is therefore rated among the interstitial lung diseases. The multifocal reddish areas are typical of this lesion and correspond to different degrees of severity among pulmonary lobules. Executive summary an update of the 2011 clinical practice guideline ganesh raghu, bram rochwerg, yuan zhang, carlos a. Overview of idiopathic interstitial pneumonias pulmonary.
Features of usual interstitial pneumonia in patients with. Usual interstitial pneumonia coexisted with nonspecific. Of these, nsip is the most common, but uip is not rare. Predictors of diagnosis and survival in idiopathic. These air sacs, called the alveoli, are where the exchange of oxygen and carbon dioxide takes place between the lungs and the bloodstream. Usual interstitial pneumonia uip is a form of lung disease characterized by progressive scarring of both lungs. Idiopathic pulmonary fibrosis abbreviated ipf redirects here.
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. Interstitial pneumonia in pigs is characterized by heavy and noncollapsed lungs with rubbery texture. Acute interstitial pneumonia pulmonary disorders merck. Usual interstitial pneumonia cryptogenic organizing pneumonia. Idiopathic nonspecific interstitial pneumonia nsip is one of the varieties of idiopathic interstitial pneumonias. According to data from registries of interstitial lung diseases ilds in 3 european countries, hp accounts for 4% to 15% of all ild cases 4, but this figure falls to 2% according to a populationbased study conducted in new mexico 5. Interstitial pneumonitis interstitial lung disease center.
Idiopathic nonspecific interstitial pneumonia, or idiopathic nsip, is a chronic lung disease in which inflammation andor scar tissue fibrosis builds up in the. This lesion is highly indicative of viral pulmonary infection, namely, pcv2 and prrs. Usual interstitial pneumonia uip is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis ipf. Interstitial pneumonia medical definition merriamwebster. Synonyms for interstitial pneumonia in free thesaurus. Usual interstitial pneumonia radiology reference article. It is particularly unclear how nsip and usual interstitial pneumonia uip are related. The fibrosis involves the interstitium of the lung and is therefore rated among the interstitial lung diseases in addition to the pathologic findings in the morphology of the lung, uip may be from a known or unknown cause and should be investigated for. The scarring involves the supporting framework interstitium of the lung. Interstitial pneumonitis interstitial lung disease. The term idiopathic pulmonary fibrosis is applied solely to the clinical syndrome associated with the morphologic pattern of uip.
Usual interstitial pneumonia an overview sciencedirect topics. Interstitial pneumonia synonyms, interstitial pneumonia. However, the incidence and prevalence of hp are difficult to estimate with precision, mainly because of. Nitrofurantoin toxicity 004026 004026 nitrofurantoin toxicity as cellular nsip pattern vr11811 fibrotic nsip with slight inhomogeneity. Idiopathic interstitial pneumonias usual interstitial. Jan 08, 2019 usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue.
Interstitial lung disease ild of unknown etiology in immunocompetent patients is rare in children. Imaging findings is compatible with usual interstitial pneumonia uip pattern fibrosis. Nonspecific interstitial pneumonia nsip has recently been proposed as a histologic type of idiopathic interstitial pneumonia iip, but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. Architecture destroyed, honeycomb change prominent. Nonspecific interstitial pneumonia nsip is one class of idiopathic interstitial pneumonia iip. Granulation tissue foci without significant collagen. A usual interstitial pneumonia pattern is a hallmark of idiopathic pulmonary fibrosis and is essential for its diagnosis. In the early stages of the condition, affected people may experience upper respiratory andor virallike symptoms such as cough, shortness of breath, and fever. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually those 40 years. Hammanrich syndrome, also known as acute interstitial pneumonia, is a rare and fulminant form of idiopathic interstitial lung disease. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Ipf is characterized by progressive worsening of dyspnea and lung.
Usual interstitial pneumonia uip is a chronic lung disease characterized by the progressive scarring of both lungs. Interstitial pneumonia is a disease in which the meshlike. Interstitial pneumonia atlas of swine pathology pig333. Pathogenesis and natural history of usual interstitial. Pathogenesis and natural history of usual interstitial pneumonia. Jan 02, 2020 usual interstitial pneumonia uip refers to a morphologic entity defined by a combination of 1 patchy interstitial fibrosis with alternating areas of normal lung, 2 temporal heterogeneity of fibrosis characterized by scattered fibroblastic foci in the background of dense acellular collagen, and 3 architectural alteration due to chronic. A complete understanding of the natural history of ipf could potentially help to identify different mechanisms that are operative at the early.
May 05, 2020 usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. A national survey was carried out in the united kingdom and ireland over a 3year period in. Nonspecific interstitial pneumonia radiology reference. Pdf clinical management of acute interstitial pneumonia. Acute interstitial pneumonitis is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome ards but it is distinguished from the chronic forms of interstitial pneumonia such as idiopathic pulmonary fibrosis. Usual interstitial pneumonia diagnostic hrct criteria for usual interstitial pneumonia uip pattern atsersjrsalat 2018 24 public playlist includes this case.
The term acute interstitial pneumonia aip describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory. Differentiating between nonspecific and usual interstitial. Usual interstitial pneumonia uip is a distinct histological lesion observed in idiopathic pulmonary fibrosis ipf but can be found in other etiologies. All interstitial lung diseases affect the interstitium, a part of your lungs. Aip is similar in presentation to the acute respiratory distress syndrome ards and probably.
Aip is classified as an idiopathic interstitial pneumonia iip, and among the iips, it has the most acute onset and rapidly progressive course. The id iopathic interstitial pneumonias include the entities of idiopathic pulmonary fibrosis ipf, nonspecific interstitial pneumonia. Idiopathic pulmonary fibrosis ipf, the most common and severe among idiopathic interstitial pneumonias, has now been definitively recognized as a distinct clinical entity, defined in the american thoracic societyeuropean respiratory society atsers consensus statement as a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the. Sep 05, 2014 nonspecific interstitial pneumonia nsip has recently been proposed as a histologic type of idiopathic interstitial pneumonia iip, but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. Chronic and progressive respiratory failure due to fibrosis in the lung. Acute interstitial pneumonitis is a rare, severe lung disease that usually affects otherwise healthy individuals. Diagnostic value of kl6 in idiopathic interstitial pneumonia ncbi. Treatment and prognosis depends upon the type of lung disease. The most common symptoms are a dry cough and shortness of breath.
Jan 03, 2016 usual interstitial pneumonia is a form of lung disease characterized by progressive scarring of both lungs. In this study, we analyzed the lesions of right lung removed from a 58yearold patient by gross and microscopy. Patients with acute exacerbation may show a combination of features of diffuse alveolar damage or cryptogenic organizing pneumonia and uip defining feature of dad hyaline membranes, intact or organizing. It should be considered as a cause of idiopathic acute. Prevalence of interstitial pneumonia in rheumatoid arthritis the reported prevalence of interstitial pneumonia in rheumatoid arthritis varies markedly, depending on the criteria used to define disease and also the study population. Classification of usual interstitial pneumonia in patients. Idiopathic interstitial pneumonias california thoracic society.
Interstitial lung disease guideline british thoracic society. Acute interstitial pneumonia aip is a rare and fulminant idiopathic pulmonary disorder that manifests similarly to acute respiratory distress syndrome ards. Jul 22, 2017 acute interstitial pneumonia aip is a rare and serious condition that affects the lungs. Patients with acute exacerbation may show a combination of features of diffuse alveolar damage or cryptogenic organizing pneumonia and uip defining feature of. Lymphoid interstitial pneumonia merck manuals consumer version. Usual interstitial pneumonia uip is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis ipf on imaging, uip usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing. The eventual goal of this research is to develop a method to. Acute interstitial pneumonia or hammanrich syndrome. Doctors may try to treat usual interstitial pneumonia with. Acute interstitial pneumonia aip is a rare and fulminant form of diffuse lung injury originally described by hamman and rich in 1935. Aug 09, 2016 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. Interstitial lung diseases ilds are diffuse parenchymal lung diseases that affect the lung interstitium, pulmonary alveoli, and bronchioles. Case report open access usual interstitial pneumonia.
551 1001 1403 608 1491 230 537 424 1368 754 438 1476 684 16 563 114 1011 864 873 413 956 65 364 1226 780 408 1031 1352 301 1155 481 1430 663 715 1126 776 1518 762 1340 430 896 168 1290 339 1116 1479 773 1022 225 739 1472