One of the most common and most lethal lung diseases. Classification of usual interstitial pneumonia in patients. 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 ild. Idiopathic pulmonary fibrosis ipf, a progressive and fatal diffuse parenchymal lung disease, is defined pathologically by the pattern of usual interstitial pneumonia uip. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually those 40 years. Executive summary an update of the 2011 clinical practice guideline ganesh raghu, bram rochwerg, yuan zhang, carlos a. Idiopathic pulmonary fibrosisusual interstitial pneumonia. Foci of fibroblastic activity and honeycomb change are hallmarks of the uip pattern. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and ongoing research continues to investigate multiple hypotheses. Usual interstitial pneumonia an overview sciencedirect topics. Usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Acute interstitial pneumonia aip is a rare and fulminant idiopathic pulmonary disorder that manifests similarly to acute respiratory distress syndrome ards. Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia.
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. With atypical pneumonia will also have certain extrapulmonary symptoms that others with typical pneumonia will often not have. Usual interstitial pneumonia uip is the most common idiopathic interstitial pneumonia and the underlying histology in cases of idiopathic pulmonary fibrosis. 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.
Minor histopathological features in usual interstitial pneumonia normal dense scar dense scar micro honeycombing this is uip fibroblast focus. A complete understanding of the natural history of ipf could potentially help to identify different mechanisms that are operative at the early. During the followup period, ct was performed 223 times for each patient average, 8 times. Usual interstitial pneumonia uip and idiopathic pulmonary fibrosis ipf symptoms and treatments idiopathic pulmonary fibrosis ipf belongs to a group of diseases whose specific cause is not well known, which keeps clinicians and researchers worried. Pdf clinical management of acute interstitial pneumonia. Predictors of diagnosis and survival in idiopathic. Pathology outlines usual interstitial pneumonia uip. The scarring involves the supporting framework of the lung. The majority of cases have a recent history of viral upper respiratory tract infection symptoms prior to presenting. Groundglass attenuation, if present, is less extensive than reticular abnormality. Ct features of the usual interstitial pneumonia pattern. It may occur when an injury to the lungs triggers an abnormal healing. Usual interstitial pneumonia cryptogenic organizing pneumonia.
Watch the video lecture usual interstitial pneumonia uip. Usual interstitial pneumoniaoverview usual interstitial. Acute interstitial pneumonia pulmonary disorders merck. Rosane duarte achcar md, in idiopathic pulmonary fibrosis, 2019. Jan 03, 2016 usual interstitial pneumonia is a form of lung disease characterized by progressive scarring of both lungs. Acute interstitial pneumonia shows diffuse alveolar damage, which is almost completely identical to acute respiratory distress syndrome diffuse alveolar damage morphologically eur respir j 2000. Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein c in familial pulmonary fibrosis. Usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. Comparison of usual interstitial pneumonia and nonspecific. 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. Although a defining pathologic finding in idiopathic pulmonary fibrosis ipf, it has been found in other chronic fibrotic lung disease such as the connective tissuedisease associated. 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.
It is particularly unclear how nsip and usual interstitial pneumonia uip are related. Jan 08, 2019 usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and. Idiopathic interstitial lung injury demonstrating temporal and geographic heterogeneity. The most common symptoms are a dry cough and shortness of breath. Usual interstitial pneumonia radiology reference article. However, within 2 years of the publication of the first bts guidelines, a new consensus classification had been proposed by a joint american thoracic society ats and european respiratory society ers committee. Usual interstitial pneumonia is a form of lung disease characterized by progressive scarring of both lungs. Architectural distortion suggestive of lung fibrosis is also frequently recognized. Predictors of diagnosis and survival in idiopathic pulmonary.
Pathogenesis and natural history of usual interstitial. Differential diagnosis usual interstitial pneumonia. Idiopathic pulmonary fibrosis ipf usual interstitial pneumonia uip is not wellunderstood. Diagnosis of idiopathic pulmonary fibrosis american thoracic. A usual interstitial pneumonia uip pattern on chest ct scans is highly suggestive of uip pathologic findings. Previously defined patterns of usual interstitial pneumonia uip. Pdf desquamative interstitial pneumonia dip is characterised by the accumulation of. 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 idiopathic interstitial pneumonias radiologic clinics. Doctors may try to treat usual interstitial pneumonia with.
It can occur in children, usually those infected with the human immunodeficiency virus hiv. Elliot tl, lynch da, newell jd jr, cool c, tuder r, markopoulou k, veve r, brown kk. Usual interstitial pneumonia surgical pathology criteria. A thoracic radiologist with 10 years of clinical experience evaluated a total of 600 typical regions of interest rois circular rois of 32pixel diameter by. The aim of this study was to determine whether quantitative immunohistochemical evaluation of the expression levels of hsp47, type i procollagen and. On hrct images, usual interstitial pneumonia uip is characterized by the presence of reticular opacities, often associated with traction bronchiectasis. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues.
Lip lymphoid interstitial pneumonia, nsip nonspeci. What every radiologist should know about idiopathic. Usual interstitial pneumonia uip is characterized by temporally heterogenous parenchymal fibrosis with architectural distortion, interstitial thickening, fibroblast foci, and honeycombing. Pathogenesis and natural history of usual interstitial pneumonia the whole story or the last chapter of a long novel robert m. 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. Interstitial pneumonitis interstitial lung disease center. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy.
For a long time, there has been no effective therapy but recent studies show promising response to pirfenidone and nintedanib. Acute exacerbation of interstitial lung disease, especially idiopathic pulmonary fibrosis ipf. Acute interstitial pneumonia usually presents acutely with dyspnea and cough followed by rapid development of respiratory failure with most patients requiring mechanical ventilation. Overview of idiopathic interstitial pneumonias pulmonary. We reasoned that if such a signal were present, this approach might be e. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Strieter, md, fccp idiopathic pulmonary fibrosis ipf usual interstitial pneumonia uip is not wellunderstood. Differentiating connective tissue diseaseassociated interstitial lung disease from idiopathic pulmonary fibrosis jonathan h. Imaging findings is compatible with usual interstitial pneumonia uip pattern fibrosis. In patients with atypical radiologic and clinical features, what specific. Distinguishing the usual interstitial pneumonia pattern from the other iip patterns is the most critical.
The term acute interstitial pneumonia aip describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory. Uip is thus classified as a form of interstitial lung disease. Fibroblastic foci adjacent to mature collagen, covered by epithelium. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Jun 14, 2005 heat shock protein hsp 47, a collagenspecific molecular chaperone, is involved in the processing andor secretion of procollagens, and its expression is increased in various fibrotic diseases. 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.
Usual interstitial pneumonia uip is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis ipf. Iip idiopathic interstitial pneumonia, ipf idiopathic pulmonary fibrosis, nsip nonspe cific interstitial pneumonia, rbild respiratory bronchiolitisassociated interstitial lung disease, uip usual interstitial pneumonia radiographics 2015. Idiopathic interstitial pneumonias usual interstitial. Nonspecific interstitial pneumonia and usual interstitial. Granulation tissue foci without significant collagen. On imaging, uip usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing.
Lymphoid interstitial pneumonia is a form of idiopathic interstitial pneumonia. Although investigators of previous gene expression. Pneumonia, pleural effusion, pneumothorax, interstitial pulmonary fibrosis pneumoconiosis, granulomatous and collagenic diseases, druginduced, idiopathic dr. Acute interstitial pneumonia, also commonly referred to as hammanrich syndrome, is a fulminant lung disease of unknown etiology occurring in previously healthy patients. May 05, 2020 usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. Usual interstitial pneumonia an overview sciencedirect.
Usual interstitial pneumonia idiopathic pulmonary fibrosis nonspecific interstitial pneumonia nonspecific interstitial pneumonia respiratory bronchiolitis rb rb associated ild rbild desquamative interstitial pneumonia desquamative interstitial pneumonia diffuse alveolar damage acute interstitial pneumonia. 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. The brazilian thoracic association guidelines for interstitial lung diseases have recently been published. Patients with acute exacerbation may show a combination of features of diffuse alveolar damage or cryptogenic organizing pneumonia and uip defining feature of. The scarring fibrosis involves the supporting framework interstitium of the lung. Clinical findings on 22 patients with lung cancer associated with chronic interstitial pneumonia characteristic. Usual interstitial pneumonia uip is a form of lung disease characterized by progressive scarring of both lungs. Classified into 8 histologic subtypes, all are characterized by varying degrees of inflammation and fibrosis and all cause dyspnea. Heat shock protein hsp 47, a collagenspecific molecular chaperone, is involved in the processing andor secretion of procollagens, and its expression is increased in various fibrotic diseases. Expression of hsp47 in usual interstitial pneumonia and. Interstitial pneumonia with autoimmune features archives of.
Highresolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. Idiopathic pulmonary fibrosis ipf is a chronic fibrosing interstitial lung disease that is usually progressive. 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. 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. Chronic and progressive respiratory failure due to fibrosis in the lung. The term usual refers to the fact that uip is the most common form of.
Read this summary and perspective regarding the clinicopathologic classification of interstitial pneumonias from the pulmonary pathology society companion meeting at. Interobserver agreement of usual interstitial pneumonia diagnosis. Doctors may try to treat usual interstitial pneumonia with corticosteroids to ease inflammation, though the. Specifically, the disease involves damage to the interstitium, which renders protection to the air sacs. Idiopathic interstitial pneumonias comprise usual interstitial pneumonia uip, nonspeci. Radiologic followup of chronic interstitial pneumonia lasted 17. Chibbar r, shih f, baga m, torlakovic e, ramlall k, skomro r, cockcroft dw, lemire eg. 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. 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. Treatment and prognosis depends upon the type of lung disease.
Proliferative organizing subacute phase of diffuse alveolar damage is most common in acute interstitial pneumonia but also exudative acute phase and fibrotic chronic phase can be seen. Although usual interstitial pneumonia uip appears to portend better survival when associated with connective tissue disease ctduip, little is known about the presenting clinical, radiologic, and pathologic features that differentiate pathologically confirmed uip with ctd from idiopathic pulmonary fibrosis ipf. 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. Recently defined diagnostic criteria include exclusion of other known causes of interstitial lung disease, the presence of a usual interstitial pneumonia uip pattern on hrct, and specific combinations of hrct and surgical lung biopsy patterns. Acute interstitial pneumonia merck manuals professional edition. Idiopathic pulmonary fibrosis ipfusual interstitial pneumonia uip is not wellunderstood. 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.