Pneumonia is an inflammatory condition of the lung affecting primarily the small air sacs known as alveoli. 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. The signs and symptoms generally develop and progress rapidly. Pdf comparison of three groups of patients with usual. Acute interstitial pneumonia genetic and rare diseases. 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. A complete understanding of the natural history of ipf could potentially help to identify different mechanisms that are operative at the early. Combined pulmonary fibrosis and emphysema cpfe is an example of coexisting patterns and does not represent a distinctive iip.
Comparison of three groups of patients with usual interstitial pneumonia. The term acute interstitial pneumonia aip describes an idiopathic clinicopathological condition, characterized clinically by an interstitial lung disease causing rapid onset of respiratory. Usual interstitial pneumonia radiology reference article. Printable usual interstitial pneumonia surgical pathology. 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. Classification of usual interstitial pneumonia pattern on computed tomography using spin image texture descriptors stephen humphries ms1, kunihiro yagihashi md2, joyce schroeder md2, rohit sood md, phd3 and david lynch mb2 for the ipfnet investigators 1dept. In patients with atypical radiologic and clinical features, what specific. Other less common patterns include organizing pneumonia and obliterative bronchiolitis 2,6. Imaging findings is compatible with usual interstitial pneumonia uip pattern fibrosis.
Usual interstitial pneumonia cryptogenic organizing pneumonia. High resolution computed tomography scan, idiopathic interstitial fibrosis, nonspecific interstitial pneumonia doi. Radiologic followup of chronic interstitial pneumonia lasted 17. Therefore, awareness of the characteristic ct manifestations of lung cancer associated with chronic interstitial pneumonia in its early stage is important. Usual interstitial pneumonia uip is a form of lung disease characterized by progressive scarring of both lungs. Interstitial lung disease is a condition that results in scarring of.
Conclusions rationale classification of usual interstitial pneumonia pattern on computed tomography using spin image texture descriptors stephen humphries ms1, kunihiro yagihashi md2, joyce schroeder md2, rohit sood md, phd3 and david lynch mb2 for the ipfnet investigators 1dept. Foci of fibroblastic activity and honeycomb change are hallmarks of the uip pattern. Use of a molecular classifier to identify usual interstitial. Predictors of diagnosis and survival in idiopathic pulmonary.
Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy men and women, usually those 40 years. Usual interstitial pneumonia an overview sciencedirect topics. Acute interstitial pneumonia merck manuals professional edition. Highresolution computed tomography features of nonspecific interstitial pneumonia and usual interstitial pneumonia. Usual interstitial pneumonia uip and idiopathic pulmonary fibrosis ipf symptoms and treatments see online here idiopathic pulmonary. This study aimed to describe the distinct clinical features of ppfe with uip pattern. Usual interstitial pneumonia uip is a distinct histological lesion observed in idiopathic pulmonary fibrosis ipf but can be found in other etiologies. 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. Acute interstitial pneumonia aip is a rare and fulminant idiopathic pulmonary disorder that manifests similarly to acute respiratory distress syndrome ards. Usual interstitial pneumonia uip also known as fibrosing alveolitis, is a relatively common form of interstitial lung disease.
Minor histopathological features in usual interstitial pneumonia normal dense scar dense scar micro honeycombing this is uip fibroblast focus. 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. Usual interstitial pneumonia, abbreviated uip, is a relatively common pattern in diffuse lung diseases. There is growing evidence to suggest that distinguishing uip pattern from. Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. 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. What every radiologist should know about idiopathic. Identification of usual interstitial pneumonia pattern. However, uip is not synonymous with ipf as other clinical conditions may be associated with uip, including chronic hypersensitivity pneumonitis, collagen vascular disease.
Acute interstitial pneumonia aip is a rare and serious condition that affects the lungs. Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein c in familial pulmonary fibrosis. A case of lung adenocarcinoma with idiopathic pulmonary fibrosis. Usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. Differential diagnosis usual interstitial pneumonia. Previous studies have suggested an association between usual interstitial pneumonia uip and lung cancer ca. Lip lymphoid interstitial pneumonia, nsip nonspeci. Idiopathic interstitial pneumonias usual interstitial. Architectural distortion suggestive of lung fibrosis is also frequently recognized. We wanted to demonstrate and compare the serial highresolution cts hrct and the pulmonary function test pft findings of the usual interstitial pneumonia uip and the nonspecific interstitial pneumonia nsip. Usual interstitial pneumonia uip is characterized by temporally heterogenous parenchymal fibrosis with architectural distortion, interstitial thickening, fibroblast foci, and honeycombing. It is particularly unclear how nsip and usual interstitial pneumonia uip are related. Aip is classified as an idiopathic interstitial pneumonia iip, and among the iips, it has the most acute onset and rapidly progressive course.
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. Doctors may try to treat usual interstitial pneumonia with. 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. Identification of usual interstitial pneumonia pattern using rnaseq and machine learning. The scarring fibrosis involves the supporting framework interstitium of the lung. Granulation tissue foci without significant collagen. Angiogenesis, apoptosis and reepithelialization at the foci. Transbronchial biopsy in usual interstitial pneumonia ema a. Acute interstitial pneumonia aip, a form of idiopathic interstitial pneumonia, equally affects apparently healthy. 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. Usual interstitial pneumonia is a characteristic pattern of idiopathic pulmonary fibrosis ipf, which is the most frequent of the ilds. Strieter, md, fccp idiopathic pulmonary fibrosis ipf usual interstitial pneumonia uip is not wellunderstood.
These findings are an important first step towards the development of a molecular test that could be applied to bronchoscopy samples, thus avoiding surgery in the diagnosis of idiopathic pulmonary fibrosis. Clinical findings on 22 patients with lung cancer associated with chronic interstitial pneumonia characteristic. Uip pattern can also be caused by variety of other causes including. 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 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. Usual interstitial pneumonia nonspecific interstitial pneumonia organizing pneumonia op diffuse alveolar damage respiratory bronchiolitis desquamative interstitial pneumonia nonspecific interstitial pneumonia nsip a name applied to a form of idiopathic interstitial pneumonia. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and. 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. A wide range of interstitial lung disease diagnoses were made on hrct, including all classifications of usual interstitial pneumonia definite, probable, possible, and diagnosis inconsistent with usual interstitial pneumonia. Radiologic findings are strongly associated with a pathologic diagnosis of usual interstitial pneumonia. Uip is thus classified as a form of interstitial lung disease.
Survival of patients with biopsyproven usual interstitial. Jan 08, 2019 usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Classification of usual interstitial pneumonia pattern on. The most common symptoms are a dry cough and shortness of breath. Differentiating connective tissue diseaseassociated interstitial lung disease from idiopathic pulmonary fibrosis jonathan h. The term usual refers to the fact that uip is the most common form of. Fibroblastic foci adjacent to mature collagen, covered by epithelium. Rosane duarte achcar md, in idiopathic pulmonary fibrosis, 2019.
Pleuroparenchymal fibroelastosis ppfe is a rare form of interstitial pneumonia and sometimes coexists with a histologic usual interstitial pneumonia uip pattern. The outcomes were categorized as resolution, improvement, or dete. Usual interstitial pneumonia nonspecific interstitial pneumonia organizing pneumonia op diffuse alveolar damage respiratory bronchiolitis desquamative interstitial pneumonia nonspecific interstitial pneumonia nsip a name applied to a form of idiopathic interstitial pneumonia typically characterized by ground glass. Several recent studies have demonstrated variable treatment responses and survival based on this classification 28. However more recently the term idiopathic pulmonary fibrosis has been applied solely to the clinical syndrome associated with the morphologic pattern of uip, with the specific exclusion of entities such as nonspecific interstitial pneumonia nsip and desquamative interstitial pneumonia dip 1. Nonspecific interstitial pneumonia and usual interstitial. Aip is similar in presentation to the acute respiratory distress syndrome ards and probably. Pathogenesis and natural history of usual interstitial. Idiopathic pulmonary fibrosis ipf usual interstitial pneumonia uip is not wellunderstood. Differential diagnosis of usual interstitial pneumonia. Predictors of diagnosis and survival in idiopathic. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and ongoing research continues to investigate multiple hypotheses. 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 interstitial.
It may occur when an injury to the lungs triggers an abnormal healing. 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. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Serial thinsection ct findings correlated with pulmonary function objective.
Treatment and prognosis depends upon the type of lung disease. A usual interstitial pneumonia uip pattern on chest ct scans is highly suggestive of uip pathologic findings. In the early stages of the condition, affected people may experience upper respiratory andor virallike symptoms such as cough, shortness of breath, and fever. Usual interstitial pneumonia and nonspecific interstitial. Pathogenesis and natural history of usual interstitial pneumonia. May 05, 2020 usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. Idiopathic pulmonary fibrosisusual interstitial pneumonia. Idiopathic uip later called as uip is a progressive and usually fatal lung disease without an efficient treatment. Groundglass attenuation, if present, is less extensive than reticular abnormality. Chibbar r, shih f, baga m, torlakovic e, ramlall k, skomro r, cockcroft dw, lemire eg.
Transbronchial biopsy in usual interstitial pneumonia. Acute interstitial pneumonia aip is a rare and fulminant form of diffuse lung injury originally described by hamman and rich in 1935. In the past, the term usual interstitial pneumonia was used synonymously with ipf. Usual interstitial pneumonia uip nonspecific interstitial pneumonia nsip organizing pneumonia op diffuse alveolar damage dad desquamative interstitial pneumonia dip respiratory bronchioloitis rb lymphocytic interstitial pneumonia lip usual interstitial pneumonia uip the most common type of interstitial pneumonia. Objective to investigate the relationship between psoriasis and interstitial pneumonia ip. Interstitial pneumonitis interstitial lung disease center. Pathogenesis and natural history of usual interstitial pneumonia the whole story or the last chapter of a long novel robert m.
Pdf a case of lung adenocarcinoma with idiopathic pulmonary. 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. Interstitial lung disease european respiratory society. During the followup period, ct was performed 223 times for each patient average, 8 times. Acute interstitial pneumonia pulmonary disorders merck. The functional disease course for all patients with ild was assessed for clinical manifestations, abnormal pft findings, and hrct abnormalities. Elliot tl, lynch da, newell jd jr, cool c, tuder r, markopoulou k, veve r, brown kk. Rheumatoid arthritisassociated interstitial lung disease. 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. Pneumonia is usually caused by infection with viruses or bacteria and less commonly by other microorganisms, certain medications. Patients and methods we analyzed the clinical data of patients with psoriasis treated with biologic. Symptoms typically include some combination of productive or dry cough, chest pain, fever and difficulty breathing.
Ct, highresolution ct, interstitial lung disease, lung cancer. Our results show that the development of a genomic signature that predicts usual interstitial pneumonia is feasible. Interstitial lung disease is a condition that results in scarring of the lungs, due to damage to the tissue of the lungs rather than the airways bronchial tubes. Usual interstitial pneumonia pattern on highresolution computed tomography ipf is the most common iip subgroup and is thought to be a disease of ageing, as most cases develop in people aged 60 years and older. Classification of usual interstitial pneumonia in patients. Technique of lus lus may be performed with a usual ultrasound machine. Idiopathic pulmonary fibrosis ipf is a chronic fibrosing interstitial lung disease that is usually progressive. Idiopathic pulmonary fibrosis abbreviated ipf redirects here. Usual interstitial pneumonia an overview sciencedirect.
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