A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Enfermedad pulmonar intersticial difusa idiopatica clinica las. The basic approach to diagnosing these entities is included in american thoracic societyeuropean respiratory society atsers. In interstitial lung diseases, the concepts of patterns and diseases are constantly changing as the knowledge base increases. Stanford medicine school of medicine departments surgical pathology criteria usual interstitial pneumonia navigation for this section. Patients present with progressively worsening dyspnea and nonproductive cough, 3. Usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred.
Usual interstitial pneumonia cryptogenic organizing pneumonia. Ct features of the usual interstitial pneumonia pattern. Overview of idiopathic interstitial pneumonias pulmonary. Tomografia computadorizada por raios x, doencas pulmonares intersticiais. Pneumonia indicates lung abnormality, which includes fibrosis and inflammation.
Describe the morphologic patterns associated with the iips. Minor histopathological features in usual interstitial pneumonia normal dense scar dense scar micro honeycombing this is uip fibroblast focus. Learn more about the symptoms, causes, diagnosis, treatment, risks, and complications of ild. 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. Usual interstitial pneumonia radiology reference article. The characteristic findings on highresolution computed tomography of usual interstitial pneumonia uip are reticular abnormality and honeycombing with basal predominance. After an incidentfree postoperative period, the patient was referred to the pulmonology unit for. Usual interstitial pneumonia uip is the prototype of pulmonary fibrosis with socalled temporal heterogeneity. It is particularly unclear how nsip and usual interstitial pneumonia uip are related. Mar 26, 2020 usual interstitial pneumonia is a type of lung disease in which patches of inner lung tissue become inflamed and scarred. Idiopathic pulmonary fibrosis ipfusual interstitial pneumonia uip is not wellunderstood. On hrct images, usual interstitial pneumonia uip is characterized by the presence of reticular opacities, often associated with traction bronchiectasis. Usual interstitial pneumonia an overview sciencedirect topics. Interstitial lung disease ild is a group of many lung conditions.
What every radiologist should know about idiopathic interstitial pneumonias1 learning objectives for test 1 after reading this article and taking the test, the reader will be able to. Differential diagnosis of usual interstitial pneumonia. 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. Nonspecific interstitial pneumonia and usual interstitial pneumonia. Many patients also report that the subtle onset of their symptoms months or even years earlier was mistaken for a less serious respiratory disease, which delayed referral to a specialized center, 5. However, uip is not synonymous with ipf as other clinical conditions may be associated with uip, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug.
Neumonia intersticial usual usual interstitial pneumonia, uip. It typically occurs in patients with usual interstitial pneumonia uip. Delayed pulmonary fibrosis usual interstitial pneumonia in. The histopathologic patterns in chronic hp include organizing pneumonia, cellular nonspecific interstitial pneumonia nsip, fibrotic nsip, and usual interstitial pneumonia uip, applying the 2002 american thoracic societyeuropean respiratory society atsers criteria for the classification of idiopathic interstitial pneumonias. To evaluate the differences between surgical biopsies of distinct lung lobes in terms of the histopathological.
The typical patient with ipf is 50 years old or older. Nonspecific interstitial pneumonia nsip is the second most common morphological and pathological pattern of interstitial lung diseases. Pathogenesis and natural history of usual interstitial. Classified into 8 histologic subtypes, all are characterized by varying degrees of inflammation and fibrosis and all cause dyspnea. Pdf idiopathic pulmonary fibrosis is a severe and progressive chronic. Usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Differential diagnosis usual interstitial pneumonia. Usual interstitial pneumonia uip is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis ipf. Architectural distortion suggestive of lung fibrosis is also frequently recognized. Idiopathic interstitial pneumonias comprise a number of relatively uncommon clinicopathologic entities, which can be distinguished from one another and from other forms of diffuse interstitial lung disease by their clinical, radiologic, and histologic features. Granulation tissue foci without significant collagen.
Acute interstitial pneumonia aip is a rare and fulminant form of diffuse lung injury originally described by hamman and rich in 1935. Idiopathic pulmonary fibrosis ipf usual interstitial pneumonia uip is not wellunderstood. A histologic pattern of nonspecific interstitial pneumonia. Ipaf is a clinical entity that encompasses patients with idiopathic interstitial pneumonia iip and features suggesting an underlying autoimmune process but not meeting the established criteria for a connective tissue disease ctd. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. Nonspecific interstitial pneumonia radiology reference. Temporal heterogeneity refers to the variegated appearance of the lung biopsy in uip, where areas of advanced fibrosis are seen adjacent to entirely normal lung, with interspersed areas of active fibroblastic proliferation known as fibroblastic foci. A usual interstitial pneumonia uip pattern on chest ct scans is highly suggestive of uip pathologic findings. In contrast, fibrotic nonspecific interstitial pneumonia has a bilateral lower lobe distribution with architectural derangement on hrct. The scarring involves the supporting framework of the lung.
Delayed pulmonary fibrosis usual interstitial pneumonia. This study aimed to investigate whether there was a difference in outcome related to histologic pattern in cryptogenic fibrosing alveolitis cfa and to see whether there were correlations between clinical and radiologic findings and histology. Ground glass opacity on chest imaging studies and reduced dlco have been reported in h1n1 pneumonia in a study with 3 months followup. Thinking of uip as a disease has a direct impact on current patient care, the use of immunosuppressive therapies requiring more caution and researchers having greater freedom to study the use of antiipf drugs in patients with uip. Patients with acute exacerbation may show a combination of features of diffuse alveolar damage or cryptogenic organizing pneumonia and uip defining feature of. How is nonspecific interstitial pneumonia treated most patients with the cellular type of nsip respond well to treatment with oral corticosteroids, such as prednisone. Acute exacerbation of usual interstitial pneumonia after. A histologic pattern of nonspecific interstitial pneumonia is. Nonspecific interstitial pneumonia cleveland clinic. Pdf supervivencia en pacientes con neumonia intersticial usual. 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. 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.
Dendriform pulmonary ossification in the absence of usual. Doctors may try to treat usual interstitial pneumonia with corticosteroids to ease inflammation, though the. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis zoe d. Usual interstitial pneumonia is a form of lung disease characterized by progressive scarring of both lungs. The term usual refers to the fact that uip is the most common form of interstitial fibrosis. Usual interstitial pneumonia uip is a form of lung disease characterized by progressive scarring of both lungs. 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. At the top, two areas of honeycombing can be observed haematoxylin and eosin stain, 20. It may occur when an injury to the lungs triggers an abnormal healing.
Nonspecific interstitial pneumonia nsip is a rare disorder that affects the tissue that surrounds and separates the tiny air sacs of the lungs. Usual interstitial pneumonia an overview sciencedirect. Aip is similar in presentation to the acute respiratory distress syndrome ards and probably. The scarring fibrosis involves the supporting framework interstitium of the lung. We assessed patients with ct findings of dpo without uip to determine possible causative factors and to assess the clinical and ct course. Dendriform pulmonary ossification dpo is a rare lung disease in which mature bone is present in the peripheral interstitium of the lung. Desquamative interstitial pneumonia, respiratory bronchiolitis and their rela. Current explanations of the natural history and pathogenesis of ipfuip are controversial, and ongoing research continues to investigate multiple hypotheses. However, patients who do not respond to corticosteroid therapy may require additional treatment with immunesuppressing drugs.
Uip is thus classified as a form of interstitial lung disease. Fibroblastic foci adjacent to mature collagen, covered by epithelium. Jan 08, 2019 usual interstitial pneumonia uip is a progressive condition in which there is an increased scarring of the lung tissue. Pathogenesis and natural history of usual interstitial pneumonia. 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 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. 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 surgical pathology criteria. The role of highresolution computed tomography in the diagnosis of interstitial lung disease is increasing as our understanding of its diagnostic accuracy improves. Webmd explains various types of interstitial lung disease. On imaging, uip usually presents with a lung volume loss and a c. Nonspecific interstitial pneumonia and usual interstitial. 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 condition tends to progress rapidly, causing irreparable lung damage in as little as one year after symptoms begin and ultimately leading to respiratory failure.
Survival in patients with usual interstitial pneumonia secondary to. Neumonia intersticial descamativaenfermedad pulmonar intersticial con. A complete understanding of the natural history of ipf could potentially help to identify different mechanisms that are operative at the early. The term usual refers to the fact that uip is the most common form of. 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. 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. Usual interstitial pneumonia pattern in two patients with idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis ipf, the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological andor pathological pattern of usual interstitial pneumonia uip. Stanford medicine school of medicine departments surgical pathology criteria usual interstitial pneumonia surgical pathology.
These air sacs, called the alveoli, are where the exchange of oxygen and carbon dioxide takes place between the lungs and the bloodstream. All interstitial lung diseases affect the interstitium, a part of your lungs. Specifically, the disease involves damage to the interstitium, which renders protection to the air sacs. Idiopathic pulmonary fibrosis ipf is a chronic fibrosing interstitial lung disease that is usually progressive. What every radiologist should know about idiopathic. On imaging, the most common features are relatively symmetric. 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. The scarring involves the supporting framework interstitium of the lung. List the seven entities included in the atsers classi. Aip is classified as an idiopathic interstitial pneumonia iip, and among the iips, it has the most acute onset and rapidly progressive course. Histopathological analysis revealed widened alveolar septa with type ii pneumocyte proliferation and mononuclear inflammatory infiltrate with interstitial fibrosis in patchwork pattern, suggesting usual interstitial pneumonia fig. Unlike other iip, uip is not incorporated in the diagnostic criteria of ipaf. Cuando una enfermedad pulmonar intersticial incluye tejido cicatricial. Groundglass attenuation, if present, is less extensive than reticular abnormality.
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