Overview of Interstitial Lung Disease (ILD) Interstitial lung disease is a broad term for a number of diseases that lead to inflammation or scarring of the lungs, leading to fibrosis. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Radiology. 9. Cellular NSIP shows an even better response to corticosteroids and carries a substantially better prognosis than the fibrotic type. The presence of the following features, although they can be seen in NSIP, should make us think about other differentials: In general, non-specific interstitial pneumonia (NSIP) carries a much more favorable prognosis than a UIP-type pattern with 90% 5-year survival rate for cellular and ~60% (range 45-90%) 5-year survival in the fibrotic subtype. 27 28 These diseases may be acute or chronic and have a variety of underlying causes, including infection, exposure to dust or other particles, or an … Radiographics. Nishino M, Itoh H, Hatabu H. A practical approach to high-resolution CT of diffuse lung disease. Overall prevalence is higher in women due to high association with collagen vascular diseases, but the prevalence of idiopathic NSIP is similar in both genders. ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Symptoms of NSIP vs. IPF. Idiopathic interstitial pneumonias comprise usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis–associated interstitial lung disease (RB-ILD), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), and lymphoid … Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. Cellular NSIP shows an even better response to corticosteroids and carries a substantially better prognosis than the fibrotic type. It can be normal in early stage of disease and in plain radiographs. UIP may be diagnosed by a radiologist using computed tomography (CT) scan of the chest, or by a pathologist using tissue obtained by a lung biopsy. (2014) European journal of radiology. IDIOPATHIC NSIP VERSUS BOOP AND IPF 1011 In this report, the authors' experience with a series of 31 cases of idiopathic NSIP patients is reviewed and com-pared with cases of IPF (UIP) and idiopathic BOOP, to id- Smoking is not associated, and it is not a protective factor either. 15. 14. Solely or predominantly upper lobe involvement or purely unilateral disease makes the diagnosis of NSIP less likely. The key differential is a usual interstitial pneumonitis (UIP) pattern, with which there can be some overlap in imaging features 3. Radiology. How-ever, the survival rate of idiopathic fibrotic NSIP is far worse than that of cellular NSIP but better than that of UIP: 5-year survival rates … 1987;149 (2): 265-8. Bouros et al. Radiologically, the main feature required for a confident diagnosis of UIP is honeycomb change in the periphery and the lower portions (bases) of the lungs. Current status of idiopathic nonspecific interstitial pneumonia. AJR Am J Roentgenol. You are now leaving a Boehringer Ingelheim Pharmaceuticals, Inc. site. Radiology. A usual interstitial pneumonia (UIP) pattern on chest CT scans is highly suggestive of UIP pathologic findings; the most common cause of UIP is idiopathic pulmonary fibrosis (IPF) [1–5].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 … Check for errors and try again. Radiographics. Flaherty KR, Thwaite EL, Kazerooni EA, et al. Sverzellati N, Wells AU, Tomassetti S et-al. Several studies have evaluated the ability to differentiate between UIP and NSIP by HRCT. The Journal of rheumatology. Nonspecific interstitial pneumonitis in patients with AIDS: radiologic features. Idiopathic interstitial pneumonias with a histological pattern of nonspecific interstitial pneumonia (NSIP) have a better prognosis than UIP, and may present … NSIP may be idiopathic or associated with collagen vascular diseases or exposure to drugs or chemicals. The importance of radiological evaluation in the discrimination between UIP and NSIP Dr. Figen Ba aran Demirkaz k Hacettepe niversity Department of Radiology – A free PowerPoint PPT presentation (displayed as a Flash slide show) on PowerShow.com - id: 577c8a-ZjkyM J Comput Assist Tomogr. various lesions are of different ages). Clinical, radiological, and pathological evaluation of "NSIP with OP overlap" pattern compared with NSIP in patients with idiopathic interstitial pneumonias Respir Med . 25 (4): 447-9. At the other end of the spectrum, idiopathic cellular NSIP and DIP have a survival rate of nearly 100% (14,15). NSIP is a very inhomogeneous group. Imaging features can overlap between cellular and fibrotic types as well as usual interstitial pneumonitis (UIP) in as high as 30% of patients. Case 8: with background systemic sclerosis, immunoglobulin G4 (IgG4)-related sclerosing disease, fibrotic non-specific interstitial pneumonia, cellular non-specific interstitial pneumonia, acute unilateral airspace opacification (differential), acute bilateral airspace opacification (differential), acute airspace opacification with lymphadenopathy (differential), chronic unilateral airspace opacification (differential), chronic bilateral airspace opacification (differential), osteophyte induced adjacent pulmonary atelectasis and fibrosis, pediatric chest x-ray in the exam setting, normal chest x-ray appearance of the diaphragm, posterior tracheal stripe/tracheo-esophageal stripe, obliteration of the retrosternal airspace, leflunomide-induced acute interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, diagnostic HRCT criteria for UIP pattern - ATS/ERS/JRS/ALAT (2011), diagnostic HRCT criteria for UIP pattern - Fleischner society guideline (2018)​, domestically acquired particulate lung disease, lepidic predominant adenocarcinoma (formerly non-mucinous BAC), micropapillary predominant adenocarcinoma, invasive mucinous adenocarcinoma (formerly mucinous BAC), lung cancer associated with cystic airspaces, primary sarcomatoid carcinoma of the lung, large cell neuroendocrine cell carcinoma of the lung, squamous cell carcinoma in situ (CIS) of lung, minimally invasive adenocarcinoma of the lung, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), calcifying fibrous pseudotumor of the lung, IASLC (International Association for the Study of Lung Cancer) 8th edition (current), IASLC (International Association for the Study of Lung Cancer) 7th edition (superseeded), 1996 AJCC-UICC Regional Lymph Node Classification for Lung Cancer Staging, tends to be a dominant feature: can be symmetrically or diffusely distributed in all zones or display a basal predominance, mostly subpleural in distribution (~68%) but can be random (21%), diffuse (8%), and rarely central in distribution (3%), lung volume loss: particularly lower lobes, it is important to carefully scrutinise the images, looking for findings such as joint or bony changes, esophageal dilatation, pleural and pericardial effusion, etc., as it has been mentioned earlier NSIP pattern is also associated with many other conditions. Unable to process the form. 2. {"url":"/signup-modal-props.json?lang=us\u0026email="}, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":11007,"mcqUrl":"https://radiopaedia.org/articles/non-specific-interstitial-pneumonia-1/questions/1647?lang=us"}. Clinical; Insidious onset of dyspnea, cough, fatigue; Mean age 40-50, may be seen in children; Pulmonary function tests: restrictive with decreased diffusing capacity; Generally less severe than UIP; Overall 80=90% 5 year survival Responds to steroids; High resolution computed tomography (HRCT) Biopsy-proved idiopathic pulmonary fibrosis: spectrum of nondiagnostic thin-section CT diagnoses. Katzenstein first described NSIP in 1994. NSIP ranges from type I which is a cellular pattern seen as ground glass opacity on HRCT to type IV with a fibrotic pattern, which may be … 83 (1): 6-19. differential diagnoses of airspace opacification, presence of non-lepidic patterns such as acinar, papillary, solid, or micropapillary, myofibroblastic stroma associated with invasive tumor cells, Non specific interstitial pneumonia (NSIP), Non specific interstitial pneumonitis (NSIP). 17. In contrast, UIP is associated with extensive fibrosis which is temporally inhomogeneous (i.e. While helpful, the differences between UIP and NSIP are often not as obvious as this figure suggests, and the difficulty lies in distinguishing the cases that lie toward the middle. 2001;221 (3): 583-4. Radiographics. However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic … Radiology. Nonspecific Interstitial Pneumonia NSIP is less common than UIP but is still one of the most common histologic findings in patients with IIPs (, 21). Take a quiz on differentiating between chronic hypersensitivity pneumonitis (HP), nonspecific interstitial pneumonia (NSIP), and usual interstitial pneumonia (UIP). This outcome is quite different from that seen in UIP, which has a poor prognosis. There may be ill-defined or ground glass opacities with lower lobe distribution or consolidation in a patchy, reticulonodular or mixed pattern. 13. Mycophenolate mofetil (MMF) has also shown to improve lung function 15. 2008;247 (1): 251-9. Nonspecific interstitial pneumonia with fibrosis: radiographic and CT findings in seven patients. 2006;3 (4): 285-92. 3. Idiopathic pulmonary fibrosis (IPF), which has the histological pattern of usual interstitial pneumonia (UIP), is a progressive interstitial lung disease with a poor prognosis. In this study of patients with histologically well defined UIP and NSIP we found that: (1) patients with an HRCT pattern of UIP are likely to have a histopathological pattern of UIP, but patients with an HRCT pattern other than UIP may have either a histological pattern of UIP or NSIP on the surgical lung biopsy … There is a broad overlap in ages for the two diseases. Bronchoalveolar lavage (BAL) has been crucial in elucidating the immune effector cells involved in the inflammatory processes in idiopathic interstitial pneumonias 1. Arakawa H, Yamada H, Kurihara Y et-al. Classification and natural history of the idiopathic interstitial pneumonias. It may be common in Caucasian-European populations 9. 1995;195 (3): 645-8. 27 (3): 595-615. UIP, indeterminate, probable NSIP, or definite NSIP. NSIP is most often associated with underlying connective tissue disease but can also occur in an idiopathic form . 2012;33 (05): 440-9. Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT. Radiology. LIP lymphoid interstitial pneumonia, NSIP nonspecific interstitial pneumonia, RB-ILD respiratory bronchiolitis–associated interstitial lung disease, UIP usual interstitial pneumonia RadioGraphics 2007; 27:595–615 Published online 10.1148/rg.273065130 Content Code: 1From the Departments of Several key features can help to distinguish NSIP from UIP. While helpful, the differences between UIP and NSIP are often not as obvious as this figure suggests, and the difficulty lies in distinguishing the cases that lie toward the middle. Organizing pneumonia is caused by inflammation 10 and may be idiopathic in nature, termed cryptogenic organizing pneumonia, or may occur secondary to infection, collagen vascular disease, or drug toxicity. NSIP has a relative good prognosis and the majority of patients respond to treatment with corticosteroids. The features which favor the diagnosis of NSIP over UIP are symmetrical bilateral ground-glass opacities with fine reticulations and sparing of the immediate subpleural space. It is thought to have been initially described by Katzenstein and Fiorelli in 1994 14. Rossi SE, Erasmus JJ, Mcadams HP et-al. Historically non-specific interstitial pneumonia was divided into three groups; however, due to similar outcomes, groups II and III (mixed cellular and fibrotic and mostly fibrotic, respectively) are now both classified as fibrotic type: Important negative histological findings are the absence of acute lung injury including hyaline membranes, granulomas, organisms or viral inclusions, dominant airways disease or organizing pneumonia, eosinophils and coarse fibrosis. noted little difference in the survival of patients with systemic sclerosis–related ILD with NSIP, compared with those with UIP (91% vs 82%, respectively). 40 (5): 640-6. In general, both affect patients over 50 years of age. Chest. Thorax … 5. An increase in neutrophils and/or … Clinical, Epidemiological, and Histopathological Features of Respiratory Involvement in Rheumatoid Arthritis (2010) The Korean journal of internal medicine. 8. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Kligerman SJ, Groshong S, Brown KK et-al. In general, non-specific interstitial pneumonia (NSIP) carries a much more favourable prognosis than a UIP-type pattern with 90% 5-year survival rate for cellular and ~60% (range 45-90%) 5-year survival in the fibrotic subtype. Boehringer Ingelheim Pharmaceuticals, Inc. has not reviewed the contents of all pages and sites that may be linked to this site. Non-specific interstitial pneumonia (NSIP) is the second most common morphological and pathological pattern of interstitial lung diseases. Park JS, Lee KS, Kim JS et-al. 2003;123 (4): 1096-103. Differentiating Chronic HP, NSIP, & UIP – Radiology Rounds 2000;217 (3): 701-5. 4. 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