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Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). In a heterogeneous group of subjects with CTD, no significant difference in survival was noted between patients with CTD-related NSIP and UIP . Take a quiz on differentiating between chronic hypersensitivity pneumonitis (HP), nonspecific interstitial pneumonia (NSIP), and usual interstitial pneumonia (UIP). 40 (5): 640-6. There is a broad overlap in ages for the two diseases. Involvement tends to be subpleural and generally symmetrical with an apicobasilar gradient. NSIP may be idiopathic or associated with collagen vascular diseases or exposure to drugs or chemicals. Clinical, Epidemiological, and Histopathological Features of Respiratory Involvement in Rheumatoid Arthritis In contrast, UIP is associated with extensive fibrosis which is temporally inhomogeneous (i.e. Cox regression analysis examined the relationships between histopathological and radiological diagnoses and mortality, controlling for Immediate subpleural sparing, when present is considered very specific for NSIP. Correct and early diagnosis has a significant impact on patients' outcome because NSIP usually responds well to the corticosteroid therapy or cessation of inciting causes like drugs or organic allergens 12. In the past, the term usual interstitial pneumonia was used synonymously with IPF. NSIP is temporally and spatially homogeneous, while UIP is typically heterogeneous, patchy, and irregular in size.36 The extent of honeycombing and traction bronchiectasis is greater in UIP than the extent of ground glass opacity or micronodules, which are more commonly associated with an NSIP pattern.536, Copyright © 2019, Boehringer Ingelheim Pharmaceuticals, Inc. All rights reserved.Use of this site is subject to the Internet Site Legal Notices and Disclaimers and Privacy Policy. Chest. The symptoms of non-specific interstitial pneumonia are - by definition - non-specific and include insidious onset of dyspnea and dry cough with a restrictive pattern of decreased lung function and reduced gas exchange capacity. Semin Respir Crit Care Med. Katzenstein first described NSIP in 1994. 14. NSIP; Diagnostic Criteria. 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) 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 In general, both affect patients over 50 years of age. Symptoms are not able to distinguish IPF from NSIP. Radiological versus histological diagnosis in UIP and NSIP: survival implications. It may be common in Caucasian-European populations 9. Linking to any other page or site is at your own risk. 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). Smoking is not associated, and it is not a protective factor either. Bronchoalveolar lavage (BAL) has been crucial in elucidating the immune effector cells involved in the inflammatory processes in idiopathic interstitial pneumonias 1. Radiology. 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. 2005;29 (3): 339-45. Primarily idiopathic but the morphological pattern can be seen in association with a number of conditions: If there is no underlying cause, it is termed idiopathic NSIP; which is now considered a distinct entity. The presence of macroscopic honeycombing is almost diagnostic for UIP. Thorax … Several studies have evaluated the ability to differentiate between UIP and NSIP by HRCT. 6. Kim TS, Lee KS, Chung MP et-al. Silva CI, Müller NL, Hansell DM et-al. 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 … various lesions are of different ages). Nonspecific interstitial pneumonitis in patients with AIDS: radiologic features. You are now leaving a Boehringer Ingelheim Pharmaceuticals, Inc. site. Hartman TE, Swensen SJ, Hansell DM et-al. In a study by McDonald and coworkers of 21 patients with NSIP and 32 patients with UIP, the sensitivity, specificity, and accuracy of a CT diagnosis of NSIP were 70, 63, and 66%, respectively . Radiology. noted little difference in the survival of patients with systemic sclerosis–related ILD with NSIP, compared with those with UIP (91% vs 82%, respectively). 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. 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