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Idiopathic <jats:styled-content style=\"fixed-case\">NSIP<\/jats:styled-content> is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid\u2010adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High\u2010resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground\u2010glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. <jats:styled-content style=\"fixed-case\">NSIP<\/jats:styled-content> must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary <jats:styled-content style=\"fixed-case\">NSIP<\/jats:styled-content>. A multi\u2010disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic <jats:styled-content style=\"fixed-case\">NSIP<\/jats:styled-content>, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic <jats:styled-content style=\"fixed-case\">NSIP<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/resp.12674","type":"journal-article","created":{"date-parts":[[2015,11,13]],"date-time":"2015-11-13T09:56:54Z","timestamp":1447408614000},"page":"259-268","source":"Crossref","is-referenced-by-count":93,"title":["Idiopathic non\u2010specific interstitial pneumonia"],"prefix":"10.1111","volume":"21","author":[{"given":"Elizabeth A.","family":"Belloli","sequence":"first","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of Michigan Health System  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosemarie","family":"Beckford","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of Michigan Health System  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan","family":"Hadley","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of Michigan Health System  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin R.","family":"Flaherty","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of Michigan Health System  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,11,13]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199402000-00003"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.123.4.1096"},{"key":"e_1_2_12_5_1","first-page":"121","article-title":"The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia","volume":"19","author":"Kim DS","year":"2002","journal-title":"Sarcoidosis Vasc. 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