Nonspecific Interstitial Pneumonia

Flaherty, Kevin R.; Martínez, Fernando J.

doi:10.1007/978-1-4419-9771-5_11

Cited by 5 publications

(5 citation statements)

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“…Given the recent institution of iNSIP as a diagnosis, prevalence and incidence are unknown. Based on retrospective data from mixed cohorts of idiopathic pulmonary fibrosis (IPF) and iNSIP, some have estimated the prevalence to be 1 to 9/100 000 for iNSIP compared with 2 to 20/100 000 for IPF . Determination of true incidence and prevalence was set forth as a future goal of study by the ATS project on iNSIP and was not addressed in the 2008 report…”

Section: Epidemiologymentioning

confidence: 99%

Idiopathic non‐specific interstitial pneumonia

et al. 2015

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Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood 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-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass 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. NSIP 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 NSIP. A multidisciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, 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 NSIP.

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Section: Epidemiologymentioning

confidence: 99%

Idiopathic non‐specific interstitial pneumonia

et al. 2015

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“…For instance, bronchoalveolar lavage fluid analysis has revealed a nonspecific lymphocytosis in related lung disorders. 24,25 Counseling of individual patients should include the recommendation to avoid the use of all tobacco products.…”

Section: Ildmentioning

confidence: 99%

Hermansky-Pudlak Syndrome: Health Care Throughout Life

2013

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Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disease that displays genetic heterogeneity; there are 9 known subtypes. HPS is characterized by oculocutaneous albinism, a platelet storage pool deficiency and resultant bleeding diathesis, and lysosomal accumulation of ceroid lipofuscin. Patients with HPS, specifically those with the genotypes HPS-1, HPS-2, or HPS-4, are predisposed to interstitial lung disease. In addition, some patients with HPS develop granulomatous colitis. Optimal health care requires a thorough knowledge of the unique health risks and functional limitations associated with this syndrome.

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“…The presence of crackles, together with dyspnoea or gas exchange abnormalities, may indicate interstitial lung disease even if the chest radiograph is normal [17,23]. Crackles are also present in the majority of patients with idiopathic nonspecific interstitial pneumonia [24], a condition that affects individuals younger by about 10 yrs than those with IPF [25], and in pulmonary fibrosis associated with connective tissue disease.…”

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confidence: 99%