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Abstract

Acute respiratory distress syndrome (ARDS) is a well-known description of progressive respiratory decline. ARDS can be suspected in patients that meet the following criteria: (1) progressive dyspnea with onset within 1 week of a suspected clinical insult, (2) reduction in arterial oxygen saturation, and (3) bilateral alveolar infiltrates not due to a primary cardiogenic process.1 The management of ARDS is largely unchanged regardless of the etiology and thus will not be within the scope of discussion for this report. However, the etiology of ARDS is of significance given that the cause of a patient’s respiratory failure has prognostic implications. In fact, trauma related causes of ARDS have a better prognosis compared to non-traumatic triggers.2 It is not incomprehensible that individuals who may have underlying lung pathology portend an increased risk of developing severe ARDS with subsequent increased morbidity and mortality. Any condition that results in decreased pulmonary reserve could result in more severe forms of ARDS, including interstitial lung disease (ILD). Given the heterogeneity of this disease spectrum, prognosis of ILD depends on the subtype, duration/severity of respiratory symptoms, modifiable patient factors, and other elements. Overall, individuals with ILD have reduced quality of life and early mortality.8 Additionally, there are inheritable forms of ILD such as familial pulmonary fibrosis with varying phenotypic expressions and disease severity that may be underrecognized given its varying presentations.4 This is the case of a patient with rapid respiratory decline secondary to ARDS from an unknown etiology in the setting of significant family history of ILD.

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