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Rare respiratory diseases in the ICU

when to suspect them and specific approaches

Gibelin, Audea,b,c; Parrot, Antoinea,d; Fartoukh, Muriela,b,c; de Prost, Nicolasc,e

Current Opinion in Critical Care: February 2019 - Volume 25 - Issue 1 - p 29–36
doi: 10.1097/MCC.0000000000000572
RESPIRATORY SYSTEM: Edited by Laurent J. Brochard and Tài Pham
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Purpose of review A prompt identification of the cause of acute respiratory failure (ARF)/acute respiratory distress syndrome (ARDS) is required in order to initiate a targeted treatment. Yet, almost 10% of ARDS patients have no identified ARDS risk factor at ARDS diagnosis. Numerous rare causes of ARF have been reported in this setting. The purpose of this review is to delineate the main rare causes of ARF/ARDS and to provide clinicians with a pragmatic diagnostic work-up.

Recent findings Recent epidemiological data have proposed the identification of a subgroup of ARDS patients lacking exposure to common risk factors. These can be categorized as having immune, drug-induced, malignant and idiopathic diseases. A standardized diagnostic work-up including chest imaging, the objective assessment of left heart filling pressures, bronchoalveolar lavage fluid microbiological investigations and cytological analysis, immunological tests and search for pneumotoxic drugs may allow for identifying the cause of ARF in most cases. Open lung biopsy should be considered in other cases.

Summary A prompt identification of the cause of ARF is required to initiate a targeted treatment. Patients with no identified ARDS risk factor should undergo a comprehensive and hierarchized diagnostic work-up.

aAssistance Publique-Hôpitaux de Paris, Hôpital Tenon, Service de Réanimation médico-chirurgicale, Pôle Thorax Voies aériennes, Groupe hospitalier des Hôpitaux Universitaires de l’Est Parisien

bFaculté de Médecine Sorbonne Université, Paris

cUniversité Paris Est Créteil, Faculté de Médecine de Créteil, Groupe de Recherche Clinique CARMAS, Créteil

dAssistance Publique-Hôpitaux de Paris, Hôpital Tenon, Service de Pneumologie, Pôle Thorax Voies aériennes, Groupe hospitalier des Hôpitaux Universitaires de l’Est Parisien, Paris

eAssistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Henri Mondor, DHU A-TVB, Service de Réanimation Médicale, Créteil, France

Correspondence to Dr Nicolas de Prost, MD, PhD, Service de Réanimation Médicale , Hôpital Henri Mondor 51, Avenue du Maréchal de Lattre de Tassigny, 94010 Créteil Cedex, France. Tel: + 33 1 49 81 23 91; fax: + 33 1 49 81 25 42; e-mail: nicolas.de-prost@aphp.f

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