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Case Rep Med. 2013;2013:981232. doi: 10.1155/2013/981232. Epub 2013 Jan 17.

Adult Onset Still's Disease: A Case Report with a Rare Clinical Manifestation and Pathophysiological Correlations.

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1
Interstitial Lung Disease Unit, Thoracic Medicine Department, University Hospital of Heraklion, 71110 Heraklion, Crete, Greece.

Abstract

Adult-onset Still's disease is an inflammatory multisystemic disease of unknown etiology. Pleuritis is the most common pulmonary manifestation and pleural effusions are usually exudates with a predominance of neutrophils. We report a case of an eosinophilic pleural effusion as a novel and hitherto unrecognized manifestation of active adult-onset Still's disease. We also observed a marked NLRP3 inflammasome activation with increased production of IL-1β which coincided with the development and resolved upon remission of the pleural effusion suggesting a possible novel pathogenetic pathway for the development of pleural effusions in the context of the auto-inflammatory disorders.

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