Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra

Parenchymal lung involvement (PLI) in adult-onset Still's disease (AOSD) has seldom, if ever, been studied. We examine here retrospective cohort AOSD cases and present a review of the literature (1971–2014) on AOSD-related PLI cases.Patients with PLI were identified in 57 AOSD cases. For inclusion, the patients had to fulfill Yamaguchi or Fautrel classification criteria, show respiratory symptoms, and have imaging evidence of pulmonary involvement, and data allowing exclusion of infectious, cardiogenic, toxic, or iatrogenic cause of PLI should be available. This AOSD + PLI group was compared with a control group (non–PLI-complicated AOSD cases from the same cohort).AOSD + PLI was found in 3 out of the 57 patients with AOSD (5.3%) and the literature mentioned 27 patients. Among these 30 AOSD + PLI cases, 12 presented an acute respiratory distress syndrome (ARDS) and the remaining 18 another PLI. In the latter, a nonspecific interstitial pneumonia computed tomography pattern prevailed in the lower lobes, pulmonary function tests showed a restrictive lung function, the alveolar differential cell count was neutrophilic in half of the cases, and the histological findings were consistent with bronchiolitis and nonspecific interstitial pneumonia. Corticosteroids were fully efficient in all but 3 patients. Ten out of 12 ARDS cases occurred during the first year of the disease course. All ARDS-complicated AOSD cases received corticosteroids with favorable outcomes in 10 (2 deceased). Most PLIs occurred during the systemic onset of AOSD.PLI may occur in 5% of AOSDs, of which ARDS is the most severe. Very often, corticosteroids are efficient in controlling this complication.

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“…Interestingly, recent biological treatments such as anakinra or tocilizumab seemed efficient in controlling this complication. [66,70] …”

Section: Discussionmentioning

confidence: 99%

Parenchymal lung involvement in adult-onset Still disease

et al. 2016

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“…In a case report, the IL-1beta receptor antagonist Anakinra was shown to resolve pulmonary hypertension in a patient with Adult-Onset Still’s Disease [22]. …”

Section: Reviewmentioning

confidence: 99%

Inflammatory cytokines in pulmonary hypertension

et al. 2014

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Pulmonary hypertension is an “umbrella term” used for a spectrum of entities resulting in an elevation of the pulmonary arterial pressure. Clinical symptoms include dyspnea and fatigue which in the absence of adequate therapeutic intervention may lead to progressive right heart failure and death. The pathogenesis of pulmonary hypertension is characterized by three major processes including vasoconstriction, vascular remodeling and microthrombotic events. In addition accumulating evidence point to a cytokine driven inflammatory process as a major contributor to the development of pulmonary hypertension.This review summarizes the latest clinical and experimental developments in inflammation associated with pulmonary hypertension with special focus on Interleukin-6, and its role in vascular remodeling in pulmonary hypertension.

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“…Nonetheless, the association of both pathologies in the same patient must arouse suspicion as too whether it is only an uneventful situation or if there could be a direct causality between the two entities. In recent years, a few papers have made the case for this association [25][26][27][28]. Only five cases have now been described.…”

Section: Discussionmentioning

confidence: 99%

STILL out of Breath?

Robillard

2013

J Med Cases

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Adult onset Still's disease (AOSD) is a rare inflammatory syndrome mostly seen in young adults. Known for its wide range of clinical manifestations, AOSD often presents with non-remitting systemic signs and symptoms. Many rare case associations have been described with AOSD, but only few with pulmonary hypertension (PAH). We are presenting a sixth known case of a young female adult with AOSD and PAH in the literature.

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Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: Rapid Response to Anakinra

Cited by 23 publications

References 14 publications

Parenchymal lung involvement in adult-onset Still disease

Parenchymal lung involvement in adult-onset Still disease

Inflammatory cytokines in pulmonary hypertension

STILL out of Breath?

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