Rescued from desperation: adult-onset Still’s disease with life-threatening interstitial lung disease and macrophage activation

Zhang, GuangFeng; Liu, Wenxue; Zhang, Xiao; Liu, Liang

doi:10.1007/s00393-021-01063-7

Cited by 1 publication

(2 citation statements)

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“…As recently discussed (13), it is essential to understand the molecular mechanisms of immunological diseases to make tailored therapies possible. In this sense, AOSD seems to be mainly driven by an IL-1β-, IL-18-, and IL-6-mediated immune response, evidenced by great successes of IL-1-and IL-6blockade (14,15).…”

Section: Pathophysiologymentioning

confidence: 99%

“…While TNFα blockade has turned out not to be very effective but shows effects on articular manifestations (57), IL-1 blockade is successfully used for management of systemic courses (58)(59)(60) and IL-6 blockade shows good efficacy for both joint involvement and systemic courses (61,62). Recently, Zhang et al reported an excellent response of a patient treated with tocilizumab as a rescue approach (14).…”

Section: Treatmentmentioning

confidence: 99%

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Rare, rarer, lung involvement in adult-onset Still's disease: A mini-review

Nies¹,

Schneider

Krusche

2022

Front. Med.

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Adult-onset Still's disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediate a type 1 and type 3 inflammatory signature of the adaptive immune system. This is evidenced by the success of IL-1- and IL-6 inhibition in the management of AOSD. However, anaphylactic reactions to treatment with IL-1- or IL-6-inhibitors is currently being discussed as a potential trigger for lung involvement inf AOSD, while genetic risk factors have also been identified. Clinically, pulmonary involvement in AOSD can manifest in many different forms. Parenchymal inflammation with peripheral consolidations is the most frequent form while PAH is less common, but often very difficult to manage. This mini-review provides an overview of the pathophysiology as well as the clinical presentation and the diagnostic features of pulmonary involvement in AOSD.

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Section: Pathophysiologymentioning

confidence: 99%