![]() Over a median follow-up of 17 months, we observed a clear benefit of JAK1/2 inhibition on certain systemic features of AGS, and reproduced results reported using the AGS neurologic severity scale. Here, we report our real-word approach study of JAK1/2 inhibition in 11 patients with AGS, providing extensive assessments of clinical and radiological status interferon signaling, including in cerebrospinal fluid (CSF) and drug concentrations in blood and CSF. Contrastingly, treatment efficacy at the level of the neurological system has been less conclusive. Premised on a chronic enhancement of type I interferon signaling, JAK1/2 inhibition has been trialed in AGS, with clear improvements in cutaneous and systemic disease manifestations. AGS is considered an immune-mediated disease, poorly responsive to conventional immunosuppression. ![]() The paradigm type I interferonopathy Aicardi-Goutières syndrome (AGS) is most typically characterized by severe neurological involvement. ![]() ![]() JAK Inhibition in Aicardi-Goutières Syndrome: a Monocentric Multidisciplinary Real-World Approach Study ![]()
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