Effectiveness and safety of ruxolitinib for the treatment of refractory systemic idiopathic juvenile arthritis like associated with interstitial lung disease : a case report

Abstract

Figure 1 Radiological response to JAK1/JAK2 blockade with ruxolitininb. (A) Chest CT scan before the initiation of ruxolitinib. (B) Chest CT scan 12 months after the initiation of ruxolitinib. Figure 2 T helper immunophenotyping. (A) Gating strategy in the patient's peripheral blood mononuclear cells (PBMCs) for T helper immunophenotyping. (B) Representative fluorescence activated cell sorting (FACS) plots are presented. The horizontal bars represent the mean+/-SD. Frequencies of T helper subsets within the CD4 + memory compartment in controls and P. Subsets were defined as follows: Th1 (CXCR5 − CXCR3 + CCR4 − CCR6 −), Th2 (CXCR5 − CXCR3 − CCR4 + CCR6 −), Th17 (CXCR5 − CXCR3 − CCR4 + CCR6 +) and Tfh (CXCR5 +).

Response to: ‘Effectiveness and safety of ruxolitinib for the treatment of refractory systemic idiopathic juvenile arthritis like associated with interstitial lung disease: case report’ by Bader-Meunier et al

“…We were very interested to read the correspondence from Dr Bader-Meunier and colleagues, who report a case of systemic juvenile idiopathic arthritis (sJIA) with associated lung disease 1. This case indeed matches the description of and provides further evidence for the striking and unusual clinical characteristics we recently detailed in an international case series of such patients 2.…”

Hypersensitivity reactions to IL-1 and IL-6 inhibitors are linked to common HLA-DRB1*15 alleles

“…Drug-induced interstitial lung disease, including PAP, are reported. 10,31-34 It will be critical to determine if DLD in sJIA improves by withdrawing DHR-associated drugs, if there is a window of opportunity for this intervention, and if drug tolerance develops over time, especially with concurrent immune suppression. In light of our results, it seems unlikely that DLD is HLA-DRB1*15-associated, independent of DHR.…”

Update – Systemische juvenile Arthritis

“…JAKi wurden erfolgreich zur Therapie der systemischen Arthritis eingesetzt [55][56][57] (s. hierzu den Artikel B. Hügle: "JAK-Hemmer bei adulter und juveniler Arthritis" in diesem Heft).…”