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Comment
. 2019 Nov 1;142(11):e59.
doi: 10.1093/brain/awz293.

Treatment of anti-MDA5 autoantibody-positive juvenile dermatomyositis using tofacitinib

Sara Sabbagh  1 , Adriana Almeida de Jesus  2 , SuJin Hwang  3 , Hye Sun Kuehn  3 , Hanna Kim  4 , Lawrence Jung  5 , Ruy Carrasco  6 , Sergio Rosenzweig  3 , Raphaela Goldbach-Mansky  2 , Lisa G Rider  7
Affiliations
Comment

Treatment of anti-MDA5 autoantibody-positive juvenile dermatomyositis using tofacitinib

Sara Sabbagh et al. Brain. .
No abstract available

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Figures

Figure 1
Figure 1
Twenty-eight gene IFN scores in peripheral blood and phosho-STAT signal in PBMCs pre- and post-treatment with tofacitinib. (A and C) Twenty-eight-gene interferon (IFN) scores in Patient 1 (A) and Patient 2 (C) in comparison with healthy and disease controls. (A) Patient 1 at 18 months and 7 months pre-treatment (red dots), and 9 months and 15 months post-treatment (black dots). (C) Patient 2 at 24 months, 18 months and 2 months pre-treatment (red dots), and 5 months and 12 months post-treatment (black dots). Time in months in relation to tofacitinib start date is shown as plus or minus number of months. (B and D) Overlays of flow cytometry histograms of phospho-STATs (pSTAT1) signal in unstimulated (tinted) and indicated cytokines-stimulated (solid) peripheral blood mononuclear cells (PBMCs) from a healthy control and from Patient 1 (B) and Patient 2 (D) pre- and post-treatment samples. Gating was on CD4+ T cells or monocytes by forward scatter (FSC) versus side scatter (SSC).
Figure 2
Figure 2
Lung high-resolution CT in Patient 1 and cutaneous findings in Patient 2, pre- and post-treatment with tofacitinib. (A) Lung view HRCT of Patient 1 showing bilateral lower lobe reticular nodular infiltrates and ground glass opacities 17 months prior to starting tofacitinib therapy. (B) Lung view HRCT of Patient 1 showing near resolution of infiltrates and ground glass opacities 16 months after tofacitinib therapy. (C) Palmar surface of right hand with erythema and ulcerations prior to tofacitinib therapy in Patient 2. (D) Palmar surface of right hand with complete resolution of erythema and ulceration 12 months after tofacitinib therapy in Patient 2.
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References

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