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Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis

BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of autoimmune diseases with high mortality. We report the effectiveness of baricitinib as an option for the maintenance therapy in MAS secondary to nodular panniculitis. CASE SUMMARY: A 24-year-old female came to our hospital...

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Autores principales: Yi, Guanqun, Huang, Zhengping, Huang, Zhixiang, Wang, Yunqing, Deng, Weiming, Zheng, Shaoling, Li, Tianwang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9298961/
https://www.ncbi.nlm.nih.gov/pubmed/35874753
http://dx.doi.org/10.3389/fimmu.2022.914265
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author Yi, Guanqun
Huang, Zhengping
Huang, Zhixiang
Wang, Yunqing
Deng, Weiming
Zheng, Shaoling
Li, Tianwang
author_facet Yi, Guanqun
Huang, Zhengping
Huang, Zhixiang
Wang, Yunqing
Deng, Weiming
Zheng, Shaoling
Li, Tianwang
author_sort Yi, Guanqun
collection PubMed
description BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of autoimmune diseases with high mortality. We report the effectiveness of baricitinib as an option for the maintenance therapy in MAS secondary to nodular panniculitis. CASE SUMMARY: A 24-year-old female came to our hospital with repeated fever and a skin nodule on right tibial tuberosity. Results were notable for raised serum ferritin (SF), triglycerides (TG), elevated liver function enzymes, interleukin-6 (IL-6), interferon-γ (IFN-γ), soluble interleukin-2 receptor (sIL-2R) and decreased activity of NK cells. The pathological biopsy of the subcutaneous nodules indicated nodular panniculitis. Hemophagocytic cells were found in bone marrow aspiration. She was diagnosed as MAS secondary to nodular panniculitis. With the treatment of methylprednisolone (MP) and immunoglobulin, her symptoms and laboratory data gradually improved. Nevertheless, her disease relapsed when the MP dose was tapered. Regarding the usage of JAK inhibitors in MAS, we used baricitinib (JAK1/2 inhibitor) to treat MAS and her symptom and abnormal laboratory findings returned to normal. During follow-up, though the MP dose was tapered, she was stable without a MAS recurrence. CONCLUSION: The case report suggested baricitinib is an option for MAS in the maintenance therapy phase and is potentially beneficial to prevent recurrence.
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spelling pubmed-92989612022-07-21 Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis Yi, Guanqun Huang, Zhengping Huang, Zhixiang Wang, Yunqing Deng, Weiming Zheng, Shaoling Li, Tianwang Front Immunol Immunology BACKGROUND: Macrophage activation syndrome (MAS) is a severe complication of autoimmune diseases with high mortality. We report the effectiveness of baricitinib as an option for the maintenance therapy in MAS secondary to nodular panniculitis. CASE SUMMARY: A 24-year-old female came to our hospital with repeated fever and a skin nodule on right tibial tuberosity. Results were notable for raised serum ferritin (SF), triglycerides (TG), elevated liver function enzymes, interleukin-6 (IL-6), interferon-γ (IFN-γ), soluble interleukin-2 receptor (sIL-2R) and decreased activity of NK cells. The pathological biopsy of the subcutaneous nodules indicated nodular panniculitis. Hemophagocytic cells were found in bone marrow aspiration. She was diagnosed as MAS secondary to nodular panniculitis. With the treatment of methylprednisolone (MP) and immunoglobulin, her symptoms and laboratory data gradually improved. Nevertheless, her disease relapsed when the MP dose was tapered. Regarding the usage of JAK inhibitors in MAS, we used baricitinib (JAK1/2 inhibitor) to treat MAS and her symptom and abnormal laboratory findings returned to normal. During follow-up, though the MP dose was tapered, she was stable without a MAS recurrence. CONCLUSION: The case report suggested baricitinib is an option for MAS in the maintenance therapy phase and is potentially beneficial to prevent recurrence. Frontiers Media S.A. 2022-07-06 /pmc/articles/PMC9298961/ /pubmed/35874753 http://dx.doi.org/10.3389/fimmu.2022.914265 Text en Copyright © 2022 Yi, Huang, Huang, Wang, Deng, Zheng and Li https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Yi, Guanqun
Huang, Zhengping
Huang, Zhixiang
Wang, Yunqing
Deng, Weiming
Zheng, Shaoling
Li, Tianwang
Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title_full Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title_fullStr Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title_full_unstemmed Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title_short Case Report: Baricitinib as an Alternative in the Maintenance Therapy for Macrophage Activation Syndrome Secondary to Nodular Panniculitis
title_sort case report: baricitinib as an alternative in the maintenance therapy for macrophage activation syndrome secondary to nodular panniculitis
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9298961/
https://www.ncbi.nlm.nih.gov/pubmed/35874753
http://dx.doi.org/10.3389/fimmu.2022.914265
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