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IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review

Hemophagocytic lymphohistiocytosis (HLH) is a severe and life-threatening hyperinflammatory condition characterized by excessive activation of macrophages and T cells and resulted in multi-organ dysfunction. HLH can be a primary disease or secondary to infections, malignancy, and some autoimmune dis...

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Autores principales: Zeng, Zhipeng, Tu, Wei, Ji, Bai, Liu, Jie, Huang, Kecheng, Nie, Daan, Yang, Liu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10436618/
https://www.ncbi.nlm.nih.gov/pubmed/37600795
http://dx.doi.org/10.3389/fimmu.2023.1223062
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author Zeng, Zhipeng
Tu, Wei
Ji, Bai
Liu, Jie
Huang, Kecheng
Nie, Daan
Yang, Liu
author_facet Zeng, Zhipeng
Tu, Wei
Ji, Bai
Liu, Jie
Huang, Kecheng
Nie, Daan
Yang, Liu
author_sort Zeng, Zhipeng
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) is a severe and life-threatening hyperinflammatory condition characterized by excessive activation of macrophages and T cells and resulted in multi-organ dysfunction. HLH can be a primary disease or secondary to infections, malignancy, and some autoimmune diseases, including adult-onset Still’s disease (AOSD) and systemic lupus erythematosus (SLE). However, it is rare for HLH to occur as a secondary condition to drug-induced lupus erythematosus (DILE). In this report, we present a case of HLH as an unusual complication during SLE treatment in a 31-year-old male patient. The patient initially suffered from active chronic hepatitis B (CHB) and was treated with pegylated INFα-2b (Peg-INFα-2b), tenofovir disoproxil and lamivudine. After 19 months, CHB obtained biochemical and virological response with HBsAg positive to HBsAb. The patient developed fever, headache, and cytopenia after Peg-INFα-2b treatment for 33 months, and laboratory studies revealed that ANA and anti dsDNA were positive. He displayed 5 features meeting the HLH-2004 criteria for diagnosis including fever, pancytopenia, hyperferritinemia, high levels of soluble CD25, and hemophagocytosis on bone marrow biopsy. The patient was initiated with a combination treatment of intravenous methylprednisolone pulse therapy, oral cyclosporine, and etoposide (VP-16), which was followed by a course of oral prednisolone, intravenous cyclophosphamide pulse therapy, and entecavir with complete response. To our knowledge, this is the first report of IFN-α induced SLE complicating with HLH. Physicians should consider the potential autoimmune side effects of IFN-α therapy and be alert to insidious HLH in patients diagnosed with SLE.
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spelling pubmed-104366182023-08-19 IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review Zeng, Zhipeng Tu, Wei Ji, Bai Liu, Jie Huang, Kecheng Nie, Daan Yang, Liu Front Immunol Immunology Hemophagocytic lymphohistiocytosis (HLH) is a severe and life-threatening hyperinflammatory condition characterized by excessive activation of macrophages and T cells and resulted in multi-organ dysfunction. HLH can be a primary disease or secondary to infections, malignancy, and some autoimmune diseases, including adult-onset Still’s disease (AOSD) and systemic lupus erythematosus (SLE). However, it is rare for HLH to occur as a secondary condition to drug-induced lupus erythematosus (DILE). In this report, we present a case of HLH as an unusual complication during SLE treatment in a 31-year-old male patient. The patient initially suffered from active chronic hepatitis B (CHB) and was treated with pegylated INFα-2b (Peg-INFα-2b), tenofovir disoproxil and lamivudine. After 19 months, CHB obtained biochemical and virological response with HBsAg positive to HBsAb. The patient developed fever, headache, and cytopenia after Peg-INFα-2b treatment for 33 months, and laboratory studies revealed that ANA and anti dsDNA were positive. He displayed 5 features meeting the HLH-2004 criteria for diagnosis including fever, pancytopenia, hyperferritinemia, high levels of soluble CD25, and hemophagocytosis on bone marrow biopsy. The patient was initiated with a combination treatment of intravenous methylprednisolone pulse therapy, oral cyclosporine, and etoposide (VP-16), which was followed by a course of oral prednisolone, intravenous cyclophosphamide pulse therapy, and entecavir with complete response. To our knowledge, this is the first report of IFN-α induced SLE complicating with HLH. Physicians should consider the potential autoimmune side effects of IFN-α therapy and be alert to insidious HLH in patients diagnosed with SLE. Frontiers Media S.A. 2023-08-04 /pmc/articles/PMC10436618/ /pubmed/37600795 http://dx.doi.org/10.3389/fimmu.2023.1223062 Text en Copyright © 2023 Zeng, Tu, Ji, Liu, Huang, Nie and Yang 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
Zeng, Zhipeng
Tu, Wei
Ji, Bai
Liu, Jie
Huang, Kecheng
Nie, Daan
Yang, Liu
IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title_full IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title_fullStr IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title_full_unstemmed IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title_short IFN-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
title_sort ifn-α induced systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report and literature review
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10436618/
https://www.ncbi.nlm.nih.gov/pubmed/37600795
http://dx.doi.org/10.3389/fimmu.2023.1223062
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