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Review
. 2023 Aug 4:14:1223062.
doi: 10.3389/fimmu.2023.1223062. eCollection 2023.

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

Zhipeng Zeng  1 , Wei Tu  1 , Bai Ji  2 , Jie Liu  3 , Kecheng Huang  4 , Daan Nie  5 , Liu Yang  6
Affiliations
Review

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

Zhipeng Zeng et al. Front Immunol. .

Abstract

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.

Keywords: Hemophagocytic lymphohistiocytosis (HLH); Peg-INFα-2b; hepatitis B virus (HBV); interferon-α; systemic lupus erythematosus (SLE).

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Bone marrow biopsy showing hemophagocytosis by histiocyte.
Figure 2
Figure 2
The patient’s clinical course in hospital. DXM, dexamethasone; MP, Methylprednisolone; PSL, prednisolone;VP-16,etoposide;CsA, Cyclosporine A;CTX, cyclophosphamide.
See this image and copyright information in PMC

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This work was supported by the National Natural Science Foundation of China (No. 82000422).