Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Genotyping for APOL1 risk alleles revealed the presence of double (G1/G2) risk alleles. Our patient achieved a complete renal recovery and resolution of HLH within 1 month of treatment with steroids and mycophenolate mofetil, highlighting the importance of prompt, aggressive therapy.
- renal system
- acute renal failure
- systemic lupus erythematosus
- haematology (INCL blood transfusion)
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Contributors BC: helped to report the findings and acquired relevant literature for writing and wrote the manuscript. VD’A: helped to interpret the pathology findings and significantly improved the discussion and writing of the manuscript. LB: helped to acquire literature for writing and contributed to the writing of the paper. BM: helped to report the clinical findings, reviewed and approved the final version of the paper. BJ: helped to report the clinical findings, reviewed and approved the final version of the paper, orchestrated the reporting, writing and review of the literature for publication. All the authors have agreed to be accountable for the article and accuracy and integrity of the article.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.