Cytomegalovirus Infection-Associated Hemophagocytic Lymphohistiocytosis and Histiocytic Necrotising Lymphadenitis Progressing to Systemic Lupus Erythematosus: A Case Report

TL;DR

A young woman with a CMV infection developed HLH and HNL, later progressing to SLE, highlighting a rare autoimmune disease progression.

Contribution

This case report documents a rare progression from CMV-associated HLH and HNL to SLE in an immunocompetent individual.

Findings

CMV infection led to HLH and HNL in an immunocompetent patient.

The patient later developed SLE following initial recovery from HLH/HNL.

Treatment with intravenous methylprednisolone was effective for HLH/HNL.

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune multisystem disorder predominantly seen in young females. Its clinical presentation is enormously heterogeneous. Genetic, epigenetic and environmental factors cause SLE. Secondary hemophagocytic lymphohistiocytosis (HLH) associated with cytomegalovirus (CMV) infection is observed in immunocompromised patients and is rare in immunocompetent patients. Histiocytic necrotising lymphadenitis (HNL) is a self-limiting hyperimmune reaction rarely associated with HLH. CMV infection can be associated with HLH, HNL, and SLE. In this case, we discuss an immunocompetent young female patient who presented with an upper respiratory tract infection, which led to HLH and HNL associated with CMV infection. She recovered with initial intravenous methylprednisolone pulse immunosuppressive treatment. However, the patient later evolved into SLE.

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