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Case report
Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus with sensorineural hearing loss in a young male patient
  1. Chou Luan Tan1,
  2. Muhammad Hafizuddin Yahaya2,
  3. Noor Shahrazat Ahmad2 and
  4. Chong Hong Lim1
  1. 1Rheumatology Unit, Department of Internal Medicine, Hospital Sultanah Bahiyah, Alor Setar, Malaysia
  2. 2Department of Internal Medicine, Hospital Sultanah Bahiyah, Alor Setar, Malaysia
  1. Correspondence to Dr Chong Hong Lim; chong_hong{at}


An 18-year-old male adolescent presented with prolonged high spiking temperature, photosensitive rash, oral ulcers and reduced hearing bilaterally of recent onset. Examination revealed malar rash, vasculitis rash over bilateral palms and soles, oral and buccal ulcers, palpable posterior auricular and inguinal lymph nodes, and reduced hearing bilaterally. His further investigations noted pancytopaenia, elevated transaminases, hyperferritinaemia, low C3 and C4 levels, positive antinuclear antibody, double-stranded DNA and direct Coombs test, while bone marrow aspiration revealed active phagocytic activity suggestive of hemophagocytic syndrome. We made a diagnosis of systemic lupus erythematosus with macrophage activation syndrome. We treated him with pulse intravenous methylprednisolone and his condition improved drastically. Temperature resolved on the next day after intravenous methylprednisolone; bilateral sensorineural hearing loss improved to near-normal hearing after treatment. He remained well during follow-up with a tapering dose of prednisolone.

  • rheumatology
  • connective tissue disease
  • systemic lupus erythematosus
  • haematology (including blood transfusion)
  • pathology

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  • Contributors CLT: conception, design and writing of the manuscript. MHY: drafting and proofreading, NSA: acquisition of data and proofreading. CHL: conception, design, writing of the manuscript and proofreading.

  • 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 for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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