Affiliations 

  • 1 Rheumatology Unit, Department of Internal Medicine, Hospital Sultanah Bahiyah, Alor Setar, Malaysia
  • 2 Department of Internal Medicine, Hospital Sultanah Bahiyah, Alor Setar, Malaysia
  • 3 Rheumatology Unit, Department of Internal Medicine, Hospital Sultanah Bahiyah, Alor Setar, Malaysia chong_hong@hotmail.com
BMJ Case Rep, 2020 Mar 12;13(3).
PMID: 32169986 DOI: 10.1136/bcr-2019-233330

Abstract

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.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.