A Rare Case of Juvenile Systemic Lupus Erythematosus with Disseminated Histoplasmosis

Piyabi Sarkar1, Keya Basu1, Mamata Guha Mallick Sinha1

  • 1Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.

Insights

Progressive disseminated histoplasmosis is a rare, severe fungal infection in children with juvenile systemic lupus erythematosus. This case highlights the critical risk of opportunistic infections in immunocompromised youth, emphasizing the need for vigilance.

Area of Science:

  • Medical Mycology
  • Immunology
  • Pediatric Rheumatology

Background:

  • Histoplasmosis is a systemic fungal infection caused by *Histoplasma capsulatum*.
  • Immunocompromised individuals are at high risk for disseminated disease.
  • Juvenile systemic lupus erythematosus (JSLE) significantly increases susceptibility to opportunistic infections.

Purpose of the Study:

  • To report an extremely rare case of progressive disseminated histoplasmosis in a patient with JSLE.
  • To highlight the severe consequences of opportunistic fungal infections in pediatric autoimmune diseases.
  • To underscore the importance of early diagnosis and aggressive management.

Main Methods:

  • Case report of a 13-year-old female diagnosed with JSLE.
  • Clinical presentation and diagnostic workup for disseminated fungal infection.
  • Treatment course involving antifungal therapy and management of septic shock.

Main Results:

  • The patient developed severe opportunistic histoplasmosis despite aggressive antifungal treatment.
  • The infection rapidly progressed to septic shock, leading to a fatal outcome.
  • This case represents only the second documented instance of this association in medical literature.

Conclusions:

  • Progressive disseminated histoplasmosis is a life-threatening complication in pediatric patients with JSLE.
  • Aggressive antifungal therapy may not always prevent fatal outcomes in severe opportunistic infections.
  • Vigilance for fungal infections is crucial in immunocompromised pediatric patients with autoimmune conditions.

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