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Invasive Rhinocerebral Mucormycosis Is a Rare Complication in Systemic Juvenile Idiopathic Arthritis Patient

Summary

A 5-year-old girl with a serious joint disease called systemic juvenile idiopathic arthritis developed a dangerous fungal infection in her nasal cavity and brain area. The fungal infection, caused by a mold organism called Lichtheimia, occurred because her immune system was severely weakened by medications used to treat her arthritis. Doctors treated the infection with antifungal drugs and surgery, and the patient eventually recovered by switching to a different arthritis medication. This case highlights how the strong medications needed to control some childhood arthritis can unexpectedly cause serious fungal infections.

Background

Mucormycosis is an opportunistic infection caused by mold fungi of the order Mucorales that typically occurs in immunocompromised patients. Systemic juvenile idiopathic arthritis (sJIA) is a severe form of JIA that requires intensive immunosuppressive treatment, which predisposes patients to serious infections. Invasive mycoses are a frequent cause of death in pediatric rheumatic disease patients.

Objective

To report a rare case of invasive rhinocerebral mucormycosis in a pediatric patient with systemic juvenile idiopathic arthritis and discuss the risk factors for this life-threatening fungal infection in rheumatic disease patients.

Results

The patient presented with nasal discharge, necrotic changes in nasal tissues and fingertips, and orbital inflammation. Lichtheimia spp. was identified as the causative fungal agent. Combined antifungal therapy with posaconazole and liposomal amphotericin B followed by posaconazole monotherapy led to improvement. The patient achieved remission with canakinumab treatment and has remained disease-free for over 2 years without antifungal therapy.

Conclusion

Invasive mucormycosis is a rare but serious complication in pediatric rheumatic disease patients receiving intensive immunosuppression. Risk factors include highly active sJIA, macrophage activation syndrome, prolonged lymphopenia, high-dose corticosteroids, and biologic agents. Clinicians should consider fungal infections as potential complications in rheumatic patients presenting with atypical symptoms.
  • Published in:Turkish Archives of Pediatrics,
  • Study Type:Case Report,
  • Source: PMID: 41037057, DOI: 10.5152/TurkArchPediatr.2025.24282
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