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

Summary

A 5-year-old girl with systemic juvenile arthritis being treated with immunosuppressive medications developed a rare and serious fungal infection in her nose and sinuses caused by a mold called Lichtheimia. The infection caused tissue death and spread toward her brain, but was successfully treated with combination antifungal drugs and surgery. This case highlights how powerful immunosuppressive treatments for severe arthritis can increase the risk of life-threatening fungal infections.

Background

Mucormycosis is an opportunistic infection caused by mold fungi that typically occurs in immunocompromised patients with high mortality rates. Systemic juvenile idiopathic arthritis (sJIA) is a severe form of juvenile arthritis that can require intense immunosuppression, predisposing patients to infections including invasive mycoses. Mucormycosis in pediatric rheumatic disease patients is rare but can be life-threatening.

Objective

To present a case report of a 5-year-old girl with sJIA who developed invasive rhinocerebral mucormycosis during biologic treatment and immunosuppressive therapy. The report aims to highlight this rare complication and discuss the risk factors associated with mucormycosis development in pediatric rheumatic disease patients.

Results

A 5-year-old girl with sJIA on tocilizumab developed disease relapse requiring re-initiation of therapy plus high-dose corticosteroids and anakinra for macrophage activation syndrome. She subsequently developed invasive rhinocerebral mucormycosis caused by Lichtheimia spp. with necrosis of nasal structures and orbital involvement. Treatment with posaconazole and liposomal amphotericin B led to improvement, followed by successful long-term remission with canakinumab therapy.

Conclusion

Mucormycosis should be considered as a potential serious complication in pediatric rheumatic disease patients undergoing intensive immunosuppression, particularly those with active disease and prolonged lymphopenia. Risk factors in sJIA include highly active disease, macrophage activation syndrome, prolonged lymphopenia, long-term glucocorticoid use, and biologic treatment. Further research is needed to develop prevention and management strategies for mucormycosis in children with rheumatic diseases.
  • Published in:Turkish Archives of Pediatrics,
  • Study Type:Case Report,
  • Source: PMID: 41037057, DOI: 10.5152/TurkArchPediatr.2025.24282
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