Key result
Surgical resection plus immunosuppression stabilizes a rare intracardiac mass in pediatric granulomatosis with polyangiitis.
Why the study?
Cardiac involvement with an intracardiac mass is an exceedingly rare manifestation of granulomatosis with polyangiitis, especially in the pediatric population where data are very limited.
Case Report (n=1)
No
Rare intracardiac mass in pediatric GPA warrants consideration; extends single prior report but leaves optimal management open.
Granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is a systemic necrotizing vasculitis of unknown etiology that commonly involves the upper airways, lungs, and kidneys. Cardiac involvement with an intracardiac mass is an exceedingly rare manifestation of this disease, especially in the pediatric population where, to our knowledge, only one article exists to date that has described such a finding. In this report, we present the case of an adolescent female who initially presented with renal failure and an intracardiac mass. Subsequent work-up led to a diagnosis of granulomatosis with polyangiitis (GPA). Cardiac manifestations in pediatric GPA are not common; however, they may be more prevalent than reported given recent adult literature and concern for clinically silent abnormalities.
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Harris et al. (2012) conducted a case report in Granulomatosis with polyangiitis (GPA) (n=1). Surgical resection and immunosuppressive therapy was evaluated on Clinical outcome and mass pathology. Surgical resection of an extremely rare asymptomatic intracardiac mass and immunosuppressive therapy in a 14-year-old girl with granulomatosis with polyangiitis led to clinical stabilization and discharge.
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