Pulmonary renal syndromes are unusual, but frequently life-threatening manifestations of a distinct group of disorders in the pediatric age group. Although IgA nephropathy is a common cause of hematuria, it is an extremely rare cause of pulmonary renal syndrome, causing high mortality, and has mostly been reported in adult patients. We describe the youngest patient with this presentation reported to date, a 14-year-old male, who presented with end stage renal disease and pulmonary hemorrhage and was found to have IgA nephropathy by renal biopsy and pulmonary capillaritis by open lung biopsy. His lung disease was successfully treated with immunosuppressive medications. Despite this being a rare manifestation of IgA nephropathy, clinicians need to be aware of this presentation as it is potentially fatal, but amenable to aggressive immunosuppression.
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