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A5657 - All that Wheezes Is Not Asthma: An Atypical Presentation of Granulomatosis with Polyangiitis in an 8-Year-Old Female
Author Block: M. R. Smiley, K. E. Madison; Naval Medical Center San Diego, San Diego, CA, United States.
INTRODUCTION
Granulomatosis with Polyangiitis (GPA) is a systemic vasculitis of the small and medium sized arteries that typically affects the upper and lower respiratory tract and kidneys. GPA is a rare disease in childhood, with an estimated annual incidence of less than one per one million children. Common clinical presentations of GPA include fatigue, fever, weight loss, arthralgias, rhinosinusitis, cough, and urinary abnormalities. We present the case of GPA masquerading as an asthma exacerbation in a pre-adolescent female.
CASE DESCRIPTION
An 8-year-old Hispanic female previously diagnosed with asthma was admitted to the pediatric intensive care unit after presenting with dyspnea, increased work of breathing, and diffuse wheezing. Pertinent diagnostics on admission included a mild leukocytosis, elevated erythrocyte sedimentation rate of 101, a positive polymerase chain reaction for mycoplasma pneumoniae, and a CXR with concerns for a right lower lobe consolidation. The patient appeared to respond to a combination of bronchodilators, intravenous methylprednisone, and non-invasive positive pressure ventilation; however, attempts to discontinue systemic steroids were met with clinical decompensation. On hospital day 5, the patient had an isolated episode of hemoptysis. Further questioning revealed that the patient had recently been diagnosed with bilateral anterior uveitis by an ophthalmologist and had several months of a migratory polyarthritis which she was waiting to undergo clinical evaluation for. In response to her hemoptysis, a Chest CT and broadened laboratory investigation was performed. The Chest CT demonstrated non-specific ground glass opacities in the right lower lobe in addition to a small nodule; there were cavitary lesions. Additional pertinent lab findings included a positive anti-neutrophil cytoplasmic antibodies (ANCA), anit-proteinase 3 (PR3) antibodies, and a urinalysis showing both proteinuria and hematuria. These results made clinicians suspect a systemic vasculitis and ultimately the patient had a renal biopsy which confirmed the diagnosis of granulomatosis with polyangiitis.
DISCUSSION
This case of an 8-year-old female presenting in acute respiratory failure secondary to GPA mimicking an asthma exacerbation adds yet another clinical presentation to a disease rarely seen in pediatrics. While there are reports of children as young as 7-year-old being diagnosed with GPA, most case series describe subglottic stenosis, glomerulonephritis, and prominent constitutional symptoms as presenting features. Given the rarity of this condition and paucity of literature, this case is significant because it adds yet another clinical presentation of a rare disease. In this case, the rare disease imitated a much more common disease, asthma.