Granulomatosis with polyangiitis (GPA; previously Wegener granulomatosis) is a rare, multisystem, necrotizing granulomatous vasculitis that classically affects the upper and lower respiratory tracts and kidneys but can have diverse clinical features. The clinical course is often rapidly progressive, and without appropriate treatment is almost universally fatal. Early symptoms are often nonspecific and a high index of suspicion is needed to recognize this serious disease, particularly in the absence of overt pulmonary or renal manifestations. Because initial symptoms can mimic those of infection, patients often present first to the general pediatrician. We present a case of pediatric GPA in a 14-year-old boy who initially presented with constitutional symptoms, sore throat, and hematuria, and then developed grossly necrotic-appearing tonsils before rapid deterioration requiring intensive life-saving measures. We review the common manifestations of GPA, including those that may be unique to the pediatric population, and emphasize the importance of early diagnosis and intervention in preventing devastating outcomes from this disease.
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Case Report|
May 01 2014
A 14-Year-Old Boy With Sore Throat and Tea-Colored Urine
Ashley Cooper, MD;
Departments of aPediatric Rheumatology and
Address correspondence to Ashley Cooper, MD, Pediatric Rheumatology, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: amcooper@cmh.edu
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Dinesh Rakheja, MD;
Dinesh Rakheja, MD
bPathology and Pediatrics, University of Texas Southwestern Medical Center, Dallas, Texas
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Marilynn Punaro, MD
Marilynn Punaro, MD
Departments of aPediatric Rheumatology and
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Address correspondence to Ashley Cooper, MD, Pediatric Rheumatology, Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: amcooper@cmh.edu
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
Pediatrics (2014) 133 (5): e1377–e1380.
Article history
Accepted:
October 21 2013
Citation
Ashley Cooper, Dinesh Rakheja, Marilynn Punaro; A 14-Year-Old Boy With Sore Throat and Tea-Colored Urine. Pediatrics May 2014; 133 (5): e1377–e1380. 10.1542/peds.2013-0466
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