Wegener's granulomatosis in a pediatric patient.
Wegener's Granulomatosis (WG) is characterized by the clinical triad of upper and lower respiratory disease, and renal insufficiency. Because WG masquerades as a syndrome, its diagnosis is often delayed. Astute clinicians should include WG in their differential diagnosis when a patient presents with these classic findings. The diagnosis is a clinicopathologic one made by biopsy of appropriate tissue showing necrotizing granulomatous vasculitis. Lung tissue obtained by open thoracotomy has the highest diagnostic yield. At one time a universally fatal illness, WG is now treatable with cyclophosphamide and prednisone therapy with remission in most cases. The authors present a case report of a pediatric patient who was eventually diagnosed with WG. The case serves as a reminder to all clinicians to keep this entity as a part of their diagnostic armamentarium.
Kinn
Mary C
MC
Saint Anthony Hospital Family Practice Residency, Oklahoma City, OK, USA.
Dowell
Matthew
M
D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015415:Biomarkers / Q000097:blood; D003520:Cyclophosphamide / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D020551:Pulse Therapy, Drug; D016896:Treatment Outcome
D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D003520:Cyclophosphamide; D008775:Methylprednisolone