TITLE:
Orbital pseudotumor: A single precursor clinical manifestation of Wegener granulomatosis in a ten year old boy
AUTHORS:
Paraskevi Maggina, Varvara Askiti, Mersini Maurikou, Maria Mila, Dionisios Papadatos, Lela Stamogianou, Constantinos J. Stefanidis
KEYWORDS:
Pseudotumor; Hematuria; Wegener Granulomatosis; ANCA; Childhood
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.2 No.1,
March
21,
2012
ABSTRACT: Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms and the close follow up in cases of uncertain initial diagnosis.