An Atypical Presentation of Wegener's Granulomatosis in a Child

Abstract

Wegener`s granulomatosis is an autoimmune small vessel necrotising vasculitis associated with both granulomatosis and polyangiitis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We report a case of a 14 year old girl, admitted with fever, cough, haemoptysis, nose bleeds and following admission developed hoarseness of voice.There was anemia, elevated CRP. Chest X-ray and HRCT chest showed a cavitary consolidation of left upper lobe of the lung. In view of respiratory symptoms, fever, haemoptysis and radiological ndings, the child was started on antitubercular therapy to which she did not respond. Subsequently she developed features of nephtitis and Wegener`s granulomatosis was suspected and conrmed by renal biopsy and positive c-ANCA. She was treated with steroids and cyclophosphamide to which she responded dramatically in our institution. We should suspect Wegener`s granulomatosis in any child presenting with respiratory symptoms, nose bleeds and symptoms of nephritis. Presence of cough, haemoptysis, and fever with obvious consolidation with cavitations may not always be tuberculosis.

Keywords

• Wegener`s
• Necrotising Vasculitis
• Epistaxis