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An adolescent with both Wegener's Granulomatosis and chronic blastomycosis

DOI: 10.1186/1546-0096-6-13

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Abstract:

Wegener's Granulomatosis is a systemic small vessel vasculitis of unknown etiology, associated with the presence of anti-neutrophil cytoplasmic antibodies ANCA usually C-ANCA directed against Proteinase 3 (PR-3). It is postulated that auto-immune and infectious mechanisms play a role in the pathogenesis [1]. Viral, bacterial and fungal infections have been reported in association with ANCA positive vasculitis. The presence of infection suggests that pathogens may act as potential triggers of an inflammatory cascade ultimately resulting in vascular inflammation [2]. We report a case of WG that may have been triggered by blastomycosis. To our knowledge, this is the first reported case of such an association.A 16 year old previously healthy girl presented with a 3 day history of progressive pain and swelling in the right calf with inability to bear weight. On further questioning, she also complained of arthralgias, myalgias for the past month with history of intermittent chest pain in the absence of cough or dyspnea for the previous 3 months. In addition, she had generalized constitutional symptoms of generalized fatigue and malaise for the past 2 weeks. She had no history of fever, night sweats weight loss, rash, ocular, ear, nose, throat, gastrointestinal or genitourinary. These symptoms occurred 3 months following a 2 week travel to Kenora, Ontario. Physical examination was unremarkable, with the exception of a swollen warm and exquisitely tender right calf.A Computerized Tomography (CT) scan of the chest and an ultrasound Doppler of right leg were performed because of concern of deep vein thrombosis (DVT) and pulmonary embolism. Chest CT showed a 3 cm cavitary lesion in the left upper lobe with surrounding consolidation (figure 1). Ultrasound Doppler showed no evidence of DVT. Acute phase reactants were elevated: ESR 56 mm/hr and C reactive protein 60 mg/l. Urinalysis showed trace protein and 9 RBC/HPF with no urinary sedimentation or casts. Other initial investiga

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