Limited form of wegener’s granulomatosis in a patient with Crohn’s disease. A case report

Abstract

Wegener's granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation. Although, the gastrointestinal tract can be involved in this vasculitis, the association between WG and Crohn's disease (CD) exists and was previously described. We report the case of a 29 year-old Tunisian white patient previously diagnosed with CD, presenting 3 years later with a perforated nasal septum, mucosal ulcers and sinusitis. He had also a skin ulcerative eruption of the trunk. There was no evidence of a flare of his CD. A computed tomography scan of the head showed nasal septal perforation, and maxillary pansinusitis. A biopsy from the nasal septum demonstrated nasal mucosa with extensive necrotizing granulomatous reaction associated with vasculitis. The patient was diagnosed as having limited form of Wegener's granulomatosis. He was treated with prednisone 60 mg/day and methotrexate 15 mg weekly. After Two months of treatment, he had no complaints, skin eruption and orofacial lesions were improved. Although, the gastrointestinal tract can be involved in patients with WG, the association with a CD exists and was previously described. The distinction between the two conditions and their possible coexistence is very important and requires histological examination of involved tissue. Keywords: Crohn's disease, vasculitis; Wegener's granulomatosis, corticoid therapy INTRODUCTION Wegener's granulomatosis (WG) is a necrotizing vasculitis of the small to medium-sized arteries characterized by involvement of the upper and lower respiratory tract and the kidneys (1-3). Although, the gastrointestinal tract can be involved in 10%-24% of patients with WG (4-8), the association with a Crohn's disease (CD) exists and was previously described (9,10). The distinction between the two conditions and their possible coexistence requires histological examination of involved tissues. We describe a patient previously diagnosed with CD, presenting 3 years later with a perforated nasal septum and histological evidence of nasal granulomas and ultimately diagnosed as having WG. CASE PRESENTATIONA 29-year-old year-old Tunisian white man first developed a chronic profuse diarrhea with blood and mucus in 2008. He consulted in gastroenterology. The colonoscopy revealed multiple, deep, irregular ulcers of rectosigmoid mucosa. The rest of the colon was normal to the cecum. The ileal mucosa was ulcerated and very inflammatory. The histological diagnosis was consistent with CD, based on transmural inflammatory process including fissure ulcers and the finding of many lymphoid follicles in the subserosa with several non-necrotizing granuloma. He was subsequently treated for one year with mesalazine (2 g per day per os) with rapid resolution of the diarrhea. Intermittent bloody diarrhea had exacerbated Case Report Turk J Gastroenterol 2014; 25 (Suppl.-1): 191-5 Thereafter, intermittent bloody diarrhea was improved and mesalazine alone was used to maintain surgically induced remiss...

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