Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis
A 57-year-old Ukranian male with a 45-year history of smoking but no other medical history presented in August 2008 complaining of months of intermittent abdominal pain, diarrhea, and a 40 lb weight loss. His symptoms presented over the course of 1 year. His diarrhea was initially evaluated and attributed to gastroenteritis. Routine studies were sent, including a stool culture. S. arizonae (subtype III), which is associated with osteomyelitis and gastroenteritis in reptile handlers, was isolated and the patient reported contact with a pet iguana. Computed tomography of the abdomen and pelvis was ordered and revealed a 1.5 × 1.7 cm speculated pulmonary nodule in the right upper lobe and thickening of the duodenal, jejunal, and colonic wall with scattered surrounding lymphadenopathy.\nThe patient was placed on antibiotics and continued to suffer from continued diarrhea and weight loss, so inflammatory bowel disease was suspected. In September 2008 he underwent endoscopic studies which revealed a hiatal hernia, old blood in the stomach, mucosal swelling near the gastric antrum, and three areas of circumferential ulceration and inflammation with mucosal pigmentation/erythema (see ). The areas were biopsied, and the biopsy showed reported duodenitis and jejunitis with acute on chronic inflammation. There were no signs of architectural distortion or cryptitis/crypt abscesses, Helicobacter pylori stains were negative, and no bacteria were apparent on biopsy. Given that the biopsies did not show any findings consistent with the suspected diagnosis of inflammatory bowel disease, his symptoms were attributed to S. arizonae gastroenteritis.\nFine needle aspiration of his pulmonary nodule was nondiagnostic, showing only clustered atypical epithelial cells. A positron emission tomography scan was obtained and showed a focus of hypermetabolic activity in the right upper lobe which was read as probable granulomatous disease.\nHe continued to have recurrent episodes of abdominal pain and vomiting in November 2008, and treatment of the presumptive S. arizonae gastritis/enteritis with trimethoprim-sulfamethoxazole and ciprofloxacin failed to improve his symptoms. S. arizonae was not grown from stool samples that were resent.\nOne month later, the patient presented with back pain and decreased urine output. Acute kidney injury was noted, with a serum creatinine of 4.37 mg/dL, while urine studies revealed severe proteinuria and hematuria. Initially the acute kidney injury was thought to be secondary to prerenal azotemia from dehydration or acute interstitial nephritis secondary to treatment with ciprofloxacin and trimethoprim-sulfamethoxazole. A c-ANCA level was drawn for workup of his renal failure and came back at >100 U/L. He then underwent repeat endoscopy which showed normal gastric mucosa, but granular and unusually pigmented duodenal mucosa (). Biopsies of multiple sites were obtained, which later came back positive only in the ascending colon, which showed a tubular adenoma. Slides from a macroscopic jejunal ulcer seen on endoscopy () showed inflammation but no features typical of inflammatory bowel disease. Given the unexplained nature of his systemic symptoms, acute kidney injury, and the positive c-ANCA, a kidney biopsy was performed and showed rapidly progressive glomerulonephritis due to pauci-immune crescentic glomerulonephritis with 25% crescents ().\nThis renal biopsy result was clearly consistent with an ANCA-associated lesion, and the patient received three doses of cyclophosphamide between February and April 2009. Given the finding of a pulmonary nodule, video-assisted thoracoscopic surgery was performed in June 2009. The pathology results later came back positive for adenocarcinoma with clear margins. The lesion was surgically excised and a subsequent positron emission tomography scan was found to be negative for metastasis. He resumed cyclophosphamide therapy in December of 2009 and has had significant improvement of his gastrointestinal symptoms, renal impairment, and systemic symptoms on steroid and immunosuppressive therapy.