Idiopathic Neutrophilic Jejunitis Responsive to Steroids

Abstract

A 22-year-old male with no medical history presented to an outside hospital with sudden onset epigastric and left upper quadrant abdominal pain. The pain was sharp, constant, non-radiating, associated with anorexia and 25lb weight loss. He had no nausea, vomiting, diarrhea, or fevers. He denied recent travel and IV drug use. A CT of the abdomen and pelvis showed a short segment of jejunitis without wall thickening. A push enteroscopy showed mucosal friability in the jejunum. The biopsy revealed chronic gastritis and acute ulcerating jejunitis. He was started on levofloxacin and metronidazole without improvement. He was seen by rheumatology given the concern for vasculitis, but workup was negative, and CT angiogram showed no vascular defect. He was then transferred to our hospital for further management. On transfer patient's vitals were stable, exam was notable for epigastric tenderness and a diffuse papular rash of his bilateral upper extremities. He had a WBC count of 14,000/mm3 despite a negative infectious workup. A repeat CT of the abdomen showed a mildly thickened loop of proximal jejunum within the LUQ and regional lymphadenopathy. A repeat push enteroscopy showed many superficial non-bleeding intermittent jejunal ulcers with friable, sloughing and erythematous mucosa spanning a 15cm stretch of jejunum. The biopsy results showed acute neutrophillic infiltration limited to the area of jejunal involvement. An MRA of the abdomen showed inflammation of the jejunum without involvement of the ileum. He was diagnosed with localized jejunitis and his symptoms improved after starting prednisone. His abdominal pain resolved by time of discharge. Jejunitis can be caused by a variety of etiologies including Henoch-Schönlein purpura (HSP) and other vasculitides, celiac disease, Crohn's disease, bacterial infections, lymphoma, eosinophilic jejunitis, medications, and recreational drugs. The etiology of the neutrophilic infiltration was unclear. The pathologist suggested a histologic appearance similar to Sweet's syndrome, but the patient lacked fevers and a characteristic rash. Nonetheless, in approaching such a case, an obtaining an expert pathologist review to distinguish between an infectious or inflammatory cause is important to determine whether antibiotic or steroid therapy would be most appropriate. Thus, it is important to consider idiopathic neutrophilic jejunitis in cases of unexplained jejunitis, as it is responsive to steroids.Figure 1