Celiac Disease Presenting as Fever of Unknown Origin

Celiac disease (CD) is a common autoimmune enteropathy that occurs, in affected individuals, with exposure to gluten in the diet and improves with removal of dietary gluten. Although CD is readily considered in patients with classical presentations of the disease, atypical manifestations may be the only presenting symptoms. We present a case of CD in a 16-year-old female presenting as fever of unknown origin, which has not been reported previously. The postulated mechanism for fever in CD and the importance of clinicians having a low threshold for considering CD in the differential diagnosis of fever of unknown origin and other enigmatic clinical presentations is discussed. 1. Introduction Celiac disease (CD), or gluten sensitive enteropathy, is a chronic inflammatory autoimmune enteropathy [1]. Classically it presents between the ages of 6 and 24 months, after gluten is introduced into the diet, with signs and symptoms of malabsorption including diarrhea, failure to thrive, abdominal pain, and signs of nutrient and vitamin deficiency [1]. Although only decades ago CD was thought to be rare, it is now accepted that the worldwide prevalence is 0.5 to 1 percent [1]. Furthermore, epidemiological screening studies suggest that the prevalence of CD is 1？:？133 in patients without risk factors or symptoms [2]. Given that atypical manifestations may be the only presenting symptoms of this common disease it is imperative that clinicians consider CD when investigating patients with enigmatic clinical presentations [2]. We present a case of CD presenting as fever of unknown origin. 2. Case A 16-year-old female presented with one month of intermittent fever, night sweats, rigors, malaise, a 5？kg weight loss, and migratory arthralgias. The fever was noncyclic, and she was well in between episodes. Nausea, emesis, diarrhea, constipation, and abdominal pain were absent. She had no sick contacts and no out of country travel. Her father had arthritis of unknown etiology in his early twenties that subsequently resolved, and there was a paternal cousin with alopecia of unknown etiology. She was on no medications. On physical examination, she appeared well, and her height and weight plotted between the 75–90th and 90–97th percentiles, respectively. There were no signs of anemia, and clubbing was absent. Her liver was 2 centimeters below the costal margin, and her spleen tip was palpable. Inflammatory markers were mildly elevated (erythrocyte sedimentation rate (ESR) = 42？mm/hr, C-reactive protein (CRP) = 17.8？mg/L). Hemoglobin (Hb) was within normal limits (12.2？g/dL), as