PRACTICAL TEACHING CASE

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4 A Disease That Is Often Missed Without 62
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6 Gastrointestinal Symptoms 64
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8 Q3 Amir Rezk, A. Clark Gunnerson, and Michael Komar 66
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10 Q1 Department of Gastroenterology, Geisinger Medical Center, Danville, Pennsylvania 68
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13 Question: A 58- 71
14 year-old woman 72
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16 years of migratory 74
print & web 4C=FPO

17 polyarthralgias in 75
18 her knees, ankles, 76
19 elbows, and wrists. 77
20 She had a large 78
21 effusion in the right 79
22 knee, tenosynovitis 80
23 in the left hand and 81
24 skin hyperpigmen- 82
25 tation. Labs were 83
26 negative for rheu- 84
27 matoid factor, anti-cyclic citrullinated peptide antibody and anti-nuclear antibody. She was diagnosed with seronegative 85
28 rheumatoid arthritis and was initially treated with hydroxychloroquine. Ten months later, prednisone and methotrexate 86
29 were added for refractory symptoms. She started etanercept six months later with little improvement in symptoms. Six 87
30 months later she developed fatigue, depression and mild cognitive dysfunction. One year later, she started having epigastric 88
31 abdominal pain with nausea, diarrhea and weight loss. CT scan of the chest and abdomen demonstrated a left pleural 89
32 effusion with mediastinal and mesenteric lymphadenopathy. Laparoscopic mesenteric lymph node biopsy revealed his- 90
33 tiocytes (Figure A) with periodic acid-Schiff positive globules (Figure B). 91
34 What is the likely cause of her symptoms? 92
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36 A. Inflammatory bowel disease with migratory C. Whipple’s disease 94
37 polyarthropathy 95
D. AIDS
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B. Connective tissue disease with gastrointestinal
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involvement
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Look on page 000 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more
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information on submitting your favorite image(s) to Practical Teaching Cases.
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Acknowledgments
46 The authors thank Dr Kai Zhang, MD for assistance with pathology photos.
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49 Conflicts of interest
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50 Q2 The authors disclose no conflicts. 108
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© 2016 by the AGA Institute
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53 http://dx.doi.org/10.1053/j.gastro.2015.11.054 111
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Gastroenterology 2016;-:1–2
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 PRACTICAL TEACHING CASE
117 Answer (Page 000): Whipple’s disease 175
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119 The correct answer is C. Whipple’s disease is a chronic systemic infection due to Tropheryma whipplei that commonly 177
120 manifests itself through gastrointestinal symptoms of malabsorption. It is a potentially fatal condition that is usually missed 178
121 due to its rarity and nonspecific symptoms.1 The disease has a prodromal stage noticeable for arthralgia and arthritis 179
122 followed by a steady state stage remarkable for diarrhea and weight loss. The average time between the two stages is six 180
123 years but immunosuppressive therapy can expedite clinical progression.2 We believe this occurred in this patient. Diagnosis 181
124 can be very challenging, especially without gastrointestinal manifestations. Articular symptoms can represent obscure 182
125 Whipple’s disease leading to misdiagnosis and administration of biological therapy that could aggravate existing Whipple’s 183
126 disease.3 Diagnosis is established by periodic acid-Schiff staining of small bowel biopsies.1 Patients can be treated with 184
127 ceftriaxone followed by long-term trimethoprim-sulfamethoxazole. Medical providers should be attentive to consider 185
128 Whipple’s disease in patients with abdominal and joint symptoms after omitting common diseases especially if symptoms 186
129 are refractory to treatment. Our patient had positive T whipplei immunolabeling. Her symptoms were unified in Whipple’s 187
130 disease. She was treated with ceftriaxone followed by trimethoprim-sulfamethoxazole. Her symptoms started to improve. 188
131 Inflammatory bowel disease is not usually associated with neurologic or skin symptoms like Whipple’s disease. Con- 189
132 nective tissue disease is less likely in this case given the negative autoimmune markers. Lymph nodes with histiocytes and 190
133 periodic acid-Schiff positive globules are not associated with AIDS. 191
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136 References 194
137 1. Schijf LJ, Becx MC, de Bruin PC, et al. Whipple’s disease: easily diagnosed, if considered. The Netherlands Journal of 195
138 Medicine 2008;66:392–395. 196
139 2. Fenollar F, Puéchal X, Raoult D. Whipple’s disease. The New England Journal of Medicine 2007;356:55–66. 197
140 3. Vanderschueren D, Dequeker J, Geboes K. Whipple’s disease in a patient with longstanding seronegative polyarthritis. 198
141 Scandinavian Journal of Rheumatology 1988;17:423–426. 199
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