Chronic Abdominal Pain in a 47-Year-Old Woman

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Question: A 47-year-old woman presented for a second opinion for a two month history of unexplained epigastric pain and a 5 kg weight loss. She described the pain as intermittent and exacerbated by meals. She denied other accompanying gastrointestinal symptoms including nausea, vomiting, or changes in her bowel habits. Her past medical history was significant for chronic sinusitis and a prior Cesarean section. Physical examination revealed moderate tenderness to palpation over the lower sternal and epigastric region without associated rebound tenderness or guarding. The remainder of the physical examination including vital signs was unremarkable. An evaluation prior to presentation including computed tomography (CT) of the abdomen and pelvis, esophagogastroduodenoscopy, and hepatobiliary scan had been unrevealing. Laboratory investigation was significant for elevated C-reactive protein and erythrocyte sedimentation rate (280 mg/L and 65 mm/1h, respectively). CT angiography demonstrated patent mesenteric vasculature and subtle beading of the right and left renal arteries (Figure A, arrows). In the setting of ongoing abdominal pain, a conventional mesenteric and renal angiography was performed and showed multiple small- to medium-sized branch aneurysms, areas of luminal irregularity, and occlusive disease (Figures B and C, arrows). Additional laboratory studies revealed normal antinuclear antibodies, cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), proteinase 3 antibodies (PR-3), and cryoglobulin levels. Which of the following diagnoses likely accounts for the patient’s abdominal pain?A.Microscopic polyangiitisB.Granulomatosis with polyangiitisC.Polyarteritis nodosaD.Eosinophilic granulomatosis with polyangiitis Look on page 628 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is C. The radiographic findings of aneurysms involving small- to medium-sized arteries on angiography (Figures B and C) in combination with normal laboratory testing for ANCA, PR-3, and cryoglobulin levels in the setting of chronic abdominal pain and weight loss are most consistent with a diagnosis of PAN. In this case, following negative testing for hepatitis B (HBV) and C virus (HCV), she was initiated on combination therapy with a prednisone taper and intravenous cyclophosphamide and achieved disease remission with resolution of her symptoms after six months of therapy. Although vasculitides are infrequently encountered in clinical practice, gastrointestinal (GI) involvement by these conditions is common and often manifest with non-specific symptoms including abdominal pain, nausea, vomiting, diarrhea, and bleeding. This case highlights how radiographic features and laboratory data may be used to distinguish between vasculitic entities. Microscopic polyangiitis (answer A) is an ANCA-associated vasculitis (AAV) characterized by involvement of small arteries and veins which conflicts with the findings in this case. Histology-confirmed features of necrotizing granulomatous vasculitis facilitate a diagnosis of granulomatosis polyangiitis (answer B) which is also frequently associated with c-ANCA and PR3 antibodies. Eosinophilic granulomatosis with polyangiitis (answer D), or Churg-Strauss syndrome, while an important consideration in patients with unexplained abdominal pain, is unlikely in the absence of concomitant asthma and peripheral eosinophilia and would not account for the described radiographic features. PAN represents a non-AAV capable of predominately affecting the muscular medium-sized arteries of any organ system except the lungs.1De Virgilio A. Greco A. Magliulo G. et al.Polyarteritis nodosa: A contemporary overview.Autoimmun Rev. 2016; 15: 564-570Crossref PubMed Scopus (121) Google Scholar GI manifestations of this condition are variable but abdominal pain, often secondary to ischemia resulting from mesenteric arteritis2Ebert E.C. Hagspiel K.D. Nagar M. et al.Gastrointestinal involvement in polyarteritis nodosa.Clin Gastroenterol Hepatol. 2008; 6: 960-966Abstract Full Text Full Text PDF PubMed Scopus (56) Google Scholar is the most common.3Levine S.M. Hellmann D.B. Stone J.H. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients.Am J Med. 2002; 112: 338-349Abstract Full Text Full Text PDF Scopus (122) Google Scholar While PAN is a clinical diagnosis and does not require histologic confirmation, its association with several infections including HBV and HCV infection is well-recognized and should prompt evaluation for these conditions if identified de novo. Treatment is tailored toward disease severity and the presence of concomitant disease processes. Induction of remission in moderate to severe disease is usually achieved with combination glucocorticoids and cyclophosphamide,1De Virgilio A. Greco A. Magliulo G. et al.Polyarteritis nodosa: A contemporary overview.Autoimmun Rev. 2016; 15: 564-570Crossref PubMed Scopus (121) Google Scholar although antiviral therapy is essential in HBV and HCV infection while limiting immunosuppressive medications. CME Exam 2: Chronic Abdominal Pain in a 47-Year-Old WomanGastroenterologyVol. 155Issue 3Preview Full-Text PDF