Splenic Lesions in a Patient With Crohn’s Disease

Abstract

Question: A 35-year-old woman with severe Crohn’s disease presented for evaluation of a persistent continuous low-grade fever and asthenia for few days. She was treated at that time with thalidomide (100 mg/d) and oral prednisone (20 mg/d) Physical examination was unremarkable. Her past medical history was significant for a colonic and perianal Crohn’s disease refractory to medical therapy including corticoids, azathioprine, infliximab, and adalimumab. She underwent total proctocolectomy with definitive ileostomy. Laboratory tests showed elevated white blood cells (14,700/μL) and C-reactive protein (57 mg/L; normal, <5). Liver function tests, amylase, and lipase levels were normal. All microbiological samples were negative. Abdominal CT (Figure A) demonstrated splenic hypodense and multiloculated lesions suggestive of splenic abscesses. Follow-up MRI (Figure B) showed progression of these splenic lesions with heterogeneous high signal on T2-weighted images and peripheral contrast enhancement. Ultrasound-guided needle puncture of the cysts showed hemorrhagic liquid with damaged neutrophils and macrophages; bacteriologic, mycologic, and parasitologic examinations were negative. Because of persistent fever and risk of splenic rupture, the patient underwent diagnostic and therapeutic splenectomy. Postoperative recovery was uneventful. Macroscopically (Figure C), there were round masses (the largest was 6 cm) into the spleen, filled with a white-grayish fluid and thick wall. Figure D shows a low-power view of microscopic sections (H&E staining). What is the diagnosis? Look on page 716 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Microscopically, the lesions were characterized by a necrotic center, containing damaged neutrophils and a peripheral area containing histiocytes and macrophages. Gram, Ziehl-Neelsen, periodic acid Schiff, and Groccott stains did not identify pathogenic microorganisms. Aseptic abscesses syndrome is a systemic disorder of unknown etiology. Aseptic abscesses are characterized by deep, sterile, round lesions consisting of neutrophils that do not respond to antibiotics, but usually improve with corticosteroids. Typical presentation of aseptic abscesses syndrome includes fever, abdominal pain, leukocytosis, and presence of intraabdominal aseptic lesions.1André M.F. Piette J.C. Kémény J.L. et al.Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature.Medicine. 2007; 84: 145-161Crossref Scopus (111) Google Scholar The abscesses involve the spleen in 90% of patients.2Coat N. Le Berre-Heresbach N. Poinsignon Y. et al.Crohn’s disease complicated by multiple and recurrent aseptic splenic abscesses.Gastroenterol Clin Biol. 2001; 25: 425-428PubMed Google Scholar Rarely, abscesses can be extraabdominal, such as pulmonary, cerebral, or cutaneous. Aseptic abscesses have been described in inflammatory bowel disease, especially in Crohn’s disease and in other inflammatory diseases.3Zakout R. Fonseca M. Santos J.M. et al.Multiple aseptic liver abscesses as the initial manifestation of Crohn’s disease: report of a case.Dis Colon Rectum. 2009; 52: 343-345Crossref PubMed Scopus (14) Google Scholar Diagnosis relies on a combination of a typical clinical and radiological presentation (ie, deep abscesses), the pathologic findings with neutrophilic features, and the exclusion of alternative diagnosis, especially infectious disease. All the infectious investigations have to be negative. Antibiotics are inefficient but there is marked improvement with corticosteroids (1 mg/kg of prednisone or equivalent recommended).1André M.F. Piette J.C. Kémény J.L. et al.Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature.Medicine. 2007; 84: 145-161Crossref Scopus (111) Google Scholar It is often a relapsing condition (within the spleen or in other locations after splenectomy) and additional immunosuppressive therapy is required in almost one-half of the patients.1André M.F. Piette J.C. Kémény J.L. et al.Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature.Medicine. 2007; 84: 145-161Crossref Scopus (111) Google Scholar