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Redalyc.Crohn Disease Autopsy and Case Reports E-ISSN: 2236-1960 [email protected] Hospital Universitário da Universidade de São Paulo Brasil Geller, Stephen A.; de Campos, Fernando P. F. Crohn disease Autopsy and Case Reports, vol. 5, núm. 2, abril-junio, 2015, pp. 5-8 Hospital Universitário da Universidade de São Paulo São Paulo, Brasil Available in: http://www.redalyc.org/articulo.oa?id=576060829002 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative Image in focus Imagem em foco Crohn disease Stephen A Gellerab, Fernando P F de Camposc Geller SA, Campos FPF. Crohn disease. Autopsy Case Rep [Internet]. 2015; 5(2):5-8. http://dx.doi.org/10.4322/acr.2015.001 Dr. Stephen A Geller personal archive Aretaeus of Cappadocia (1st century CE) described patient dying after a prolonged dysentery-like course. a young man with chronic and recurring abdominal Louis XIII, King of France (1601-1643) died at the age distress possibly representing the first recorded instance of 42 after decades of abdominal pain, fever, bloody of Crohn disease (CD).1-3 More evidence that this disease diarrhea and rectal/perianal abscesses.4 However, it was was extant for many years comes from the description in 1761 that Giovanni Battista Morgagni (1682-1771), of the 10th century English king, Alfred “the great,” a towering figure in the history of medicine, described who suffered for many years from what was a typical a 20-year-old man with typical regional ileitis, who enteritis but also had anal fistula and/or abscess.4 In died. Morgagni autopsied him and described typical medieval times, Antonio Benivieni (1443-1502) CD lesions of both the small and large intestine, described the multi-ulcerated small intestine in a including erosions, ulcerations and perforations with a Department of Pathology and Laboratory Medicine – David Geffen School of Medicine – University of California, Los Angeles/CA – USA. b Department of Pathology and Laboratory Medicine – Weill Cornell Medical College, New York/NY – USA. c Internal Medicine Division – Hospital Universitário – Universidade de São Paulo, São Paulo/SP – Brazil. Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2014. This is an Open Access article distributed of terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non- commercial use, distribution, and reproduction in any medium provided article is properly cited. Crohn disease lymphadenopathy. In the following 150 years, cases suggests there is an inherited abnormal response to the likely to be Crohn disease were reported by Mathew high burden of bacteria in the gastrointestinal lumen, Baillie, Colles, Cruveilhier, Fenwick, Fröhlich and many resulting in an exaggerated inflammatory response.11 others. Notable was the description of “singular case A third theory claims that these genetic defects are of stricture and thickening of the ileum” by Coombe responsible for a diminished inflammatory response and Saunders in 1813 and “inflammatory fibrous and therefore accumulation of stimuli in the intestinal colon tumour” by Rudolph Virchow.5,6 None of these, lumen which activate the adaptive immune system.12 however, described completely the unique pathological Clinical manifestations vary greatly in the manner and clinical entity that is Crohn disease, although the gastrointestinal and extra-intestinal involvement is Scottish surgeon Thomas Kennedy Dalziel (1861-1924), expressed. The majority of patients have small bowel in his report of “chronic interstitial enteritis,” came involvement, predominantly in the ileum, while as close. many as 20% have the disease confined to the colon. It remained to the group of clinician-investigators Quite frequently, symptoms are present two to three at The Mount Sinai Hospital, New York, Burrill B. years before the diagnosis is established. Patients Crohn, Gordon Oppenheimer and Leon Ginzburg, to typically have diarrhea, often bloody, abdominal pain, publish the classic paper, entitled “Regional ileitis: a fatigue, weight loss with emaciation, and fever.13 pathological and clinical entity” in 19327-9, identifying Transmural inflammation is characteristic often 14 patients with symptoms and surgical intestinal leading to sinus tract(s) formation which, because abnormalities, which they recognized as a singular of the characteristic serositis, leads to bowel-to- entity. bowel adhesions and subsequent fistulas. The image Crohn disease, as this entity was later coined, is above shows many of the characteristic features of a chronic inflammatory disease of unknown etiology, Crohn disease with adherent loops of thick-walled which potentially involves the gastrointestinal tract (“transmural inflammation”) bowel showing acute and from the mouth to the perianal area. chronic serositis and fistula formation as evidenced by 14 CD may affect people in early childhood until the metal probes. Perianal involvement is often seen late adulthood, but younger individuals, including with pain, fissures, perianal abscesses, and fistulas. teenagers and young adults, are more often affected. Other gastrointestinal manifestations include oral Afflicted patients have a significant decrease in the aphthous ulcers, odynophagia or dysphagia, gastric 15 16 quality of life because of the marked morbidity. outlet obstruction and gallstones. This disease also has a significant economic impact Extraintestinal manifestations that can be seen since most patients are affected in their productive are arthritis, skin and eye involvement, primary years. The incidence seems to be increasing worldwide, sclerosing cholangitis leading to secondary biliary even in low-incidence regions, probably because cirrhosis, secondary amyloidosis, hypercoagulability, of better diagnosis. Environmental factors as well renal stones, osteoporosis, vitamin B12 deficiency and as changes in life style may also have an impact. pulmonary involvement.17-22 The highest incidence rate has been reported in Canada Currently, diagnosis of CD usually requires imaging (248/100,000 inhabitants), while in Asia the rate is less studies, endoscopy, serologic markers and evaluation than 6.3/100,000 inhabitants. of inflammatory-marker protein tests. Although the etiology is still not understood, Colonoscopy with terminal ileum examination several theories have been proposed over the years. is used to establish the diagnosis of ileocolonic CD. Mycobacterium avium subspecies paratuberculosis Focal ulcerations intermingled with normal-appearing has been implicated in the pathogenesis of CD, (“skip”) areas is typical. Polypoid mucosal changes give reinforced by the great similarity between CD and a distinctive cobblestone appearance, characteristic of cattle’s Johne disease. This association has been CD, although, in the large intestine, longitudal ulcers, supported by many studies10 but has not been proven by resembling furrows made by a garden rake, are often either identification of mycobacteria in tissue samples seen. Carcinoma can develop after many years of or by consistent culture of an organism. Another theory active disease, but is exceedingly rare with modern 6 Autopsy and Case Reports 2015; 5(2):5-8 Geller SA, Campos FPF methods of following patients as well as with effective 10. Naser SA, Sagramsingh SR, Naser AS, Thanigachalam S. therapies.23 Wireless capsule endoscopy is an option Mycobacterium avium subspecies paratuberculosis causes Crohn’s disease in some inflammatory bowel disease to study the proximal segments of the intestine, when patients. World J Gastroenterol. 2014;20(23):7403- 24 ileocolonoscopy fails to disclose a diagnostic lesion. 15. http://dx.doi.org/10.3748/wjg.v20.i23.7403. The upper gastrointestinal tract may also be studied PMid:24966610. by small bowel follow-through series, which allows 11. Marks DJB, Segal AW. Innate immunity in inflammatory for the documentation of the length and location bowel disease: a disease hypothesis. J Pathol. of any strictures. Serologic markers are often helpful 2008;214(2):260-6. http://dx.doi.org/10.1002/ in distinguishing CD from ulcerative colitis, as well path.2291. PMid:18161747. as in monitoring the disease course. In this setting, 12. Comalada M, Peppelenbosch MP. Impaired innate immunity antineutrophil cytoplasmic antibody (pANCA) and in Crohn’s disease. Trends Mol Med. 2006;12(9):397-9. anti-Saccharomyces cerevisiae antibody (ASCA) have http://dx.doi.org/10.1016/j.molmed.2006.07.005. a sensitivity of 40% - 60% with greater than 90% PMid:16890491. specificity in distinguishing patients with inflammatory 13. Mekhjian HS, Switz DM, Melnyk CS, Rankin GB, Brooks bowel disease from controls.25 RK. Clinical features and natural history of Crohn’s disease. Gastroenterology. 1979;77(4 Pt 2):898-906. Keywords: Crohn Disease, Pathology, History of PMid:381094. Medicine. 14. Geller SA. Pathology of inflammatory bowel diseases. In: Targan S, Shanahan F, editors. Inflammatory bowel REFERENCES disease. Baltimore: Williams and Wilkins; 1994. chap. 23; p. 336-351. 1. Banerjee AK, Peters TJ. The history of Crohn’s disease. J R 15. Annunziata ML, Caviglia R, Papparella LG, Cicala M. Coll Physicians Lond. 1989;23(2):121-4. PMid:2659781. Upper gastrointestinal involvement of Crohn’s disease: 2. Mulder DJ, Noble AJ, Justinich CJ, Duffin JM. A tale a prospective study on the role of upper endoscopy in
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