Crohn’s disease

Description

What is the actual eponymous medical sign/syndrome/repair/classification…

History of Crohn’s disease

1769 – Giovanni Battista Morgagni (1682 – 1771) provided an early description of Ulceration of the intestines in De Sedibus et Causis Morborum (The Seats and Causes of Diseases). He describes the death and autopsy of a 20-year-old male patient who succumbed to a longstanding illness of fever, abdominal pain, and bloody diarrhoea. The autopsy revealed perforations and transmural inflammation with ulceration stretching from the terminal ileum to “two hands breadth” along the colon.

The parts in which ulceration had taken place were the extremity of the ileum, and the contiguous portion of the colon, to the extent of two spans ; and the inner surface of this tract was gangrenous, and consequently might easily be perforated. Some of the adjacent mesenteric glands were enlarged…

Morgagni 1761 Book III; Letter XXXI; Article 2

1806 – Dr. William Saunders (1743-1817) communicated, to the Royal College of Physicians in London, ‘A singular case of stricture and thickening of the ileum‘, by Charles Coombe (1743–1817). The presentation included clinical history and autopsy findings…

[the patient] had been for many years troubled with flatulency and complaints in the bowels, attended with costiveness, a quick pulse and an irregular intermittent fever…He especially complained of wind and great costiveness, and about two to three hours after eating, of excessive pain in the bowels.

[at autopsy]…the lower part of the ileum as far as the colon, was contracted, for the space of three feet, to the size of a turkey’s quill.

Coombe, Saunders 1806 [published 1813]

1909 – Braun described several cases of inflammatory disease masses involving the small intestine

1913 – Dalziel read a paper on ‘Chronic Interstitial Enteritis‘ at the Annual B.M.A. Conference at Brighton. reported, in detail, six cases similar to those of Braun, in which tuberculosis (though suspected) was excluded by careful bacteriological studies. This evoked some acceptance that a benign, chronic, granulomatous condition of the small intestine existed which was not tuberculosis.

He described 8 cases, with a detailed account of the pathology ; an attempt had been made to find a causative organism, but had revealed only B. coli in one case. There was no evidence of tuberculosis. He concluded:

As far as I am aware the disease has not been previously described, but it seems probable that many cases must have been seen and have been diagnosed as tuberculous.

1932 – Burrill Crohn, along with Leon Ginzburg and Gordon Oppenheimer, published a case series of 14 patients aged between 17-years to 52-years, with the following description:

We propose to describe, in its pathologic and clinical details, a disease of the terminal ileum, affecting mainly young adults, characterized by a subacute or chronic necrotizing and cicatrizing inflammation. The ulceration of the mucosa is accompanied by a disproportionate connective tissue reaction of the remaining walls of the involved intestine, a process which frequently leads to stenosis of the lumen of the intestine, associated with the formation of multiple fistulas. The disease is clinically featured by symptoms resembling those of ulcerative colitis, ie, fever, diarrhea, emaciation, and a mass in the right iliac fossa usually requiring surgical resection. The etiology is unknown.

Crohn, Ginzburg, Oppenheimer 1932

1933 – Franklin Harris et al published an article titled ”Chronic cicatrizing enteritis: Regional ileitis (Crohn). A new surgical entity” as the first published reference to Crohn, recognising the term ‘regional ileitis’ but proposing ‘cicatrizing enteritis’ as an alternative.

1934 – John Kantor presented series of 6 cases (four operated on by AA Berg with pathology reviewed by Dr. Paul Klemperer at the Mount Sinai Hospital) all cases diagnosed nonspecific ulcerative granulomatous inflammation of the terminal ileum according to the criteria presented by Crohn and his collaborators in 1932.

1939 – Geoffrey Hadfield, professor of pathology at the University of London, evaluated the histological changes found in the terminal ileum and its related lymph nodes in 20 cases of regional ileitis:

The earliest and possibly the specific histological lesion of regional ileitis is lymphadenoid hyperplasia with the formation of non-caseating giant-cell systems in the submucosa. Acid-fast bacilli cannot be demonstrated histologically in these lesions. This lesion is also present in the regional lymph-nodes. Ulceration and fistulae are secondary to this lesion in the submucosa and the obstructive lymphoedema which it produces.

Hadfield 1939

1950 – George Armitage and Michael Wilson

Associated Persons

• Giovanni Battista Morgagni (1682 – 1771)
• Charles Coombe (1743–1817)
• John Abercrombie (1780 – 1844)
• Thomas Kennedy Dalziel (1861 – 1924)
• Eli Moschcowitz (1879 – 1964)
• Burrill Bernard Crohn (1884 – 1983)
• Leon Ginzburg (1898 – 1988)
• Gordon David Oppenheimer (1900 – 1974)

Alternative names

• Chronic interstitial enteritis (Dalziel, 1913)
• Non-specific granuloma (Moschowitz, 1927)
• Regional ileitis (Crohn, 1932)
• Regional enteritis, Regional enterocolitis, Cicatrizing enteritis

References

Historical references

• Morgagni GB. Ulceration of the intestines with flaccidity of the heart. In: The seats and causes of diseases, investigated by anatomy 1761 [Book III; Letter XXXI; Article 2] 1822 translation Vol II: 83-90
• Coombe C, Saunders W. A singular case of stricture and thickening of the ileum. Medical transactions 1813; 4: 16-21.
• Dalziel TK. Chronic Interstitial Enteritis. Br Med J 1913; 2: 1068-1070.
• Crohn BB, Ginzburg L, Oppenheimer GD. Regional ileitis; a pathologic and clinical entity. JAMA, 1932; 99: 1323-1329.
• Harris F, Bell G, Brunn H. Chronic cicatrizing enteritis: Regional enteritis (Crohn). Surgery, Gynecology & Obstetrics 1933; 57: 637-645.

• Kantor JL. Regional (terminal) ileitis: its roentgen diagnosis. JAMA 1934; 103: 2016-21
• Hadfield G. The Primary Histological Lesion of Regional Ileitis, Lancet, 1939; 234(6058): 773-776
• Armitage G, Wilson M. Crohn’s disease; a survey of the literature and a report on 34 cases. Br J Surg. 1950; 38(150): 182‐193.
• Fein HD. The History of Crohn’s Disease. In: Korelitz B.I. (eds) Inflammatory Bowel Disease. 1982: 1-3
• Kirsner JB. Landmark perspective. Crohn’s disease. JAMA. 1984;251(1):80‐81.
• Fielding JF. Crohn’s disease and Dalziel’s syndrome. A history. J Clin Gastroenterol. 1988; 10(3): 279‐285.
• Harmer M. Crohn’s disease–a misnomer? Bristol Med Chir J. 1988;103(1):9‐10.
• Fazio VW, Aufses AH Jr. Evolution of surgery for Crohn’s disease: a century of progress. Dis Colon Rectum. 1999; 42(8): 979‐988.
• Baron JH. Inflammatory bowel disease up to 1932. Mt Sinai J Med. 2000; 67(3): 174‐189.
• Janowitz HD. Inflammatory bowel disease after 1932. Mt Sinai J Med. 2000; 67(3): 190‐197.
• Kirsner JB. Historical origins of current IBD concepts. World J Gastroenterol. 2001;7(2):175‐184.
• Aufses AH Jr. The history of Crohn’s disease. Surg Clin North Am. 2001; 81(1): 1‐vii.