History of Crohn’s Disease
There is no doubt that the existence of Crohn’s disease has pre-dated our ability to understand and define it. In fact, a precise definition continues to elude us to this day, relying mostly on phenotype to describe the diverse presentations of the disease. This lack of precision, compounded by rudimentary and perfunctory specimen evaluation techniques of the past, left Crohn’s disease an undiscovered entity until the 1932 seminal paper by Crohn and Ginzburg and Oppenheimer (CGO). We now recognize the incidence rate to be as high as 16 per 100,000 worldwide. The history of Crohn’s disease is a tale of discovery that is far from complete and has utilized many of the advances of medical science to improve the precision of both diagnosis and treatment.
Surgical therapy has evolved from primary treatment to being reserved for medically refractory or complicated cases. Medical therapy, once predominated by therapeutic nihilism between surgical episodes, has evolved with more sophisticated trials and therapies now including narrowly targeted drugs. Herein, we review the early descriptions of Crohn’s disease prior to its initial description, its origins at The Mount Sinai Hospital and the medical revolution that has occurred subsequent to the landmark 1932 article.
Crohn’s Disease Pre-1932
Reports consistent with Crohn’s disease abound in past literature, although definitive diagnosis in these prior case reports is conjectural at best.
The clinical and anatomic descriptions of Crohn’s disease were often confused with complicated appendicitis, intussusception, intestinal tumor, or infection (e.g., tuberculosis). Prior medical historians have reviewed old literature to find numerous cases suspected of Crohn’s disease including the King of France, Louis the XIII (1601- 1643). Louis the XIII’s medical record unveils a history of multiple chronic gastrointestinal complaints including perianal abscesses, bowel obstructions, and chronic diarrhea . Autopsy showed ulcerations of the small and large bowel with fistulas. The famous Italian pathologist G.B. Morgagni (1682- 1771), in his 80th year, published the “De Sedibus et Causis Morborum per Anatomen Indagatis,” a collection of cases and autopsies which also describes likely instances of inflammatory bowel disease . One of the first well-described cases of Crohn’s disease is attributed to Combe and Saunders in 1806 in which they published an account of stricture and thickening of the terminal ileum . Additional reports in the early 1800s are found within a series of case reports published by Abercrombie in 1828 entitled “Pathological and Practical Researches on Diseases of the Stomach, the Intestinal Canal, the Liver and other Viscera of the Abdomen” .
Abercrombie described cases suggestive of ileitis with associated bowel obstruction including that of a 63-year-old who died of a bowel obstruction with “great thickening and induration of the ileum” and a 20-year- old with “extensive inflammation of the ileum; the inflamed parts were extensively glued together, and pressed down into the cavity of the pelvis”. Lack of routine pathologic evaluation and rudimentary techniques available to study specimens limited the ability to differentiate cases of Crohn’s disease from other entities. At that time, many patients were diagnosed on the basis of gross anatomic findings rather than strict histologic or culture based evidence.
Crohn’s disease is an idiopathic, chronic, inflammatory disease of the gastrointestinal tract that primarily affects the small intestine and colon. Crohn’s disease is associated with significant morbidity, the need for treatment with sulfasalazine, mesalamine, corticosteroids and other immunomodulating agents, surgical resection of the intestine and a slightly increased mortality.
Knowledge of the natural history of Crohn’s disease (e.g. the likelihood of surgical intervention or the likelihood of experiencing a flare of Crohn’s disease in a given year) guides physicians when counselling Crohn’s disease patients and offering advice regarding treatment options. More importantly, Crohn’s disease is an idiopathic disorder, which may be caused by acombination of environmental and genetic factors.
In the late 1800s and early 1900s, increased rigor in the examination of anatomic and microscopic pathology of surgical specimens led to identification and study of non-malignant inflammatory abdominal masses of the ileocecal region. Many of the great names of surgery such as Billroth, Bassini, and Hartmann have been referenced describing cases of chronic fibrotic or ulcerative ileocecal disease which were often believed to be due to tuberculosis .
A notable report in 1900 came from Lartigau, a pathologist from Columbia College of Physicians and Surgeons, in which he presented an abstract before the New York Pathological Society describing an ileocecal lesion of “such a character that the inflammatory hyperplasia or ‘pseudo-neoplasm’ may easily be mistaken clinically for tumor-formation of the locality resembling carcinoma”. In his review, he commented upon many case reports in the literature which he believed to be tuberculous lesions of the ileum and cecum, which may instead have represented Crohn’s disease in a number of instances. Dieluafoy’s textbook of medicine published in 1911 extensively reviewed surgical cases of stenosis of the terminal ileum and cecum with non-caseating necrosis and bowel obstruction believed to be either tuberculosis or lymphosarcoma (i.e., lymphoma) .
Despite the likelihood of Crohn’s disease present in these reports, its differentiation from intestinal tuberculosis is not trivial, even now, in the endemic population, so definitive conclusions should be withheld. Notable case reports and series were published by Moynihan and Braun. Glasgow surgeon T. Kennedy Dalziel in 1913 published a series of cases with similarity to tuberculosis pathology of intestinal specimens; however, he was unable to prove bacterial pathogenesis . In 1923, A.O. Wilensky and Eli Moschowitz from the Mount Sinai Hospital in New York published a review of four cases of chronic and nonspecific granuloma of the intestine which had been assumed to be tuberculosis or neoplastic but these were histologically ruled out. Further reports soon followed recognizing cases of nonspecific intestinal granuloma. Certainly many additional examples of patients likely afflicted with Crohn’s disease exist beyond what was published or widely known pre-1932, as the true disease prevalence was not affected by our defining it. These were the important events leading up to the identification of Crohn’s disease as a separate clinical entity in the 1932 CGO paper.
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J. E. Bornstein , MD
Division of Colon and Rectal Surgery, Department of Surgery , Icahn School of Medicine at Mount Sinai , New York , NY 10029 , USA
R. M. Steinhagen , MD
Division of Colon and Rectal Surgery , Mount Sinai Medical Center , One Gustave L. Levy Place , Box 1259 , New York , NY 10029 , USA
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