A Brief History of Celiac Disease Long, Long Ago, Man Was a Simple Hunter/Gatherer
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SUMMER 2007 | VOL 7 ISSUE 3 A PUBLICATION OF THE UNIVERSITY OF CHICAGO CELIAC DISEASE CENTER IMPROVING LIVES THROUGH AWARENESS, impact EDUCATION, AND RESEARCH A Brief History of Celiac Disease Long, long ago, man was a simple hunter/gatherer. His diet consisted of fruits, nuts, perhaps tubers and the occasional feast of meat. But eventually, he learned to cultivate plants, and the agricultural revolution began. Soon, the hunter/gatherer way of life was replaced by domestication of crops and animals. As habits changed, some unanticipated adults causing malnutrition and characterized problems arose. The human gut had developed, by a gas-distended abdomen. He even went on over more than 2 million years, into a to suggest dietetic treatment, writing: “ Some sophisticated organ that could tolerate food patients have appeared to derive considerable antigens that were staples of the human diet over advantage from living almost entirely upon hundreds of thousands of years. But how would rice.” Baillie’s observations, however, went Dr. Stefano Guandalini, M.D. it react to new antigens, suddenly appearing practically unnoticed, and it was for the in the diet? The agricultural revolution of the English doctor Samuel Gee, a leading authority the damage to the intestinal mucosa. Yet some Neolithic period generated a whole battery of in pediatric diseases, to take full credit for the of the present-day findings, which we tend food antigens previously unknown to man, modern description of celiac disease some 75 to consider as recent advances, were indeed including protein from cow, goat, and donkey years later, when he gave a lecture to medical well known long ago, including that celiac milk, as well as birds’ eggs and cereals. Most students on the “celiac affection,” the milestone disease could be present without diarrhea, individuals were able to adapt. Among those description of this disorder in modern times. the protective role of breast-feeding in the who could not, food intolerances appeared and Like Baillie, Gee sensed that “if the patient development and severity of celiac disease, celiac disease was born. can be cured at all, it must be by means only recently documented, and the increased Some 8,000 years after its onset, celiac of diet.” He added that “the allowance of incidence in families, particularly twins. disease was identified and named. A clever farinaceous food must be small”, and also In the 1920s a new dietetic treatment erupted Greek physician named Aretaeus of Cappadocia, described “a child who was fed upon a quart on the scene and for decades established itself living in the first century AD, wrote about of the best Dutch mussels daily, throve as the cornerstone of therapy: the banana “The Coeliac Affection.” In fact, he named wonderfully, but relapsed when the season diet. In 1924 Sidney Haas described his it “koiliakos” after the Greek word “koelia” for mussels was over; next season he could successful treatment of eight children whom (abdomen). His description: “If the stomach not be prevailed upon to take them.” Thus he had diagnosed with celiac disease. Based be irretentive of the food and if it pass through he documents the improvement following on his previous success in treating a case of undigested and crude, and nothing ascends into the introduction of a gluten-free diet, and the anorexia with a banana diet, he elected to the body, we call such persons coeliacs”. relapse after reintroduction of gluten. try to experiment with the same diet in these Another 17 centuries went by, and in the As the decades passed, there was still no eight children who were also anorexic. He early 19th century a Dr. Mathew Baillie, clue as to what could be causing celiac disease published ten cases, eight of them treated probably unaware of Aretaeus, published his and no hint (in spite of autopsies frequently (“clinically cured”) with the banana diet, whilst observations on a chronic diarrheal disorder of performed given the high mortality rate) of >> continued on page 2 1 >> continued from page 1 the two untreated died. This paper encountered complete clinical remission on a gluten-free enormous success and for decades the banana diet, followed by the documentation of the diet enjoyed wide popularity. Indeed it normalization of the lesion, and finally by IMPROVING LIVES THROUGH AWARENESS, benefited a large number of celiac children its recurrence once gluten was reintroduced EDUCATION, AND RESEARCH and probably prevented many deaths. The diet into the diet. These criteria were formalized Stefano Guandalini, M.D. specifically excluded bread, crackers, potatoes in 1969 by a panel of experts in the newly Founder and Medical Director and all cereals, and it’s easy to argue that its born European Society for Pediatric University of Chicago Comer Children’s success was based on the elimination of gluten- Gastroenterology (today ESPGHAN) as the Hospital Chief, Pediatric Gastroenterology, containing grains. “Interlaken criteria” which for over 20 years Hepatology and Nutrition Haas was very proud of his insight that served worldwide as the accepted diagnostic carbohydrates were the culprit and he was standard. Advisory Board highly resistant to other viewpoints, no matter The Interlaken criteria, however, overlooked Deborah Gordon, Board Chair how well documented. Indeed, even as late an important discovery made a few years Leonard Gail, Vice Chair as forty years later, well after Dicke, a Dutch earlier: that celiac children presented in their Scott Schwefel, Treasurer pediatrician, had convincingly shown that blood antibodies caused by the ingestion of Andrea Hosbein, Membership wheat protein, not starch, was the only culprit, gluten. The first category to be discovered Lara Field, M.S., R.D., Secretary Haas still insisted that with his banana diet “all were the anti-gliadin antibodies, detected and Susan Blumenfeld patients are cured by the specific carbohydrate reported by Berger in 1964. Seven years later Mark De Meo, M.D. diet, a cure which is permanent without Seah et al. identified for the first time not an LuEllen Giera relapse.” anti-food protein, but an actual auto-antibody Stuart Gordon The breakthrough that Haas chose to in the serum of celiac children: the anti- Bana Jabri, M.D., Ph.D. downplay was to change forever our view of reticulins, although it took however several Tripti Kasal celiac disease. Dicke had noticed that during years before their diagnostic utility was fully Kim Koeller bread shortages in the Netherlands caused by appreciated. Margaret Mochel, R.N. World War II, children with celiac disease During the 1980’s it became increasingly Gail Pierce improved. He also saw that when Allied planes clear that CD could be associated with other Carol Semrad, M.D. dropped bread into the Netherlands, they conditions, mostly autoimmune disorders such Sara Star, M.D. quickly deteriorated. A few years later, working as type 1 diabetes, but also some syndromes Sueson Vess with others, he produced a series of seminal such as Down. It was also apparent that Peggy Wagener papers, documenting for the first time the role CD was changing patterns of presentation, Wendy Weil, D.P.M. that gluten from wheat and rye plays in celiac becoming less an intestinal disorder, and more disease. a variety of extra-intestinal symptoms and Carol McCarthy Shilson The next major breakthrough came in the signs. Executive Director mid-50s, when Margot Shiner described a In the late 80’s, a large multicenter Italian new jejunal biopsy apparatus with which she study that I conducted demonstrated that by Ronit Rose successfully reached and biopsied the distal relying on strict clinical and laboratoristic Program Manager duodenum. This – and the development of the criteria, a correct diagnosis of CD could be less cumbersome capsule developed shortly reached in 95% of cases by limiting to the one The University of Chicago Celiac Disease after by the American Lieutenant Colonel initial biopsy, and new diagnostic guidelines Center is dedicated to raising awareness Crosby -- finally allowed doctors to link the were published in 1990 by ESPGHAN, rates and meeting the needs of people disease with a specific, recognizable pattern guidelines that stand to this day. affected by celiac disease nationwide of damage to the proximal small intestinal After 1990, CD was increasingly accepted through education, research and advocacy. mucosa. as an example of an autoimmune disease, The contents of this newsletter are Thus, at the dawn of the 60’s we had three associated with a specific gene (either DQ2 not intended to diagnose or recommend important elements: the knowledge that gluten or DQ8) and the missing autoantigen was treatment for celiac disease. Please consult is the triggering agent for celiac disease; the finally identified in the enzyme “tissue your healthcare provider with questions notion that there was a remarkable and easy transglutaminase”. At long last, there was about your condition. identifiable mucosal lesion; and finally the universal acceptance that celiac disease is an For more information about the availability of an instrument to obtain biopsies autoimmune condition whose trigger (gluten) University of Chicago Celiac Disease and begin to unravel the mystery of celiac and autoantigen (tissue transglutaminase) are Center, please contact our office: disease pathogenesis (see below). known. In the mid to late 60’s, it had become clear What the future will bring is in the hands University of Chicago Celiac Disease Center that CD could be diagnosed with the jejunal of the gods: we can be sure, however, that we 5839 S. Maryland Ave., MC 4069 biopsy showing atrophy of the villi. However, won’t have to wait 17 more centuries before Chicago, IL 60637-1470 since that lesion could have other causes, the next earth-shaking discovery in celiac Ph.: (773) 702-7593 the medical community urged doctors not disease will be made.