Celiac disease associated with pritnary biliary cirrhosis in a Coast Salish native

A 41-year-old Coast Salish female was initially diagnosed with typical features of classical adult celiac disease. Clinical and pathological features of primary biliary cirrhosis were also present, along with a familial history of insulin-dependent diabetes. Later, childhood celiac disease was detected in a male first-degree relative with diabetes. These patients are the first reported natives in Canada with celiac disease, a disorder believed to be genetically based but dependent on environmental factors for its clinical expression. The recognition of a ‘new’ disease in the setting of an aboriginal population may reflect geographical and climatic factors that permitted subsistence of this culturally complex food-gathering society up until most recent historical times, followed by adaptation of this society to European-based agricultural methods, particularly wheat cultivation.

E PIDEMIOLCX., ICAL STUDI ES ON T l IE geographical d ismbuuon of cel1ac disease suggest that it occurs almost exclusively in Europea ns a nd their descendants, pa rtic ula rly those who have emigrated ro North A merica an<l Australia ( 1 ).In Ireland, for example, the prevalence of clinically apparent celiac <lisease is estimated to be as high as one m 300 (2).Interestingly, the re is a very high freque ncy of human le ukocyte BB antigen in western Irela nd, a gene tic marker known to be linked to a number of <l1sordcrs 1 induding celiac disease.In addi tio n, there is a very long agricultural history of wheat cultivation m Europe dating fro m at least 1000 BC (3).Such genetic and en vironmental factors appear to have played a critically important role m the clinical expression of celiac <lisease in diffe rent populations (4) .
While recem ep1de m1o log1cal studies of celiac d isease in Europe and other Mediterranean countries have been reported (5), this disorder has also been widely recognized in mhab1tants fro m a numher of other non-Eu ropea n countries (l ) anJ has occasio nally been detec ted in oche r non-Caucasian races, mcluding possibly Blacks (6,7), butnor in Asians (8).A lthough both genetic and en vmmme ntal fac t(>rs arc important ( 4 ), the very low case fatality rate of ccl1ac disease, m, relauve ran ry m must populations a nd wide variations m case ascertainmen t methods fo r Jif-FREEMAN fcrent countries have made ep1Jemiological studies of celiac disease more d ifficult (9).It has been estimated, for example, that up to one-half of all adult ccliac patients identified in some populations have few or no symptoms (9); obviously, then, case ascertainment wou ld substantially influence d ifferences in prevalence rates for celiac d isease that might be reported from different centres.Additional studies are required, therefore, to map more precisely the epidem1olog1cal topography of celiac Jisease on a global scale.
This report documents an adult Coast Salish female with celiac d isease, anJ explores possible environmental factors that may be significant historically in the appearance of a 'new disease' in the native peoples of British Columbia.
Because of diarrhea, endoscopic studies of the upper and lower gastrointestinal tracts were <lone and were normal.Small intestinal biopsies from the proximal small bowel, however, showed typical changes consistent with celtac disease.Gastric and colonic mucosa!biopsies fa iled to show changes of lymphocytic gastritis (23 ), or collagenous or lymphocytic colitis (24,25).A gluten-free diet resulted in resolution of the patient's diarrhea, weight gain of 6 kg and normalization of her small intestinal biopsies, resulting in the typical histological features of treated celiac disease.
Additional Coast Salish familial historical data revealed a 10-year-okl insulin-dependent diabetic daughter; in addition, the patient's sister and cwoyear-old nephew were diagnosed as diabetic.The nephew, a first-degree relative, also developed d iarrhea, and small intestinal biopsies Jone at another teaching hospital showed features typical of childhood celiac disease.

DISCUSSION
The native patient described in the present report had both celiac disease and primary biliary cirrhosis, two conditions that have previously been observed to coexist in over 20 patients from other populations (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22).In addition, both conditions are often associated with a range of ocher disorders having an immunological basis (21,26), including diabetes and thyroiditis, although evidence for a common genetic predisposition or a common immunological alteration has not yet been identified.Weight loss, malabsorption, bone disease, steatorrhea and elevated alkaline phosphatase activities are common features of both diseases so that at an early stage of their coexistence, celtac disease or primary biliary cmhos1s may not readily be recogrnzed.ln all patients reported with both conditions, regardless of geographical origin or race, restriction of dietary gluten caused cessation of diarrhea, as in the patient reported here, but abnormal liver chemistry tests were not improved despite a gluten-free Jiet.
A number of theories have been considered to explain the concomitant presence of celiac disease and pnmary biliary cirrhosis.One suggests that immune complexes are formed with a common antigernc basis, and this mechanism mediates tissue damage; no specific antigen, however, has been identified.Alcemattvely, diminished function of suppressor T cells in patients with both d iseases might allow effector cytotoxic lymphocytes to attack a modifymg antigen, such as gluten.These effector cells might then recognize an<l attack a patient's histocompatibilicy antigens, present m high concentrations in biliary as well as intestmal epithelial cells (23 ).Further studies are needed, however, to elucidate the very intriguing relationship between cel1ac disease anJ primary biliary cirrhosis.
The recognition of celiac disease in chis native patient has particular interest as a 'new' disorder appearing for the first time in this population.The Coast Salish seemed to have survived and attained one of the highest known levels of cultural complexity, subsisting largely on fishing as well as gathering of roots and bemes, rather than horticulture or herds (27).As a result of their rich, maritime temperate zone habitat, these peoples developed soc1et1es with permanent houses in villages of more than 1000 residents, social strat1fication with slaves an<l ranked nobility, elaborate ceremonies, multiple linguistic dialects and one of the world's great art styles (27).Early descriptions arc reported co describe the Coast Salish t ribes as a huntmg and fishing peoples without knowledge of soil cultivation methods (28).Although possible sources of potato an<l wheat cultivauon may have been Russian settlements in Alaska or some Sparnsh settlements ar Nootka Sound in British Columbia, most historical evidence suggests that the Coast Salish tribes acquired some cultivation techniques only after the establishment of Fore Langley in 1827 by the Hudson's Bay Company.This was followed by very rapid diffusion of the methods elsewhere in British Columbia (29).Previously, access to Euro-