Adult celiac disease with severe or partial villous atrophy: a comparative study

BACKGROUND AND AIMS: While severe villous atrophy (SVA) is the most typical histological feature in adult celiac disease (ACD), partial villous atrophy (PVA) is now also frequently found. So far, the impact of the severity of villous atrophy on the clinical presentation of ACD has been scarcely investigated. We aimed to compare the clinical, biological and immune features and outcomes in ACD patients presenting with PVA at diagnosis versus patients with SVA. PATIENTS AND METHODS: Medical files of 48 patients with ACD diagnosed between 1992 and 2003 were retrospectively studied. The diagnosis was based on the presence of intestinal villous atrophy, with increases in intraepithelial lymphocytes and circulating celiac specific antibodies. Villous atrophy was classified as severe (subtotal and total) or partial. Symptoms, biological signs of malabsorption, immune markers, bone mineral density at diagnosis and response to gluten-free diet were recorded. RESULTS: At diagnosis, ten patients (four M/six F) had PVA and 38 patients (five M/33 F) had SVA, with a median age of 54 and 33 years, respectively (p<0.05). Positivity for specific antibodies, HLA typing and frequency of autoimmune disease at diagnosis were similar in both PVA and SVA patients, as was their response to gluten-free diet. Diarrhea, malabsorption syndrome and osteopenia were independent of the degree of villous atrophy. CONCLUSION: PVA was observed in 21% of patients with ACD. Except for their older age at diagnosis, patients with PVA presented with similar clinical, biological and immune characteristics and outcomes as did patients with SVA.     

Adult celiac disease with secondary hyperparathyroidism

After a pregnancy the 26-year-old patient complained of pain in several joints. Waddling gait and light-grey excrements were conspicuous. Radiologically signs of calcium salt reduction with hank-like bone structures in both forearms. Hypocalcaemia, hyperphosphataemia, parthormone in the serum increased. Villi could not be proved by aspiration biopsy in the small intestine. The bone biopsy spoke for a secondary hyperparathyroidism. By glutene-free nutrition the patient was without pain after several months and was able to go without hindrance after one year.     

Adult celiac disease and hepatopathy

A retrospective study was performed in order to evaluate the prevalence of significant hepatic disease in patients with coeliac disease and its outcome after a gluten-free diet. Out of 46 patients with coeliac disease, 28 (60.87%) presented abnormal liver function test. These tests were normal after a gluten-free diet in 20 patients; one patient presented an acute B-virus hepatitis and in the remaining 7 patients a chronic liver disease was diagnosed (2 chronic persistent hepatitis, 1 chronic active hepatitis, 1 steatosis, 2 cirrhosis and 1 primary biliary cirrhosis). In conclusion, the high prevalence of significant hepatic disease in our patients with coeliac disease (15%) suggests an association between both disorders. Gluten-free diet does not modify the course of hepatic disease.     

Attending rounds: patient with hypokalemia and metabolic acidosis

Hypokalemic paralysis represents a medical emergency requiring both rapid diagnosis and treatment. In this Attending Rounds a patient with hypokalemia and metabolic acidosis is presented to emphasize the role of routine laboratory studies in the assessment of such patients so that a correct diagnosis can be made and appropriate treatment can be initiated promptly.     

Adult celiac disease and type 1 diabetes: a severe and sometimes unknown genetic association

In 6 type 1 (insulin-dependent) diabetics, treated with insulin since the age of 2 to 35 years (mean 12), coeliac disease was diagnosed between the 10 th and 73 th years of age (mean 26). Five were of North African origin. Digestive symptoms and severe malnutrition were present in all of them, associated, in the two younger, with a major growth retardation and, in one, multiple pathologic fractures. Biopsy of the small intestine demonstrated, in all, total or subtotal villous atrophy. The metabolic control of diabetes was poor, with frequent hypoglycaemic attacks, induced by minute insulin doses. Severe chronic complications of diabetes were detectable in all of them. Plasma anti-reticulin antibodies were present, at high titer before starting the gluten-free diet, declining slowly after starting this diet, and negative in the patients who followed this diet. Among the genetic markers (which were determined in 4), HLA A1 was present in 4, B8 and DR3 in 3 and DR4 in 3. The DR7 was not detected. The gluten-free diet, memorized by the patients by the use of simple rules, improved the digestive symptoms, and insulin doses could then be increased. The overall prognosis remained poor, due to diabetic complications and sociologic desinsertion. Coeliac disease occurs in 1 to 2% of type 1 diabetics and 4-6% of the coeliac patients are diabetics. Diabetic subjects from North Africa are at high risk of this association. Misdiagnosis of the coeliac disease compromises the metabolic control and nutritional state.(ABSTRACT TRUNCATED AT 250 WORDS)     

Adult celiac disease and hypertransaminasemia.

OBJECTIVE: to determine the incidence of hypertransaminasemia in adult patients with celiac disease with or without relevant chronic liver disease, and to evaluate the response after a gluten-free diet. PATIENTS AND METHODS: retrospective study of 20 cases of adult celiac disease (> 14 years old at diagnosis). Patients were included in the study if they fulfilled the revised EPSGAN criteria. If laboratory tests of liver function revealed alterations, hepatitis B and C viral serology, thyroid hormones, and use of alcohol and drugs were investigated, and liver ultrasound scans were done. Liver biopsy and endoscopic retrograde cholangiopancreatography were done only in patients for whom these studies were considered necessary. RESULTS: ten patients had hypertransaminasemia (50%), ascribed to benzodiazepine use in 1 patient, chronic HCV hepatitis in 1, and celiac disease in 8. In all of these last patients except 1 (benzodiazepine use), laboratory values returned to normal after 4-10 months on a gluten-free diet. CONCLUSIONS: celiac disease was frequently associated with hypertransaminasemia. In most patients transaminase levels returned to normal within 1 year after dietary gluten intake was restricted. If alterations in laboratory values persist, other causes that may be related (e.g., autoimmunity or tumors) or unrelated to celiac disease (e.g., virus) must be ruled out.     

Adult celiac disease and recurrent pericarditis

Three patients presented with acute pericarditis. No cause was found and, in each case, the pericarditis was recurrent. Investigation revealed evidence of malabsorption due to adult celiac disease. Two of the patients responded to a gluten-free diet and cortiscosteroid therapy. The third patient responded to a gluten-free diet alone and remains well. Celiac disease is a multisystem disorder in which extraintestinal involvement is common. Recurrent pericarditis may be such a manifestation.     

Adult celiac disease is frequently associated with sacroiliitis

No data are available on the presence and frequency of peripheral or central joint disease, routinely determined by bone scintigraphy with 740 MBq of [^99mTc]MDP, in adult celiac disease. Bone scintigraphy was carried out to detect early acute inflammatory lesions in 22 adult celiac patients (15 females and seven males; mean age 36.72 years, range 17–63). Bone scintigraphy was positive for sacroiliitis in 14 cases (63.6%). Except in the case of one patient suffering from rheumatoid arthritis, laboratory data were normal. Our data suggest that as in other chronic intestinal diseases, celiac disease in adults, is frequently associated with central joint disease. This high incidence of sacroiliitis, the joint disease most frequently found in our patients, has not been previously reported in other series. We believe, therefore, this difference could be explained by the different methodology used for the screening of joint difference could be explained by the different methodology used for the screening of joint disease.     

Adult celiac disease and the henoch-schonlein syndrome

Summary The patient in the case presented experienced two different disease entities, each involving the small intestine at different times during a period of 20 years. The original episode was diagnosed as Henoch-Schonlein syndrome based upon classic history, and physical and laboratory findings. Although the original intestinal abnormalities returned to normal radiologically within 3 months, an abnormal small bowel study was again demonstrated some 10 years later. A diagnosis of adult celiac disease was made at that time based on biopsy and a favorable clinical and histologic response to gluten restriction.Chronic changes in the small bowel following an episode of Henoch-Schonlein syndrome have not been reported in the literature. The association of these two disease entities in the same patient has also not been previously reported. Although a hereditary metabolic defect has been suggested as responsible for the development of gluten sensitivity, the question is raised as to whether other factors may initiate this process.     

Adult celiac disease in the elderly

There is an increased awareness that celiac disease may occur in the elderly although presentations with either diarrhea, weight loss or both may be less common causing delays in diagnosis for prolonged periods. Higher detection rates also seem evident owing to active case screening, largely through serodiagnostic measures. In some elderly patients who are genetically predisposed, it has been hypothesized that celiac disease might be precipitated late in life by an antigen, possibly from an infectious agent. As a result, peptide mimicry or other poorly-defined mechanisms may precipitate an autoimmune gluten-dependent clinical state. Although diarrhea and weight loss occur, only isolated iron deficiency anemia may be present at the time of initial diagnosis. In addition, the risk of other autoimmune disorders, particularly autoimmune thyroiditis, and bone disease, are increased. Osteopenia may also be associated with an increased risk of fractures. Finally, elderly celiacs have an increased risk of malignant intestinal disease, especially lymphoma.     

Prevalence of celiac disease in patients with chronic diarrhea]

The objective of this research was to study the frequency of celiac disease in patients with chronic diarrhea. The biopsy materials of the small intestine and levels of antibodies to alpha-gliadin of class A immunoglobulins (IgA) and tissue transglutaminase were studied in 206 patients with chronic diarrhea. Morphologic celiac-specific symptoms were discovered in 35 (16.9%) patients. Symptoms of the total atrophy were discovered in 28 patients (13.5%); those of subtotal one were found in 7 (3.4%) patients. The increase of antibody levels to IgA alpha-gliadin and tissue transglutaminase was discovered in all 35 patients. Their average level made up 123.7 21.2 units per milliliter and 48.7 11.3 units per milliliter, respectively. It was possible to observe the typical celiac form only in 4 (11.4%) patients; the latent form was found in 30 (85.7%) patients, and the torpid (refractory) form was discovered in 1 (2.8%) patient. The frequency of celiac disease in patients with chronic diarrhea is equal to 16.9%. Patients with the latent form of the disease prevail among patients with celiac disease. Immunological screenings with the subsequent morphologic study of the mucous coat of the small intestine should be prescribed to all patients with the chronic diarrhea syndrome to enable the early diagnostics of celiac disease.     

Celiac crisis in an adult type 1 diabetes mellitus patient presented with diarrhea,weight loss and hypoglycemic attacks

Type 1 diabetes mellitus (T1DM) is an autoimmune disease, characterized by loss of the insulin-producing β cells of the islets of Langerhans in the pancreas, causing insulin deficiency. Celiac disease has been seen in 3 to 8 % of T1DM patients. Celiac crisis, an acute severe onset of celiac disease, is a rare and life-threatening manifestation. We report a 50-year-old man with type 1 diabetes mellitus who arrived at our service with a 2-month history of watery diarrhea associated with hypoglycemic attacks, abdominal pain, and weight loss of 13 kg. The diagnosis of celiac crisis was made based on diarrhea leading to dehydration, severe metabolic and electrolyte abnormalities, and subsequent improvement after introduction of a gluten-free diet.