IgA anti-actin antibodies in celiac disease.

AIMS: The purpose of this study was to determine the sensitivity and specificity of IgA anti-actin antibodies (IgA-AAA) for celiac disease (CD), to investigate their usefulness as a marker of compliance in CD patients to the gluten-free diet (GFD), and to assess the relationship between their presence in the sera of CD patients and severity of intestinal mucosal damage. PATIENTS AND METHODS: A total of 182 patients with CD were studied: 63 patients were untreated; 50 patients were following a strict GFD; and 69 patients were non-compliant with a GFD. IgA-AAA was detected using a homemade enzyme-linked immunosorbent assay (ELISA). RESULTS: IgA-AAA showed a sensitivity of 41.3% and a specificity of 71.4% for a diagnosis of CD. In children, the frequency of IgA-AAA detection was lower in those following a strict GFD (23.1%) compared with untreated patients (39.4%) and those not complying with a GFD (32.5%). In patients following a strict GFD, IgA-AAA detection was significantly less frequent in children than in adults (23.1% vs. 58.3%, respectively; P<0.001). IgA-AAA was found in 17 out of 52 CD patients with total villous atrophy (32.7%), and in one out of 11 patients with subtotal villous atrophy (9%). CONCLUSION: IgA-AAA cannot replace anti-endomysium and anti-tissue transglutaminase antibodies in the diagnosis algorithm of CD, but it can serve as a reliable marker of severe intestinal mucosal damage in CD patients.

[Chronic intestinal pseudo-obstruction in systemic lupus erythematosus]. / Pseudo-obstruction intestinale au cours du lupus érythémateux systémique.

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.

[Primary digestive tract lymphoma in central region of Tunisia: anatomoclinical study and therapeutic results about 153 cases]. / Les lymphomes primitifs du tube digestif (LPTD) dans le centre tunisien: étude anatomoclinique et résultats thérapeutiques à propos de 153 cas.

Primary gastro-intestinal lymphoma (PGIL) is the most common type of extra-nodal non Hodgkin's lymphoma. Their clinical and histological presentations are heterogeneous depending on the site of the lesion. There is no consensus regarding the role of surgery and chemotherapy in the therapeutic approach. In our country epidemiology of the disease is unknown with IPSID being the most frequent type. We report anatomo-clinical features and prognostic factors of PGIL and compare intestinal to gastric forms in our region. This is a retrospective study of 153 cases of PGIL in adults diagnosed and treated in the department of medical oncology in Farhat Hached Hospital between 1994 and 2006. The median age was 52 years and the sex-ratio 2.1. Tumor sites were gastric (67%), intestinal (26%) and gastrointestinal (7%). Abdominal pain (87%) followed by vomiting and diarrhoea (37 and 15%) were the most common symptoms. Performance status (PS) < 2 was seen in 80% of patients, high grade lymphoma in 70.5% of cases and B phenotype was noted in 85%. MALT lymphoma accounts for 50% of cases, and IPSID for only 5% of PGIL. About 47.5% of cases were stage IE, 138 patients had chemotherapy with an objective response rate of 77%. Only 46% of patients had surgery (14 for surgical complication, 6 for residual tumor after chemotherapy and 22 to have histological diagnosis). The five-year overall survival (OS) was 62%. In high grade lymphoma patients favorable prognostic factors for OS included young age < or = 60 years, PS < 2, normal serum LDH, hemoglobin > 12 g/dL, B phenotype, localised stage (IE-IIE1), anthracycline-based chemotherapy regimen, achieving complete or partial response to induction chemotherapy and no relapse. In multivariate study only relapse and PS were significant prognostic factors for OS. In low-grade lymphoma patients, none of these factors had a significant correlation with OS: age < or = 60 years, PS < 2, stage (IE-IIE1), response to induction chemotherapy, relapse. Compared to gastric lymphomas, intestinal cases occurred at a younger age, frequently with diarrhoea, weight loss, and occlusion. They are more often high-grade, T phenotype and have locally advanced stage (IIE); surgery is more common in this group. We conclude that stomach is the main site of PGIL in our region, intestinal lymphoma is less frequent and IPSID has become rare. Recent progress in chemotherapy has allowed good therapeutic results with a conservative approach. Surgery may be performed in case of emergency or for residual lesions after medical treatment.

Primary antiphospholipid syndrome and Evan's syndrome: 2 case reports.

The main clinical features of primary antiphospholipid syndrome are recurrent foetal loss, arterial or venous thrombosis and thrombocytopaenia. Evan's syndrome is characterized by simultaneous or sequential association of autoimmune anaemia and thombocytopaenia, rarely reported in primary antiphospholipid syndrome. We describe 2 cases of this association. Treatment with steroids was started in both cases, the follow-up was marked by the normalization of haemoglobin value and platelet count, without relapse.