Association cirrhose biliaire primitive et maladie de Biermer. �� propos d��une nouvelle observation

Primary biliary cirrhosis is often associated with autoimmune diseases. However, its association with pernicious anemia has rarely been reported. We report a case of a 68-year-old woman who presented jaundice and pruritus. Mildly elevated serum levels of alkaline phosphatase and g-glutamyl transpeptidase were detected. The titer of anti-mitochondrial M2 antibody was elevated. Histology of liver biopsy showed features of primary biliary cirrhosis. She presented also a macrocytic anemia. The diagnosis of pernicious anemia was confirmed by the low level of serum vitamin B12 and the presence of anti-parietal cell antibody and anti-intrinsic factor antibody. The association of primary biliary cirrhosis and pernicious anemia may be explained by an autoimmune mechanism commonly shared by the two diseases.     

Association d’une maladie coeliaque à une achalasie: une association clinique exceptionnelle

Introduction

Digestive motor disorders are commonly associated with coeliac disease, but the association of coeliac disease with achalasia has rarely been described.

Observation

We report the case of a young man aged 20, following a gluten-free diet and presenting with coeliac disease and dysphagia, showing radiological, endoscopic and manometric signs of achalasia. Endoscopic treatment by pneumatic dilation, followed by Heller’s myotomy, led to a remission of symptoms.

Conclusion

It is probably an accidental association, but it raises the possibility of a predisposing terrain common to both diseases.     

Le syndrome de cavitation ganglionnaire mésentérique,complication rare de la maladie cœliaque de l’adulte : à propos de quatre cas et revue de la littérature

IntroductionMesenteric lymph node cavitation is an exceptional complication of celiac disease. We report four original observations of this syndrome, completed by a literature review.DiscussionThe analysis of 38 cases showed that this complication occurred exclusively in adults, with a mean age at diagnosis of 54 years. It revealed the celiac disease in the majority of cases. Hyposplenism was almost systematically associated. The risk of lymphoma appeared higher, especially enteropathy-associated T-cell lymphoma. The prognosis was poor with nearly 50% mortality and seemed related to the clinical response to the gluten-free diet.ConclusionThe severity of this complication deserves to be known and should lead to its research in celiac patients, especially in cases diagnosed in adulthood or in case of refractory disease.     

Le syndrome de tachycardie posturale (PoTS) : une maladie pour l’interniste

Postural tachycardia syndrome (PoTS) is a multifactorial syndrome defined by an increase in heart rate ≥ 30 bpm, within 10 minutes of standing (or during a head up tilt test to at least 60°), in absence of orthostatic hypotension. It is associated with symptoms of cerebral hypoperfusion that are worse when upright and improve in supine position. Patients have an intense fatigue with a high incidence on quality of life. This syndrome can be explained by many pathophysiological mechanisms. It can be associated with Ehlers-Danlos disease and some autoimmune disorders. The treatment is based on nonpharmacological measures and treatment with propranolol, fludrocortisone or midodrine.     

Étude de l’auto-immunité chez des patients tunisiens adultes atteints de maladie cœliaque : résultats d’une étude prospective comparative

Aim

To determine the frequency of autoantibodies in patients with celiac disease and identify factors associated with the higher prevalence of autoimmunity in these patients.

Materials and methods

We conducted a prospective study involving 50 adult patients with celiac disease and 50 healthy controls. We systematically searched for the presence of the following autoantibodies: antinuclear, anti-smooth muscle, anti-keratin, anti-islet-cell, anti-DNA, rheumatoid factor, thyroid peroxydase, thyroglobulin and glutamate decarboxylase.

Results

At least one autoantibody was detected in 88% of the patients and 54% of controls (p = 0.0001). In patients with celiac disease, we found a higher prevalence of the following antibodies: antinuclear (44 vs 16%; p = 0.002), anti-ENA (22 vs 2%; p = 0.01), rheumatoid factor (42 vs 15%; p = 0.008) and anti-islet-cell (12 vs 0%; p = 0.01). Factors associated with the presence of anti-thyroid antibodies were older age (37.3 ± 5.7 vs 30.3 ± 9.3 years; p = 0.005) and the lack of IgA anti-gliadin antibodies (37 vs 76%; p = 0.04). Factors associated with the presence of islet-cell antibodies were younger age (22.5 ± 5.7 vs 30.3 ± 9.3 years; p = 0.005) and male gender (67 vs 14%; p = 0.01).

Conclusion

Autoimmunity is frequent in celiac disease. Autoantibodies should be useful in screening for several autoimmune diseases, particularly thyroid disorders and diabetes.     

Association hépatite virale C et purpura thrombopénique auto-immun (nouvelle observation)

Extrahepatic manifestations of chronic viral hepatitis C are varied. The immun thrombocytopenic purpura (ITP) represents 10 to 15% [1]. We report a case concerning the association ITP and HCV in a patient of 51 years, followed A3F4 according to Metavir stage. The case presented showed that the platelet count was low prior to the initiation of combination antiviral therapy. After starting combination antiviral therapy (PEG IFN and Ribavirin) the platelet count showed a important drop which justified its stop.The association of autoimmune thrombocytopenia and the C hepatitis virus was selected to the presence of high titers of antibodies against platelet glycoprotein (GP) IIb/IIIa, GP Ia/IIa, and/or GP Ib/IX, and no other mechanism involved in the pathogenesis of HCV-associated thrombocytopenia was identified. Corticosteroid therapy was started but, there was no significant improvement in the platelet count. The predinsone was withdrawn The use of the combination antiviral therapy aggravated the thrombocytopenia then the IFN was stopped, 2 months later, the platelets stabilized under Ribavirin only.The IFN reintrocuced and the viral RNA downgraded after 7 months.The mechanism of the association HCV-ITP is not yet well established, we directly incriminate the hepatitis C virus and also the Process with immunological cellular mediation. To summrize, This association represents a great therapeutic difficulty requiring an multidisciplinary management of hépatologue and a hematologist.