Khaoula Ben Abdelghani

Systemic lupus erythematosus and celiac disease.

Joint Bone Spine - In Press.Proof corrected by the author Available online since vendredi 30 septembre 2011

Chronic Inflammatory Rheumatism Associated With Takayasu Disease

Takayasu disease is rarely associated with other autoimmune diseases. Therefore, the cases discussued herein are uncommon because we are reporting Takayasu disease associated with rheumatoid polyarthritis and spondylarthropathy. The first case concerns a 40-year-old woman presenting with Takayasu disease 11 years after the diagnosis of erosive and seronegative rheumatoid polyarthritis. The upper limb arteries and 1 lower limb artery were affected. The second 41-year-old case presented with ankylosing spondylitis that had been evolving for 10 years. Human leukocyte antigen-B27 typing was negative. Takayasu disease was revealed by severe high blood pressure. In both cases, radiologic examination revealed a typical aspect of the aorta and its main collaterals. Rarely in the literature have these associations been reported, and the pathology remains unknown.

A Case of Type 1 Autoimmune Pancreatitis (AIP), a Form of IgG4-Related Disease (IgG4-RD)

Patient: Male, 70 Final Diagnosis: IgG4 RD Symptoms: Jaundice Medication: — Clinical Procedure: — Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). Case Report: We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion. Pancreatico-biliary magnetic resonance imaging (MRI) showed a thickened rim surrounding the pancreatic duct Serum IgG4 levels were elevated, resulting in a diagnosis of IgG4-related AIP. The patient showed a good clinical, biochemical, and radiological response following steroid therapy in combination with azathioprine. Conclusions: The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, and serological analysis. As this case report has demonstrated, IgG4-RD should be considered in patients who present with pancreatitis or AIP, because of the favorable response to steroid therapy, particularly when treatment is initiated early.

Maladie cœliaque de l’adulte révélée par une polysérite

Celiac disease (CD) is an autoimmune disease affecting multiple organs. It often presents as gastrointestinal manifestations associated with malabsorption. However, serosa involvement uncommonly reveals this enteropathy, making the diagnosis difficult. We here report the case of JA, aged 63 years, admitted to hospital to detect the cause of malabsorption syndrome associated with polyserositis signs including pleurisy, pericarditis, ascites and hydrocephalus. The diagnosis of CD was based on endoscopic signs without serology tests. Patients evolution was partially favorable, due to lack of compliance with a gluten-free diet. Our study reports the first case of CD revealed by polyserositis. CD should be suspected in patients with malabsorption syndrome, in the absence of evocative signs.La maladie cœliaque (MC) représente une maladie auto immune touchant plusieurs organes. Elle est souvent révélée par des manifestations digestives avec une malabsorption biologique. Cependant, l’atteinte des séreuses peut exceptionnellement révéler cette entéropathie rendant le diagnostic difficile. Il s’agit du patient JA âgé de 63 ans admis pour exploration de syndrome de malabsorption avec des signes de polysérite à type de pleurésie, péricardite, ascite et hydrocéphalie. Le diagnostic de MC a été porté devant des signes endoscopiques sans arguments sérologiques. L’évolution était partiellement favorable à cause de la mauvaise adhésion au régime sans gluten. Notre observation est le premier cas rapporté d’une polysérite révélant la MC qui devrait être évoquée devant tout syndrome de malabsorption en l’absence de signes évocateurs.

Chronic graft versus host disease and nephrotic syndrome

Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.