Update on the Diagnosis and Management of Refractory Coeliac Disease
نویسندگان
چکیده
A small subset of coeliac disease (CD) patients experiences persisting or recurring symptoms despite strict adherence to a gluten-free diet (GFD). When other causes of villous atrophy have been excluded, these patients are referred to as refractory celiac disease (RCD) patients. RCD can be divided in two types based on the absence (type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype. RCDI usually runs a benign course and may be difficult to be differentiated from uncomplicated, slow responding CD. In contrast, RCDII can be defined as low-grade intraepithelial lymphoma and frequently transforms into an aggressive enteropathy associated T-cell lymphoma with dismal prognosis. This paper describes the clinical characteristics of RCDI and RCDII, diagnostic approach, and the latest insights in treatment options.
منابع مشابه
Update on the management of refractory coeliac disease.
True Refractory Coeliac Disease (RCD) is being currently defined as persisting or recurring villous atrophy with crypt hyperplasia and increased intraepithelial lymphocytes in spite of a strict gluten free diet for more than 12 months or when severe persisting symptoms necessitate intervention independent of the duration of the dietary therapy. Currently two categories of RCD are being recogniz...
متن کاملPrecocious Diagnosis of Traumatic Splenic Rupture by Selective Coeliac Angiography
Splenic arteriography allows the organ to be clearly demonstrated and the condition of the parenchyrna to be assessed. The methode allows traumatic lesions to be easily diagnosed. The most common reliable signs are escape of contrast medium from the torn spenic arteries and lacunar appearance in the parenchyma. Other signs, such as early venous filling or antero-posterior and median displacemen...
متن کاملTreatment failure in coeliac disease: a practical guide to investigation and treatment of non-responsive and refractory coeliac disease.
Coeliac disease is a common condition affecting up to 1% of the European adult population. Whilst the majority of patients will respond to a gluten free diet with resolution of symptoms and an improvement in histology, a significant minority have persistent problems. Refractory coeliac disease is a relatively uncommon cause of non-response to gluten free diet with potentially serious consequenc...
متن کاملA young girl with H syndrome and coeliac disease
H syndrome is an autosomal recessive genodermatosis with reports dating back to the last decade. This syndrome is caused by mutations in the SCL29A3 gene. The clinical characteristics of this syndrome consist of dermatological manifestations, including hyperpigmented, hypertrichotic, and indurated patches and plaques. It affects various systems by causing heart anomalies, hepatosplenomegaly, hy...
متن کاملManagement of urticaria: update of Iranian Society of Dermatology clinical practice guideline
Urticaria is a common and challenging skin disorder. Diagnosis and treatment of urticaria is not limited to the field of dermatology. General physicians, allergologists and clinical immunologists are also commonly involved in the management of patients with urticaria. Iranian Society of Dermatology developed a clinical practice guideline concerning diagnosis and treatment of urticaria that was ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 2013 شماره
صفحات -
تاریخ انتشار 2013