You are here: Home / Rare Disease Information / Rare Disease Database

Search Rare Diseases

Enter a disease name or synonym to search NORD's database of reports.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

Refractory Sprue

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to CiarĂ¡n P. Kelly, MD, Professor of Medicine at Harvard Medical School and Director of the Celiac Center at Beth Israel Deaconess Medical Center in Boston, for assistance in the preparation of this report.

Synonyms of Refractory Sprue

  • intractable celiac sprue
  • refractory celiac disease

Disorder Subdivisions

  • type II refractory sprue
  • type I refractory sprue

General Discussion

Refractory sprue (RS) is a complex autoimmune disorder much like the more common celiac sprue but, unlike celiac sprue, it is resistant or unresponsive to six months of treatment with a strict gluten-free diet. Gliadin, a component of the wheat storage protein gluten, together with similar proteins in barley and rye, are the villains that trigger the immune reaction in celiac sprue. The diagnosis of RS is made by exclusion, especially of any other disorder that can affect the huge number of thread-like projections that line the interior of the intestine (intestinal villi), such as intestinal lymphoma, Crohn's disease, small intestinal bacterial overgrowth or hypogammaglobulinemia.

The intestinal villi are the means by which the gut absorbs fluids and nutrients. In celiac sprue and refractory sprue, these villi shrink and shrivel (atrophy) affecting the absorption of nutrients via the intestines. In celiac sprue, treatment by means of a strict gluten-free diet is usually sufficient to overcome the disorder. However, refractory sprue is just that: refractory or stubbornly resistant to treatment. Only a small percentage of the people with celiac sprue will develop RS, and these patients are almost invariably 30 years of age or older. However, as yet, it is impossible to predict which patient of those with celiac sprue will develop RS.

Refractory Sprue Resources

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.

 
Copyright ©2014 NORD - National Organization for Rare Disorders, Inc. All rights reserved.
The following trademarks/registered service marks are owned by NORD: NORD, National Organization for Rare Disorders, the NORD logo, RareConnect. .