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{{Celiac disease}}
{{Celiac disease}}
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==Overview==
==Overview==
A minority of patients suffer from refractory disease, which means that they do not improve with a [[gluten-free diet]]. Pharmacotherapy is used if dietary modification is not beneficial. Pharmacotherapy include [[Steroid|steroids]], [[azathioprine]], [[Cyclosporine|cyclosporine]], and [[monoclonal antibodies]].
==Medical Therapy==
==Medical Therapy==
===Refractory disease===
A minority of patients suffer from refractory disease, which means they do not improve on a [[gluten-free diet]]. This may be because the disease has been present for so long that the intestines are no longer able to heal on diet alone or the patient is not adhering to the diet, or the patient is consuming foods that contain [[gluten]]. Pharmacotherapy is used if dietary modification is not effective.<ref name="pmid20332526">{{cite journal |vauthors=Rubio-Tapia A, Murray JA |title=Classification and management of refractory coeliac disease |journal=Gut |volume=59 |issue=4 |pages=547–57 |year=2010 |pmid=20332526 |pmc=2861306 |doi=10.1136/gut.2009.195131 |url=}}</ref>
* 1. '''Steroids'''
::* Preferred regimen(1): [[Prednisone]] 0.5–1 mg/kg q24h
::* Preferred regimen(2): [[Budesonide]] 9 mg q24h
::* Preferred regimen(3): [[Prednisone]] 0.5–1 mg/kg q24h and [[azathioprine]] 2 mg/kg q24h combination
* 2. '''Immunosuppressive drugs''' (Used in steroid dependent or steroid refractory disease)
** 2.1 '''Antiproliferative agents'''
*** Preferred regimen(1): [[Azathioprine]] 2 mg/kg q24h
** 2.2 '''Calcineurin Inhibitors:'''
*** Preferred regimen(1): [[Cyclosporine]] 5 mg/kg q24h PO
** 2.3 '''Monoclonal antibodies'''
*** Preferred regimen(1): [[Infliximab]] 5 mg/kg q24h
*** Preferred regimen(2): [[Alemtuzumab]] 30 mg twice a week per 12 weeks


* Nutrition referral--strict gluten-free diet
===Dermatitis herpetiformis===
* Vitamin/mineral supplements until deficiencies resolve with gluten-free diet
*1. '''Life style modification'''<ref name="pmid12477369">{{cite journal |vauthors=Collin P, Reunala T |title=Recognition and management of the cutaneous manifestations of celiac disease: a guide for dermatologists |journal=Am J Clin Dermatol |volume=4 |issue=1 |pages=13–20 |year=2003 |pmid=12477369 |doi= |url=}}</ref>
 
**1.1 '''Gluten-free diet (GFD)'''
===Diet===
*2. '''Pharmocatherapy'''<ref name="pmid18360613">{{cite journal |vauthors=Mutasim DF |title=Therapy of autoimmune bullous diseases |journal=Ther Clin Risk Manag |volume=3 |issue=1 |pages=29–40 |year=2007 |pmid=18360613 |pmc=1936286 |doi= |url=}}</ref><ref name="pmid20729961">{{cite journal |vauthors=Han A |title=A practical approach to treating autoimmune bullous disorders with systemic medications |journal=J Clin Aesthet Dermatol |volume=2 |issue=5 |pages=19–28 |year=2009 |pmid=20729961 |pmc=2924135 |doi= |url=}}</ref>
{{main|Gluten-free diet}}
**2.1 '''Sulfones'''
 
*** Preferred treatment(1): [[Dapsone]] 50 mg q24h increased every week until clearance or tolerance
Presently, the only effective treatment is a life-long [[gluten-free diet]].<ref>{{cite journal | author = Kupper C | title = Dietary guidelines and implementation for celiac disease | journal = Gastroenterology | volume = 128 | issue = 4 Suppl 1 | pages = S121-7 | year = 2005 | id = PMID 15825119}}</ref> No medication exists that will prevent damage, or prevent the body from attacking the gut when gluten is present. Strict adherence to the diet allows the intestines to heal, leading to resolution of all symptoms in the vast majority of cases and, depending on how soon the diet is begun, can also eliminate the heightened risk of osteoporosis and intestinal cancer.<ref>{{cite journal | author = Treem W | title = Emerging concepts in celiac disease | journal = Curr Opin Pediatr | volume = 16 | issue = 5 | pages = 552-9 | year = 2004|id = PMID 15367850}}</ref> [[Dietician]] input is generally requested to ensure the patient is aware which foods contain gluten, which foods are safe, and how to have a balanced diet despite the limitations. In many countries gluten-free products are available on [[Medical prescription|prescription]] and may be reimbursed by [[health insurance]] plans. More manufacturers are producing gluten-free products, some of which are almost indistinguishable from their gluten-containing counterparts.
**2.2 '''Suhphonamides'''
 
*** Alternative treatment (1):  [[Sulfasalazine]] 500 mg q8h (maximum, 2g q8h)
The diet can be cumbersome; while young children can be kept compliant by their parents, teenagers may wish to hide their problem or rebel against the dietary restrictions, risking relapse. Many food products contain traces of gluten even if apparently wheat-free. Gluten-free products are usually more expensive and harder to find than common wheat-containing foods.
**2.3 '''Combination treatment'''<ref name="pmid28133723">{{cite journal |vauthors=Bevans SL, Sami N |title=Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis |journal=Int. J. Dermatol. |volume=56 |issue=5 |pages=e90–e92 |year=2017 |pmid=28133723 |doi=10.1111/ijd.13542 |url=}}</ref>
 
*** Alternative treatment (1): [[Dapsone]] plus [[sulfasalazine]]
Even while on a diet, health-related quality of life (HRQOL) may be decreased in people with coeliac disease. Some have persisting digestive symptoms or [[dermatitis herpetiformis]], mouth ulcers, osteoporosis and fractures. Symptoms suggestive of [[irritable bowel syndrome]] may be present, and there is an increased rate of anxiety, fatigue, [[dyspepsia]] and musculoskeletal pain.<ref>{{cite journal | author = Häuser W, Gold J, Stein J, Caspary W, Stallmach A | title = Health-related quality of life in adult coeliac disease in Germany: results of a national survey | journal = Eur J Gastroenterol Hepatol | volume = 18 | issue = 7 | pages = 747-54 | year = 2006 | id = PMID 16772832}}</ref>
**2.4 '''Other treatment''' (intolerance or allergies to dapsone and [[sulfasalazine]])
 
*** Alternative treatment (1): [[Colchicine]]<ref name="pmid7458365">{{cite journal |vauthors=Silvers DN, Juhlin EA, Berczeller PH, McSorley J |title=Treatment of dermatitis herpetiformis with colchicine |journal=Arch Dermatol |volume=116 |issue=12 |pages=1373–84 |year=1980 |pmid=7458365 |doi= |url=}}</ref>
===Refractory disease===
*** Alternative treatment (2): [[Cholestyramine]]
A tiny minority of patients suffer from refractory disease, which means they do not improve on a gluten-free diet. This may be because the disease has been present for so long that the intestines are no longer able to heal on diet alone, or because the patient is not adhering to the diet, or because the patient is consuming foods that are inadvertently contaminated with gluten. If alternative causes have been eliminated, [[glucocorticoid|steroids]] or [[Immunosuppressive drug|immunosuppressants]] (such as [[azathioprine]]) may be considered in this scenario.
*** Alternative treatment (3): [[Tetracycline]]<ref name="pmid10844495">{{cite journal |vauthors=Shah SA, Ormerod AD |title=Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide |journal=Clin. Exp. Dermatol. |volume=25 |issue=3 |pages=204–5 |year=2000 |pmid=10844495 |doi= |url=}}</ref>
*** Alternative treatment (4): [[Nicotinamide|Niacinamide]]<ref name="pmid10844495">{{cite journal |vauthors=Shah SA, Ormerod AD |title=Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide |journal=Clin. Exp. Dermatol. |volume=25 |issue=3 |pages=204–5 |year=2000 |pmid=10844495 |doi= |url=}}</ref>
*** Alternative treatment (5): [[Heparin]]<ref name="pmid10844495">{{cite journal |vauthors=Shah SA, Ormerod AD |title=Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide |journal=Clin. Exp. Dermatol. |volume=25 |issue=3 |pages=204–5 |year=2000 |pmid=10844495 |doi= |url=}}</ref>
*** Alternative treatment (6): [[Cyclosporine|Cyclosporin]]
**2.5 '''Monoclonal antibodies'''<ref name="pmid28030659">{{cite journal |vauthors=Albers LN, Zone JJ, Stoff BK, Feldman RJ |title=Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis |journal=JAMA Dermatol |volume=153 |issue=3 |pages=315–318 |year=2017 |pmid=28030659 |doi=10.1001/jamadermatol.2016.4676 |url=}}</ref>
*** Preferred treatment(1): [[Rituximab]]  
**: '''Note:''' Used is severe cases not improved by other medications.


==References==
==References==
{{reflist|2}}
{{reflist|2}}
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[[Category:Gastroenterology]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]
[[Category:Autoimmune diseases]]
[[Category:Genetic disorders]]
[[Category:Malnutrition]]
[[Category:Pediatrics]]
[[Category:Dermatology]]
[[Category:Up-To-Date]]

Latest revision as of 20:50, 29 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anmol Pitliya, M.B.B.S. M.D.[2]

Overview

A minority of patients suffer from refractory disease, which means that they do not improve with a gluten-free diet. Pharmacotherapy is used if dietary modification is not beneficial. Pharmacotherapy include steroids, azathioprine, cyclosporine, and monoclonal antibodies.

Medical Therapy

Refractory disease

A minority of patients suffer from refractory disease, which means they do not improve on a gluten-free diet. This may be because the disease has been present for so long that the intestines are no longer able to heal on diet alone or the patient is not adhering to the diet, or the patient is consuming foods that contain gluten. Pharmacotherapy is used if dietary modification is not effective.[1]

  • 1. Steroids
  • 2. Immunosuppressive drugs (Used in steroid dependent or steroid refractory disease)
    • 2.1 Antiproliferative agents
    • 2.2 Calcineurin Inhibitors:
    • 2.3 Monoclonal antibodies
      • Preferred regimen(1): Infliximab 5 mg/kg q24h
      • Preferred regimen(2): Alemtuzumab 30 mg twice a week per 12 weeks

Dermatitis herpetiformis

  • 1. Life style modification[2]
    • 1.1 Gluten-free diet (GFD)
  • 2. Pharmocatherapy[3][4]

References

  1. Rubio-Tapia A, Murray JA (2010). "Classification and management of refractory coeliac disease". Gut. 59 (4): 547–57. doi:10.1136/gut.2009.195131. PMC 2861306. PMID 20332526.
  2. Collin P, Reunala T (2003). "Recognition and management of the cutaneous manifestations of celiac disease: a guide for dermatologists". Am J Clin Dermatol. 4 (1): 13–20. PMID 12477369.
  3. Mutasim DF (2007). "Therapy of autoimmune bullous diseases". Ther Clin Risk Manag. 3 (1): 29–40. PMC 1936286. PMID 18360613.
  4. Han A (2009). "A practical approach to treating autoimmune bullous disorders with systemic medications". J Clin Aesthet Dermatol. 2 (5): 19–28. PMC 2924135. PMID 20729961.
  5. Bevans SL, Sami N (2017). "Dapsone and sulfasalazine combination therapy in dermatitis herpetiformis". Int. J. Dermatol. 56 (5): e90–e92. doi:10.1111/ijd.13542. PMID 28133723.
  6. Silvers DN, Juhlin EA, Berczeller PH, McSorley J (1980). "Treatment of dermatitis herpetiformis with colchicine". Arch Dermatol. 116 (12): 1373–84. PMID 7458365.
  7. 7.0 7.1 7.2 Shah SA, Ormerod AD (2000). "Dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide". Clin. Exp. Dermatol. 25 (3): 204–5. PMID 10844495.
  8. Albers LN, Zone JJ, Stoff BK, Feldman RJ (2017). "Rituximab Treatment for Recalcitrant Dermatitis Herpetiformis". JAMA Dermatol. 153 (3): 315–318. doi:10.1001/jamadermatol.2016.4676. PMID 28030659.

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