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{{Celiac disease}}
{{Celiac disease}}
{{CMG}}; {{AE}}  
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==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
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]].
 
OR
 
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
 
OR
 
The majority of cases of [disease name] are self-limited and require only supportive care.
 
OR
 
[Disease name] is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR
 
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].


==Medical Therapy==
==Medical Therapy==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
===Refractory disease===
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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>
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
* 1. '''Steroids'''
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
::* Preferred regimen(1): [[Prednisone]] 0.5–1 mg/kg q24h
===Disease Name===
::* Preferred regimen(2): [[Budesonide]] 9 mg q24h
 
::* Preferred regimen(3): [[Prednisone]] 0.5–1 mg/kg q24h and [[azathioprine]] 2 mg/kg q24h combination
* '''1 Stage 1 - Name of stage'''
* 2. '''Immunosuppressive drugs''' (Used in steroid dependent or steroid refractory disease)
** 1.1 '''Specific Organ system involved 1'''
** 2.1 '''Antiproliferative agents'''
*** 1.1.1 '''Adult'''
*** Preferred regimen(1): [[Azathioprine]] 2 mg/kg q24h
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
** 2.2 '''Calcineurin Inhibitors:'''  
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
*** Preferred regimen(1): [[Cyclosporine]] 5 mg/kg q24h PO
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
** 2.3 '''Monoclonal antibodies'''
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
*** Preferred regimen(1): [[Infliximab]] 5 mg/kg q24h
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*** Preferred regimen(2): [[Alemtuzumab]] 30 mg twice a week per 12 weeks
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*** 1.1.2 '''Pediatric'''
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
****1.1.2.2 (Specific population e.g. ''''''children < 8 years of age'''''')
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
** 2.1 '''Specific Organ system involved 2'''
*** 2.1.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 2.1.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
 
* 2 '''Stage 2 - Name of stage'''
** 2.1 '''Specific Organ system involved 1 '''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.1.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.1.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) ''''''(Contraindications/specific instructions)''''''
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''  
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
 
 
 
* Nutrition referral--strict gluten-free diet
* Vitamin/mineral supplements until deficiencies resolve with gluten-free diet
 
===Diet===
{{main|Gluten-free diet}}
 
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.
 
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.


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>
===Dermatitis herpetiformis===
 
*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>
===Refractory disease===
**1.1 '''Gluten-free diet (GFD)'''
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.
*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>
**2.1 '''Sulfones'''
*** Preferred treatment(1): [[Dapsone]] 50 mg q24h increased every week until clearance or tolerance
**2.2 '''Suhphonamides'''
*** Alternative treatment (1):  [[Sulfasalazine]] 500 mg q8h (maximum, 2g q8h)
**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]]
**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>
*** Alternative treatment (2): [[Cholestyramine]]
*** 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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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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