Polymyositis and dermatomyositis medical therapy: Difference between revisions

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==Overview==
==Overview==
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, [[glucocorticoids]] could be discontinued at 9 to 12 months. [[Disease-modifying antirheumatic drug|DMARDs]] might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of [[prednisone]], adding or increasing dose of [[methotrexate]] or [[azathioprine]]. [[Rituximab]], IVIg, and [[Mycophenolate sodium|mycophenolate mofetil]] might be used in resistant diseases.
 
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].
*Pharmacologic medical therapies for polymyositis and dermatomyositis include [[Corticosteroid|corticosteroids]] and [[Disease-modifying antirheumatic drug|disease-modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]).<ref name="KhanChristopher-Stine2011">{{cite journal|last1=Khan|first1=Sabiha|last2=Christopher-Stine|first2=Lisa|title=Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features|journal=Rheumatic Disease Clinics of North America|volume=37|issue=2|year=2011|pages=143–158|issn=0889857X|doi=10.1016/j.rdc.2011.01.001}}</ref><ref name="DoblougGaren2015">{{cite journal|last1=Dobloug|first1=Cecilie|last2=Garen|first2=Torhild|last3=Bitter|first3=Helle|last4=Stjärne|first4=Johan|last5=Stenseth|first5=Guri|last6=Grøvle|first6=Lars|last7=Sem|first7=Marthe|last8=Gran|first8=Jan Tore|last9=Molberg|first9=Øyvind|title=Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort|journal=Annals of the Rheumatic Diseases|volume=74|issue=8|year=2015|pages=1551–1556|issn=0003-4967|doi=10.1136/annrheumdis-2013-205127}}</ref><ref name="ChinoyFertig2007">{{cite journal|last1=Chinoy|first1=H.|last2=Fertig|first2=N.|last3=Oddis|first3=C. V|last4=Ollier|first4=W. E R|last5=Cooper|first5=R. G|title=The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis|journal=Annals of the Rheumatic Diseases|volume=66|issue=10|year=2007|pages=1345–1349|issn=0003-4967|doi=10.1136/ard.2006.068502}}</ref><ref name="DalakasHohlfeld2003">{{cite journal|last1=Dalakas|first1=Marinos C|last2=Hohlfeld|first2=Reinhard|title=Polymyositis and dermatomyositis|journal=The Lancet|volume=362|issue=9388|year=2003|pages=971–982|issn=01406736|doi=10.1016/S0140-6736(03)14368-1}}</ref><ref name="DouglasTazelaar2001">{{cite journal|last1=Douglas|first1=William W.|last2=Tazelaar|first2=Henry D.|last3=Hartman|first3=Thomas E.|last4=Hartman|first4=Robert P.|last5=Decker|first5=Paul A.|last6=Schroeder|first6=Darrell R.|last7=Ryu|first7=Jay H.|title=Polymyositis–Dermatomyositis-associated Interstitial Lung Disease|journal=American Journal of Respiratory and Critical Care Medicine|volume=164|issue=7|year=2001|pages=1182–1185|issn=1073-449X|doi=10.1164/ajrccm.164.7.2103110}}</ref>
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, [[glucocorticoids]] could be discontinued at 9 to 12 months. [[Disease-modifying antirheumatic drug|DMARDs]] might be discontinued at 6 months.
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
 
==== Corticosteroids ====
*  Prednisone
* The side effects of corticosteroids include weight gain, redistribution of body fat, thinning of the skin, osteoporosis, cataracts, and muscle weakness. 
 
==== Disease Modifying Antirheumatic Drugs (DMARDs) ====
*  Methotrexate
* Azathioprine
* Intravenous immunoglobulin (IVIg)
* Cyclosporine (Neoral,Sandimmune)
* Tacrolimus (Prograf)
* Mycophenolate mofetil (CellCept)
* Rituximab (Rituxan)
 
==== Lifestyle modification ====
* Lifestyle modification is important in the treatment of polymyositis and dermatomyositis. Some of the important precautions are as follows:
**  Physical therapy and exercise
** Healthy and well-balanced diet
** Watching weight
** Sun exposure protection including
*** Limiting time outdoors
*** Using sunscreen
 
===Disease Name===


* '''1 Stage 1 - Name of stage'''
===Polymyositis and dermatomyositis===
** 1.1 '''Specific Organ system involved 1'''
*** 1.1.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 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
*** 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. '<nowiki/>'''''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)
** 1.2 '''Specific Organ system involved 2'''
*** 1.2.1 '''Adult'''
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
*** 1.2.2  '''Pediatric'''
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)


* 2 '''Stage 2 - Name of stage'''
* '''1 Initial disease'''
** 2.1 '''Specific Organ system involved 1 '''
** 1.1 '''[[Corticosteroid|Corticosteroids]]'''
**: '''Note (1):'''
*** Preferred regimen (1): [[Prednisone]] 1 mg/kg PO qd (maximum 80 mg daily) for 4-6 weeks and then [[prednisone]] should be tapered over 26 weeks to reach 5 mg/d. [[Prednisone]] should be tapered by 10 mg every week until 40 mg/day then tapered by 5 mg every week until 20 mg/d then tapered by 2.5 mg every week until 10 mg/day then tapered by 1 mg every two weeks until the patient reaches 5 mg/day.   
**: '''Note (2)''':
*** Alternative regimen (1): [[Methylprednisolone]] 1000 mg IV qd for 3 days
**: '''Note (3):'''  
'''Note (1)''': The side effects of [[Corticosteroid|corticosteroids]] include [[weight gain]], redistribution of [[Adipose tissue|body fat]], thinning of the [[skin]], [[osteoporosis]], [[Cataract|cataracts]], and [[muscle weakness]]. 
*** 2.1.1 '''Adult'''
:* 1.2 '''[[Disease-modifying antirheumatic drug|Disease modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]) '''
**** Parenteral regimen
::* Preferred regimen (1): [[Azathioprine]] 50 mg PO qd for 2 weeks and then increase the daily dose by 50 mg each week to 1.5 mg/kg/day (maximum 2.5 mg/kg/day)  
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
::* Preferred regimen (2): [[Methotrexate]] 15 mg PO every week then increase the daily dose by 2.5 mg increments to 25 mg/week (side effects include [[hepatotoxicity]] and [[pulmonary toxicity]].) 
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
::* Alternative regimen (1): [[Hydroxychloroquine]] 200-400 mg PO qd (for controlling skin disease)
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
* 2 '''Recurrent disease'''  
**** Oral regimen
** 2.1 Patients with a flare of polymyositis and dermatomyositis requires following actions:
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
*** Adding [[methotrexate]] or [[azathioprine]] if they are not receiving any of them
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
*** Increasing the dose of [[prednisone]] to 1 mg/kg/day  
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
*** Increasing the dose of [[azathioprine]] or [[methotrexate]] to the maximum
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
* 3 '''Recurrent disease'''
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
** 3.1 '''Disease modifying antirheumatic drugs (DMARDs) '''
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** Preferred regimen (1): [[Rituximab]] 1 gr IV every 2 weeks for 2 doses
*** 2.1.2 '''Pediatric'''
*** Alternative regimen (1): [[Intravenous immunoglobulin]] ([[IVIG|IVIg]]) 2 g/kg every month for 3 months
**** Parenteral regimen
*** Alternative regimen (1): [[Mycophenolate sodium|Mycophenolate mofetil]] 1-1.5 gr PO bid
***** 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) '<nowiki/>'''''(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)


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 14:55, 18 April 2018

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

Overview

Pharmacologic medical therapies for polymyositis and dermatomyositis include corticosteroids and disease-modifying antirheumatic drugs (DMARDs). Patients with polymyositis and dermatomyositis might require long-term treatment. However, if there are no disease flares during the course of the taper, glucocorticoids could be discontinued at 9 to 12 months. DMARDs might be discontinued at 6 months. Patients with recurrent flare of polymyositis and dermatomyositis require higher dose of prednisone, adding or increasing dose of methotrexate or azathioprine. Rituximab, IVIg, and mycophenolate mofetil might be used in resistant diseases.

Medical Therapy

Polymyositis and dermatomyositis

  • 1 Initial disease
    • 1.1 Corticosteroids
      • Preferred regimen (1): Prednisone 1 mg/kg PO qd (maximum 80 mg daily) for 4-6 weeks and then prednisone should be tapered over 26 weeks to reach 5 mg/d. Prednisone should be tapered by 10 mg every week until 40 mg/day then tapered by 5 mg every week until 20 mg/d then tapered by 2.5 mg every week until 10 mg/day then tapered by 1 mg every two weeks until the patient reaches 5 mg/day.
      • Alternative regimen (1): Methylprednisolone 1000 mg IV qd for 3 days

Note (1): The side effects of corticosteroids include weight gain, redistribution of body fat, thinning of the skin, osteoporosis, cataracts, and muscle weakness. 

  • Preferred regimen (1): Azathioprine 50 mg PO qd for 2 weeks and then increase the daily dose by 50 mg each week to 1.5 mg/kg/day (maximum 2.5 mg/kg/day)
  • Preferred regimen (2): Methotrexate 15 mg PO every week then increase the daily dose by 2.5 mg increments to 25 mg/week (side effects include hepatotoxicity and pulmonary toxicity.) 
  • Alternative regimen (1): Hydroxychloroquine 200-400 mg PO qd (for controlling skin disease)
  • 2 Recurrent disease
  • 3 Recurrent disease

References

  1. ↑ Khan, Sabiha; Christopher-Stine, Lisa (2011). "Polymyositis, Dermatomyositis, and Autoimmune Necrotizing Myopathy: Clinical Features". Rheumatic Disease Clinics of North America. 37 (2): 143–158. doi:10.1016/j.rdc.2011.01.001. ISSN 0889-857X.
  2. ↑ Dobloug, Cecilie; Garen, Torhild; Bitter, Helle; Stjärne, Johan; Stenseth, Guri; Grøvle, Lars; Sem, Marthe; Gran, Jan Tore; Molberg, Øyvind (2015). "Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort". Annals of the Rheumatic Diseases. 74 (8): 1551–1556. doi:10.1136/annrheumdis-2013-205127. ISSN 0003-4967.
  3. ↑ Chinoy, H.; Fertig, N.; Oddis, C. V; Ollier, W. E R; Cooper, R. G (2007). "The diagnostic utility of myositis autoantibody testing for predicting the risk of cancer-associated myositis". Annals of the Rheumatic Diseases. 66 (10): 1345–1349. doi:10.1136/ard.2006.068502. ISSN 0003-4967.
  4. ↑ Dalakas, Marinos C; Hohlfeld, Reinhard (2003). "Polymyositis and dermatomyositis". The Lancet. 362 (9388): 971–982. doi:10.1016/S0140-6736(03)14368-1. ISSN 0140-6736.
  5. ↑ Douglas, William W.; Tazelaar, Henry D.; Hartman, Thomas E.; Hartman, Robert P.; Decker, Paul A.; Schroeder, Darrell R.; Ryu, Jay H. (2001). "Polymyositis–Dermatomyositis-associated Interstitial Lung Disease". American Journal of Respiratory and Critical Care Medicine. 164 (7): 1182–1185. doi:10.1164/ajrccm.164.7.2103110. ISSN 1073-449X.
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