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 therapies for polymyositis and dermatomyositis include corticosteroids, disease modifying antirheumatic drugs (DMARDs),  
*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>
*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 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.  


===Polymyositis and dermatomyositis===
===Polymyositis and dermatomyositis===


* '''1 Stage 1 - Initial treatment'''
* '''1 Initial disease'''
** 1.1 '''Corticosteroids'''
** 1.1 '''[[Corticosteroid|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.     
*** 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
*** 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. 
'''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]]. 
:* 1.2 '''Disease modifying antirheumatic drugs (DMARDs) '''
:* 1.2 '''[[Disease-modifying antirheumatic drug|Disease modifying antirheumatic drugs]] ([[Disease-modifying antirheumatic drug|DMARDs]]) '''
::* Preferred regimen (1): 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.) 
::* 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): 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]].) 
::* Preferred regimen (1): Methotrexate  
::* Alternative regimen (1): [[Hydroxychloroquine]] 200-400 mg PO qd (for controlling skin disease)
::* Preferred regimen (1): Hydroxychloroquine 200-400 mg PO qd (for controlling skin disease)  
* 2 '''Recurrent disease'''
::* Alternative regimen (1): Intravenous immunoglobulin (IVIg)  
** 2.1 Patients with a flare of polymyositis and dermatomyositis requires following actions:
::* Alternative regimen (1): Cyclosporine  
*** Adding [[methotrexate]] or [[azathioprine]] if they are not receiving any of them
::* Alternative regimen (1): Tacrolimus   
*** Increasing the dose of [[prednisone]] to 1 mg/kg/day
::* Alternative regimen (1): Mycophenolate mofetil
*** Increasing the dose of [[azathioprine]] or [[methotrexate]] to the maximum
::* Alternative regimen (1): Rituximab 
* 3 '''Recurrent disease'''
::*   
** 3.1 '''Disease modifying antirheumatic drugs (DMARDs) '''
* 2 '''Stage 2 - Resistant'''
*** Preferred regimen (1): [[Rituximab]] 1 gr IV every 2 weeks for 2 doses
** 2.1
*** Alternative regimen (1): [[Intravenous immunoglobulin]] ([[IVIG|IVIg]]) 2 g/kg every month for 3 months
*** Alternative regimen (1): [[Mycophenolate sodium|Mycophenolate mofetil]] 1-1.5 gr PO bid


==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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