Mixed connective tissue disease medical therapy: Difference between revisions

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==Overview==
==Overview==
 
The treatment of patients with MCTD depends on type of internal organ involvement, phase of the disease, and rate of disease progression. Treatment strategies must follow conventional treatments that are used for similar problems in other [[Rheumatology|rheumatic]] diseases ([[systemic lupus erythematosus|SLE]], [[scleroderma]], [[polymyositis]]). Usually the treatment of patients with MCTD include low doses of [[Steroid|steroids]], [[Non-steroidal anti-inflammatory drug|NSAIDs]], [[Immunosuppressive drug|immunosuppressive drugs]], and [[Biology|biologic]] agents. The treatment options in [[refractory]] cases or in severe clinical conditions include [[Antibody|immunoglobulins]], [[Chemotherapy|cytotoxic agents]], and [[Biology|biologic]] drugs.


==Medical Therapy==
==Medical Therapy==
* In MCTD, the medical treatment is determined based on the kind of the involved organ and intensity of the disease activity. Patients usually react to low doses of steroids and non-steroidal anti-inflammatory drugs, in combination with immunosuppressive drugs or biologic agents (monoclonal antibodies).<ref name="pmid24353496">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref>
* The treatment of patients with MCTD depends on:<ref name="pmid24353496">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref>
.
** Type of Internal organ involvement
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
** Phase of the disease
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
** Rate of disease progression
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
* Treatment strategies must follow conventional treatments of similar problems in other [[Rheumatology|rheumatic]] diseases ([[systemic lupus erythematosus|SLE]], [[scleroderma]], [[polymyositis]]).<ref name="pmid16084325">{{cite journal |vauthors=Kim P, Grossman JM |title=Treatment of mixed connective tissue disease |journal=Rheum. Dis. Clin. North Am. |volume=31 |issue=3 |pages=549–65, viii |date=August 2005 |pmid=16084325 |doi=10.1016/j.rdc.2005.04.008 |url=}}</ref>
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
** For more information about treatment of [[systemic lupus erythematosus]], click '''[[Systemic lupus erythematosus medical therapy|here]]'''.
===Disease Name===
** For more information about treatment of [[scleroderma]], click '''[[Scleroderma medical therapy|here]]'''.
 
** For more information about treatment of [[Polymyositis and dermatomyositis|polymyositis]], click '''[[Polymyositis and dermatomyositis medical therapy|here]]'''.
* '''1 Stage 1 - Name of stage'''
* Usually the treatment of patients with MCTD include:<ref name="pmid24353496">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref>
** 1.1 '''Specific Organ system involved 1'''
** Low doses of [[Steroid|steroids]]
*** 1.1.1 '''Adult'''
** [[Non-steroidal anti-inflammatory drug|Non-steroidal anti-inflammatory drugs]] ([[Non-steroidal anti-inflammatory drug|NSAIDs]])
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
** [[Immunosuppressive drug|Immunosuppressive drugs]]
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
** [[Biology|Biologic]] agents ([[monoclonal antibodies]])
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
* The treatment options in [[refractory]] cases or in severe clinical conditions include:<ref name="pmid243534962">{{cite journal |vauthors=Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E |title=Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl |journal=Postepy Dermatol Alergol |volume=30 |issue=5 |pages=329–36 |date=October 2013 |pmid=24353496 |pmc=3858664 |doi=10.5114/pdia.2013.38365 |url=}}</ref>
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
** [[Antibody|Immunoglobulins]]
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
** [[Chemotherapy|Cytotoxic agents]] (such as [[cyclophosphamide]])
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
** [[Biology|Biologic]] drugs
*** 1.1.2 '''Pediatric'''
* [[Plasmapheresis]] combined with [[rituximab]] (a [[Monoclonal antibodies|monoclonal antibody]] anti-CD20 which can modulate the disease activity) may be a treatment option.
**** 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'''
** 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) '<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==
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[[Category: (name of the system)]]
[[Category:Medicine]]
[[Category:Up-To-Date]]
[[Category:Rheumatology]]

Latest revision as of 14:56, 7 May 2018

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

Overview

The treatment of patients with MCTD depends on type of internal organ involvement, phase of the disease, and rate of disease progression. Treatment strategies must follow conventional treatments that are used for similar problems in other rheumatic diseases (SLE, scleroderma, polymyositis). Usually the treatment of patients with MCTD include low doses of steroids, NSAIDs, immunosuppressive drugs, and biologic agents. The treatment options in refractory cases or in severe clinical conditions include immunoglobulins, cytotoxic agents, and biologic drugs.

Medical Therapy

References

  1. ↑ 1.0 1.1 Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E (October 2013). "Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl". Postepy Dermatol Alergol. 30 (5): 329–36. doi:10.5114/pdia.2013.38365. PMC 3858664. PMID 24353496.
  2. ↑ Kim P, Grossman JM (August 2005). "Treatment of mixed connective tissue disease". Rheum. Dis. Clin. North Am. 31 (3): 549–65, viii. doi:10.1016/j.rdc.2005.04.008. PMID 16084325.
  3. ↑ Latuśkiewicz-Potemska J, Zygmunt A, Biernacka-Zielińska M, Stańczyk J, Smolewska E (October 2013). "Mixed connective tissue disease presenting with progressive scleroderma symptoms in a 10-year-old girl". Postepy Dermatol Alergol. 30 (5): 329–36. doi:10.5114/pdia.2013.38365. PMC 3858664. PMID 24353496.

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