Scleroderma medical therapy

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

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

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

  • Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
  • Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
  • 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].

Scleroderma

  • 1 Treatment of skin manifestations
    • 1.1 Limited cutaneous scleroderma-morphea
      • 1.1.1 Adult
        • Preferred regimen (1): localized phototherapy with ultraviolet light for 15 to 20 treatments[1]
        • Preferred regimen (2): topical tacrolimus 0.1% ointment twice daily[2]
        • Preferred regimen (3): methotrexate 15 mg PO once weekly, maximum dose is 25 mg per week[3]
        • Alternative regimen (1): topical calcipotriene 0.005% ointment twice daily
        • Alternative regimen (2): mycophenolate mofetil 500 mg PO q12h for 7-14 days, then increase to maintenance dose of 500 mg to 1500 mg PO q12h as tolerated[4]
    • 1.2 Diffuse sclerosis of the skin
      • 1.2.1 Adult
        • Preferred regimen (1): methotrexate 15 mg PO once weekly, maximum dose is 25 mg per week[5]
        • Alternative regimen (1): mycophenolate mofetil 500 mg PO q12h for 7-14 days, then increase to maintenance dose of 500 mg to 1500 mg PO q12h as tolerated[4]
        • Alternative regimen (2): cyclophosphamide < or = 2 mg/kg PO daily[6]
    • 1.3 Calcinosis
      • 1.3.1 Adult
        • Preferred regimen (1): minocycline 50-100 mg PO q12h for 6-12 weeks[7]
        • Alternative regimen (1): infliximab[8]
        • Alternative regimen (2): rituximab[9]
    • 1.4 Raynaud's phenomenon
      • 1.4.1 Adult
        • Oral regimen
          • Preferred regimen (1): Nifedipine 30-120 mg (extended release) PO once daily[10]
          • Preferred regimen (2): Amlodipine 5-20 mg PO once daily[11]
          • Alternative regimen (1): Sildenafil 20 mg PO once daily, then increase to a maximum dose of 20 mg PO q8h[12]
          • Alternative regimen (2): losartan 50 mg PO once daily[13]
        • Topical regimen
          • Alternative regimen (3): Topical NTG


  • 2 Treatment of gastrointestinal manifestations
    • 2.1 Gastroesophageal reflux symptoms
      Note (1):
      Note (2):
      Note (3):
      • 2.1.1 Adult
        • 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 Esophageal dysmotility
      Note (1):
      Note (2):
      Note (3):
      • 2.2.1 Adult
        • Oral regimen
          • Preferred regimen (1): metoclopramide
          • Preferred regimen (2): domperidone
          • Alternative regimen (1): erythromycin
          • 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
  • 3 Treatment of pulmonary manifestations
    • 3.1 Pulmonary arterial hypertension
      Note (1):
      Note (2):
      Note (3):
      • 3.1.1 Adult
        • 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
    • 3.2 Interstitial lung disease
      Note (1):
      Note (2):
      Note (3):
      • 3.2.1 Adult
        • Oral regimen
          • Preferred regimen (1): metoclopramide
          • Preferred regimen (2): domperidone
          • Alternative regimen (1): erythromycin
          • 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

References

  1. ↑ Zwischenberger BA, Jacobe HT (November 2011). "A systematic review of morphea treatments and therapeutic algorithm". J. Am. Acad. Dermatol. 65 (5): 925–41. doi:10.1016/j.jaad.2010.09.006. PMID 21645943.
  2. ↑ Stefanaki C, Stefanaki K, Kontochristopoulos G, Antoniou C, Stratigos A, Nicolaidou E, Gregoriou S, Katsambas A (November 2008). "Topical tacrolimus 0.1% ointment in the treatment of localized scleroderma. An open label clinical and histological study". J. Dermatol. 35 (11): 712–8. doi:10.1111/j.1346-8138.2008.00552.x. PMID 19120765.
  3. ↑ Pope JE, Bellamy N, Seibold JR, Baron M, Ellman M, Carette S, Smith CD, Chalmers IM, Hong P, O'Hanlon D, Kaminska E, Markland J, Sibley J, Catoggio L, Furst DE (June 2001). "A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma". Arthritis Rheum. 44 (6): 1351–8. doi:10.1002/1529-0131(200106)44:6<1351::AID-ART227>3.0.CO;2-I. PMID 11407694.
  4. ↑ 4.0 4.1 Herrick AL, Pan X, Peytrignet S, Lunt M, Hesselstrand R, Mouthon L, Silman A, Brown E, Czirják L, Distler J, Distler O, Fligelstone K, Gregory WJ, Ochiel R, Vonk M, Ancuţa C, Ong VH, Farge D, Hudson M, Matucci-Cerinic M, Balbir-Gurman A, Midtvedt Ø, Jordan AC, Jobanputra P, Stevens W, Moinzadeh P, Hall FC, Agard C, Anderson ME, Diot E, Madhok R, Akil M, Buch MH, Chung L, Damjanov N, Gunawardena H, Lanyon P, Ahmad Y, Chakravarty K, Jacobsen S, MacGregor AJ, McHugh N, Müller-Ladner U, Riemekasten G, Becker M, Roddy J, Carreira PE, Fauchais AL, Hachulla E, Hamilton J, İnanç M, McLaren JS, van Laar JM, Pathare S, Proudman S, Rudin A, Sahhar J, Coppere B, Serratrice C, Sheeran T, Veale DJ, Grange C, Trad GS, Denton CP (July 2017). "Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS)". Ann. Rheum. Dis. 76 (7): 1207–1218. doi:10.1136/annrheumdis-2016-210503. PMC 5530354. PMID 28188239. Vancouver style error: initials (help)
  5. ↑ van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB (April 1996). "Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial". Br. J. Rheumatol. 35 (4): 364–72. PMID 8624641.
  6. ↑ Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M, Seibold JR, Riley DJ, Hsu VM, Varga J, Schraufnagel DE, Theodore A, Simms R, Wise R, Wigley F, White B, Steen V, Read C, Mayes M, Parsley E, Mubarak K, Connolly MK, Golden J, Olman M, Fessler B, Rothfield N, Metersky M (June 2006). "Cyclophosphamide versus placebo in scleroderma lung disease". N. Engl. J. Med. 354 (25): 2655–66. doi:10.1056/NEJMoa055120. PMID 16790698.
  7. ↑ Robertson LP, Marshall RW, Hickling P (March 2003). "Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline". Ann. Rheum. Dis. 62 (3): 267–9. PMC 1754479. PMID 12594118.
  8. ↑ Tosounidou S, MacDonald H, Situnayake D (May 2014). "Successful treatment of calcinosis with infliximab in a patient with systemic sclerosis/myositis overlap syndrome". Rheumatology (Oxford). 53 (5): 960–1. doi:10.1093/rheumatology/ket365. PMID 24255162.
  9. ↑ de Paula DR, Klem FB, Lorencetti PG, Muller C, Azevedo VF (February 2013). "Rituximab-induced regression of CREST-related calcinosis". Clin. Rheumatol. 32 (2): 281–3. doi:10.1007/s10067-012-2124-z. PMID 23179007.
  10. ↑ Ennis H, Hughes M, Anderson ME, Wilkinson J, Herrick AL (February 2016). "Calcium channel blockers for primary Raynaud's phenomenon". Cochrane Database Syst Rev. 2: CD002069. doi:10.1002/14651858.CD002069.pub5. PMID 26914257.
  11. ↑ La Civita L, Pitaro N, Rossi M, Gambini I, Giuggioli D, Cini G, Ferri C (June 1993). "Amlodipine in the treatment of Raynaud's phenomenon". Br. J. Rheumatol. 32 (6): 524–5. PMID 8508292.
  12. ↑ Roustit M, Blaise S, Allanore Y, Carpentier PH, Caglayan E, Cracowski JL (October 2013). "Phosphodiesterase-5 inhibitors for the treatment of secondary Raynaud's phenomenon: systematic review and meta-analysis of randomised trials". Ann. Rheum. Dis. 72 (10): 1696–9. doi:10.1136/annrheumdis-2012-202836. PMID 23426043.
  13. ↑ Dziadzio M, Denton CP, Smith R, Howell K, Blann A, Bowers E, Black CM (December 1999). "Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial". Arthritis Rheum. 42 (12): 2646–55. doi:10.1002/1529-0131(199912)42:12<2646::AID-ANR21>3.0.CO;2-T. PMID 10616013.

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