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
        • Alternative regimen (1): Topical calcipotriene 0.005% ointment twice daily
        • Alternative regimen (2): mycophenolate mofetil 500 mg PO q12h for 7-14 days
    • 1.2 Diffuse sclerosis of the skin
      • 1.2.1 Adult
        • Preferred regimen (1): methotrexate
        • 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[3]
        • Alternative regimen (2): cyclophosphamide
    • 1.3 Calcinosis
      • 1.3.1 Adult
        • Preferred regimen (1): minocycline 50-100 mg PO q12h for 6-12 weeks[4]
        • Alternative regimen (1): infliximab
        • Alternative regimen (2): rituximab
    • 1.4 Raynaud's phenomenon
      • 1.4.1 Adult
        • Oral regimen
          • Preferred regimen (1): Nifedipine
          • Preferred regimen (2): Amlodipine
          • Alternative regimen (1): Sildenafil
          • Alternative regimen (2): losartan
        • Topical regimen
          • Alternative regimen (3): Topical nitrates


  • 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. 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)
  4. 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.

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