Systemic Sclerosis Treatment & Management

Updated: Apr 16, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Different treatment regimens for systemic sclerosis exist. The therapeutic approach depends on the presentation of the disease and complexity of symptoms. In a recent review of the literature, calcium channel blockers, prostanoids, tadalafil, and bosentan received the strongest recommendations for their effectiveness for Raynaud phenomenon and digital ulcers. [45]

In pruritus, the following agents are sometimes helpful:

  • Camphor and menthol

  • Topical emollients

  • Psoralen UV-A (PUVA) treatment

  • UVA-1 phototherapy

In patients with calcinosis, surgery may be of some benefit, but healing time is often prolonged.

When Raynaud phenomenon is present, the most effective nonpharmacologic method of preventing Raynaud episodes is avoiding exposure to cold temperature and wearing layers of warm, loose-fitting clothing, including socks and gloves. Also, smoking cessation is advised. In the pharmacologic regimen, consider the use of agents such as calcium-channel blockers, vasodilating drugs, intravenous prostaglandins, prostacyclin analogs, or aspirin. [46]

In patients with kidney involvement, ACE or angiotensin II inhibitor therapy is indicated.

In patients with GI tract involvement, proton pump inhibitors (eg, omeprazole) and H2 blockers can help to control reflux symptoms.

In patients with lung involvement, calcium-channel blockers (eg, nifedipine), prostaglandins (eg, prostacyclin), and cyclophosphamide have been used with variable success. When inflammatory myositis is present, the use of high doses of corticosteroids (eg, prednisolone with a starting dose of 1 mg/kg/d) is suggested.

Antifibrotic agents have been investigated, although results have varied and none is clearly shown to be of consistent benefit. These have included D-penicillamine, interferon alfa and interferon gamma, and immunomodulatory agents. Immunomodulatory agents have included the following:

  • Photopheresis [47]

  • Corticosteroids (in inflammatory myositis, pericarditis, refractory arthritis, or alveolitis)

  • Methotrexate (15 mg/wk) [48]

  • Chlorambucil

  • Mycophenolate mofetil may benefit patients with diffuse progressive cutaneous systemic sclerosis of recent onset, a small prospective study suggested [49]

  • Cyclosporine [50]

  • FK506 (tacrolimus)

  • Thalidomide

  • Low-dose intravenous cyclophosphamide [51, 52]

  • Statins [53, 54]

  • Immunosuppressive drugs with autologous peripheral hematopoietic stem cell transplantation [55]

Because of their antifibrotic properties, the tyrosine kinase inhibitors imatinib mesylate and nintedanib may be potential therapies. [52, 53, 56, 57, 58]

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Surgical Care

The cutaneous telangiectasias can be treated. The number of pulsed dye laser treatments required to effectively clear systemic sclerosis/CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome telangiectasia was approximately 2-fold higher than with sporadic telangiectasias, a finding attributed to thickened collagen fibers and blood vessels. [59] Pulsed dye laser (PDL) and intense pulsed light (IPL) may be used, both being effective, although the former produces a better cosmetic result but with more adverse effects than the latter. [60]

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Consultations

The symptoms of systemic sclerosis are diverse; therefore, consider consultations with the following specialists, if applicable:

  • Dermatologist

  • Pulmonologist

  • Nephrologist

  • Radiologist

  • Cardiologist

  • Orthopedic surgeon

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