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Systemic Sclerosis Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 02, 2016
 

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.[37]

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.[38]

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 [39]
  • Corticosteroids (in inflammatory myositis, pericarditis, refractory arthritis, or alveolitis)
  • Methotrexate (15 mg/wk) [40]
  • Chlorambucil
  • Mycophenolate mofetil may benefit patients with diffuse progressive cutaneous systemic sclerosis of recent onset, a small prospective study suggested [41]
  • Cyclosporine [42]
  • FK506 (tacrolimus)
  • Thalidomide
  • Low-dose intravenous cyclophosphamide [43, 44]
  • Statins [45, 46]
  • Immunosuppressive drugs with autologous peripheral hematopoietic stem cell transplantation [47]

Because of their antifibrotic properties, the tyrosine kinase inhibitors imatinib mesylate and nintedanib may be potential therapies.[44, 45, 48, 49, 50]

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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.[51] 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.[52]

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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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Complications

Neoplastic diseases may complicate the disease course. Examples include breast carcinoma; multiple myeloma; lymphoma; and cancer of the ovary, esophagus, colon, or rectum.

Female patients should be evaluated for breast cancer. Epidemiologic studies have suggested that patients with scleroderma have an increased risk of cancer. However, large-scale case-control studies are needed to substantiate a possible association between scleroderma—both cutaneous and systemic—and breast cancer.[53]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Anna Zalewska, MD, PhD Professor of Dermatology and Venereology, Psychodermatology Department, Chair of Clinical Immunology and Microbiology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Mark W Cobb, MD Consulting Staff, WNC Dermatological Associates

Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Acknowledgements

Bozena Dziankowska-Bartkowiak, MD, PhD Consulting Staff, Department of Dermatology, University Hospital, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Anna Sysa-Jedrzejowska, MD, PhD Head, Professor, Department of Dermatology and Venereology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

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Face of 65-year old woman with systemic sclerosis and skin thickening of 20 years' duration: Note the pinched nose, taut skin with numerous telangiectasias, and retraction of the lips.
Telangiectasias affecting the face: They are pronounced and numerous, especially in the atrophic phase of the disease. Radical furrowing around the mouth is also characteristic in the later stage of the disease.
Raynaud phenomenon of the hands: Symmetrical acral vasospasm is present, with characteristic pallor, cyanosis, suffusion, and a sense of fullness and tautness.
Puffy appearance of the woman's hand in the edematous phase of early scleroderma.
In systemic sclerosis, ulceration at the tip of the finger is regarded to be secondary to ischemia.
Hand of a woman with scleroderma of several years' duration: The thickened, tight, thin skin over the fingers is the result of self-amputation of the distal phalanx due to ischemia. Moderately severe flexion contractures of the fingers are present.
In systemic sclerosis, skin hyperpigmentation of the lower legs is surrounded by areas of hypopigmentation. The result is a salt-and-pepper appearance.
 
 
 
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