Scleroderma Treatment & Management

  • Author: Sergio A Jimenez, MD, MACR; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jul 22, 2011
 

Medical Care

  • Skin thickening can be treated with numerous experimental drugs or interventions (D-penicillamine, interferon-gamma, mycophenolate mofetil, cyclophosphamide, photopheresis, allogeneic bone marrow transplantation[17] ). However, the US Food and Drug Administration (FDA) has not approved any therapies for systemic sclerosis. No placebo-controlled studies have demonstrated superiority, although some large uncontrolled series suggest beneficial effect from D-penicillamine. Interferon-gamma is effective, but its use is limited because it activates inflammatory and endothelial cells. Allogeneic bone marrow transplantation has been shown effective in uncontrolled studies.
  • Pruritus can be treated with moisturizers, histamine 1 (H1) and histamine 2 (H2) blockers, tricyclic antidepressants, and trazodone.
  • Raynaud phenomenon can be treated with calcium channel blockers (to tolerance),[15] prazosin, prostaglandin derivatives such as prostaglandin E1, dipyridamole, aspirin, and topical nitrates. In the event of thrombosis and vascular flow compromise, a tissue plasminogen activator, heparin, and urokinase may be necessary. In very severe cases, patients may benefit from a pharmacologic cervical sympathectomy or from surgical digital sympathectomy. Bosentan, a dual endothelin receptor antagonist, may curtail the formation of new digital ulcers. Sildenafil has also been shown to be effective and well tolerated in patients with primary Raynaud phenomenon and is currently approved to treat pulmonary hypertension.[18, 19]
  • GI symptoms may be treated with antacids, H2 blockers, reflux and aspiration precautions, proton pump inhibitors, prokinetic agents, octreotide, smaller meals, and laxatives.
  • Pulmonary fibrosing alveolitis may be treated with cyclophosphamide, either orally or in intravenous pulses.[20, 16] Several recent nonrandomized studies have also shown benefit from mycophenolate mofetil.[21, 22, 23, 24]
  • Pulmonary hypertension may require supplemental oxygen. Bosentan is effective in treating primary (idiopathic) pulmonary hypertension, as well as pulmonary hypertension associated with systemic sclerosis, and has demonstrated substantial clinical and hemodynamic improvement in patients with systemic sclerosis–associated pulmonary hypertension.[25] Numerous newer agents are available to treat pulmonary hypertension or are currently being tested in open and randomized controlled trials. These newer agents include other endothelin receptor antagonists such as sitaxsentan (clinical trials stopped worldwide because of liver injury)
  • and ambrisentan; prostaglandin derivatives such as epoprostenol, treprostinil, beraprost, and iloprost; and phosphodiesterase type 5 (PDE-5) inhibitors such as sildenafil and tadalafil. These latter two compounds have been approved by the FDA for the treatment of pulmonary hypertension.
  • On December 10, 2010, Pfizer announced that sitaxsentan (also known as sitaxentan; brand name of Thelin) is being withdrawn in regions where it is approved (the European Union, Canada, Australia). Additionally, clinical trials for sitaxsentan are being discontinued worldwide. This decision was based on a review of safety information from clinical trials and postmarketing reports of life-threatening idiosyncratic risk for liver injury.
  • Renal crisis episodes are best prevented and treated with the aggressive use of ACE inhibitors at the earliest signs of hypertension.
  • Myositis may be treated cautiously with steroids (first choice), methotrexate, and azathioprine. Doses of prednisone greater than 40 mg/d are associated with a higher incidence of sclerodermal renal crisis.[26]
  • Arthralgias can be treated with acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs).
  • Proteinuria is not uncommon in patients with scleroderma who are receiving D-penicillamine.
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Surgical Care

Digital sympathectomy may be used in patients with severe Raynaud phenomenon who have an unrelenting acute attack and who are threatened by digital loss. Many ulcers require management by a wound care specialist. Debridement or amputation may be required in severe ischemic or infected digital lesions. Hand surgery may be performed to correct severe flexion contractures. Removal of severely painful or draining of infected calcinotic deposits is occasionally required.

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Consultations

Ensure that all patients with systemic sclerosis are treated in conjunction with an experienced rheumatologist who has a full understanding of the disease, the complications of the therapies, and the frequently serious adverse effects.

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Diet

Instruct the patient to avoid large does of vitamin C (>1000 mg/d) because it stimulates collagen formation and may enhance its deposition.

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Activity

  • Ensure that the patient maintains a core body temperature to try to minimize the Raynaud phenomenon.
  • Assist the patient in avoiding contamination of any skin wound caused by ischemic lesions or calcinosis.
  • Digital ulcers must be kept clean and dry.
  • Instruct the patient to perform continuous physical and occupational therapy to maintain range of motion and to minimize or delay contractures.
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Contributor Information and Disclosures
Author

Sergio A Jimenez, MD, MACR  Fellow, Royal College of Physicians United Kingdom, Director, The Scleroderma Center; Professor and Co-Director, Jefferson Institute of Molecular Medicine; Professor of Biochemistry and Molecular Biology; Director, Division of Connective Tissue Diseases, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University

Sergio A Jimenez, MD, MACR is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Society for Biochemistry and Molecular Biology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Patrick M Cronin, DO, FACR  Clinical Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania Health System, Pennsylvania Hospital

Patrick M Cronin, DO, FACR is a member of the following medical societies: American College of Rheumatology, American Osteopathic Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Andrew S Koenig, DO  Consulting Staff, Division of Rheumatology, Rancoccas Hospital

Andrew S Koenig, DO is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Osteopathic Association

Disclosure: Nothing to disclose.

Marie Spevak O'Brien, DO  Assistant Clinical Professor of Medicine, Arthritis and Rheumatology, Lehigh Valley Physician Group

Marie Spevak O'Brien, DO is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association, American Osteopathic Association, International Society for Clinical Densitometry, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

John Varga, MD  Professor, Department of Internal Medicine, Division of Rheumatology, Northwestern University

John Varga, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Central Society for Clinical Research, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Acknowledgments

The expert assistance of Susan V. Castro, PhD, in preparation of the manuscript is gratefully acknowledged.

Sergio A Jimenez, MD, was recipient of NIH/NIAMS grant #RO-1 AR19616.

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Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles.
Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
Anterior chest demonstrating salt-and-pepper hypopigmentation and diffuse hyperpigmentation in a white woman.
A radiograph of the distal digits demonstrating calcinosis and distal phalanx reabsorption (acral osteolysis).
Fingernail capillary bed demonstrating capillary dropout with large dilated vessels.
Lung biopsy demonstrating severe interstitial fibrosis and medial fibrosis and smooth muscle hyperplasia of a pulmonary arteriole compatible with pulmonary hypertension.
Lung biopsy demonstrating expansion of the interstitium of the lung by fibrous tissue along with chronic inflammatory cells.
Barium swallow demonstrating reflux into the distal esophagus, as well as an accordion appearance in the duodenum.
Skin biopsy showing extensive fibrosis. The biopsy has a square morphology, which reflects the rigidity of the tissue biopsy specimen due to striking pan-dermal sclerosis. In addition, the fibrosing reaction extends into the panniculus. The number of adnexal structures is reduced, another characteristic feature of scleroderma. A significant inflammatory cell infiltrate is not observed. This is in contradistinction to morphea, in which a prominent inflammatory cell infiltrate is present.
Skin biopsy showing severe fibrosis. The fibrosis reflects a widening of collagen bundles in concert with an increase in the number of collagen fibers. Note the superimposed deposition of the newly synthesized delicate collagen bundles interposed between the preexisting collagen bundles, the latter appearing wide and manifesting a hyalinized morphology.
 
 
 
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