Further Inpatient Care

Patients with systemic sclerosis may need to be treated by other subspecialists, depending on the involvement of specific organ systems (eg, cardiologist, pulmonologist, gastroenterologist, nephrologist, endocrinologist, vascular surgeon, wound care specialist, hand surgeon).

Kidney and lung transplantation are performed in specialized centers for patients with end-stage kidney or lung involvement.

Current studies of autologous stem cell transplantation, including a large randomized control study (SCOT trial), are ongoing. Several uncontrolled studies have indicated that this procedure may lead to disease remission.

Patient Education

Patients should be counseled to keep regular follow up appointments with their rheumatologist. Patients should be vigilant to look for decreases in their exercise tolerance as cardiopulmonary disease is the largest cause of mortality in scleroderma. Patients should also be vigilant to watch their Raynaud's phenomenon closely, avoid cigarette smoke, conservatively warm their hands in cool weather/environments, and look for any developing digital tip ulcerations. Consider providing patients with diffuse systemic sclerosis a blood pressure cuff so they can monitor their blood pressure weekly (to screen for scleroderma renal crisis).

Questions

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Media Gallery

Scleroderma: Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles.
Scleroderma: Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
Scleroderma: 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).
Scleroderma: 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.
Scleroderma: 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.
Overall scheme illustrating a proposed sequence of events involved in tissue fibrosis and fibroproliferative vasculopathy in systemic sclerosis. An unknown causative agent induces activation of immune and inflammatory cells in genetically predisposed hosts, resulting in chronic inflammation. Activated inflammatory and immune cells secrete cytokines, chemokines, and growth factors, which cause fibroblast activation, differentiation of endothelial and epithelial cells into myofibroblasts, and recruitment of fibrocytes from the bone marrow and the peripheral blood. The activated myofibroblasts produce exaggerated amounts of extracellular matrix, resulting in tissue fibrosis.

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Table 1. ACR/EULAR Revised Systemic Sclerosis Classification Criteria

Table 1. ACR/EULAR Revised Systemic Sclerosis Classification Criteria

Item	Sub-item(s)	Score*
Skin thickening of the fingers of both hands extending proximally to the metacarpophalangeal joints (presence of this criterion is sufficient criterion for SSc classification)	None	9
Skin thickening of the fingers (count the higher score only)	Puffy fingers	2
	Sclerodactyly (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)	4
Fingertip lesions (count the higher score only)	Digital tip ulcers	2
Fingertip lesions (count the higher score only)	Fingertip pitting scars	3
Telangiectasia	None	2
Abnormal nailfold capillaries	None	2
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)	Pulmonary arterial hypertension	2
	Interstitial lung disease	2
Raynaud phenomenon	None	3
Systemic sclerosis–related autoantibodies (maximum score is 3)	Anticentromere	3
	Anti–topoisomerase I	3
	Anti–RNA polymerase III	3
The total score is determined by adding the maximum score in each category. Patients with a total score ≥ 9 are classified as having definite systemic sclerosis (modified from van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum*. Nov 2013;65(11):2737-47.^[1])

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Author

Abhishek Nandan, MD Associate Professor of Medicine, Associate Program Director for Rheumatology Fellowship, Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Virginia Commonwealth University School of Medicine; Staff Physician, Richmond VA Medical Center, Central Virginia Veterans Health Care System

Abhishek Nandan, MD is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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

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

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; 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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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 and Translational Research, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon) Professor and Co-Director, Jefferson Institute of Molecular Medicine, Director, Connective Tissue Diseases Section, Director, The Scleroderma Center, Sidney Kimmel Medical College of Thomas Jefferson University

Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon) is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Society for Biochemistry and Molecular Biology, Royal College of Physicians, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Fabian A Mendoza-Ballesteros, MD, FACR Assistant Professor of Medicine, Division of Rheumatology, Jefferson Institute of Molecular Medicine; Associate Director of The Scleroderma Center, Director of Scleroderma Program, Sidney Kimmel Medical College of Thomas Jefferson University

Fabian A Mendoza-Ballesteros, MD, FACR is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Acknowledgements

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.

Acknowledgments

The assistance of Kenneth Brown in the preparation of this manuscript is gratefully acknowledged.

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