eMedicine Specialties > Rheumatology > Systemic Rheumatic Disease

Scleroderma: Differential Diagnoses & Workup

Author: Sergio A Jimenez, MD, Director, The Scleroderma Center; Co-Director, Jefferson Institute of Molecular Medicine; Director, Division of Connective Tissue Diseases; Professor of Medicine, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University
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; Andrew Koenig, ; Marie Spevak O'Brien, DO, Assistant Clinical Professor of Medicine, Arthritis and Rheumatology, Lehigh Valley Physician Group
Contributor Information and Disclosures

Updated: Oct 14, 2008

Differential Diagnoses

Eosinophilia
Primary Biliary Cirrhosis
Eosinophilia-Myalgia Syndrome
Pulmonary Hypertension, Primary
Eosinophilic Fasciitis
Reflex Sympathetic Dystrophy
Graft Versus Host Disease
Scleroderma
Mycosis Fungoides

Other Problems to Be Considered

Bleomycin-induced scleroderma
Toxic oil syndrome (adulterated rape seed oil)
Porphyria cutanea tarda
Digital sclerosis of diabetes mellitus
Morphea
Linear scleroderma
Vibration disease
Radiation exposure
Scleroderma sine scleroderma
Intestinal obstruction
Infiltrative cardiomyopathy
Nephrogenic fibrosing dermopathy (nephrogenic systemic fibrosis)
Amyloidosis
Scleromyxedema (generalized lichen myxedematosus)
Scleredema adultorum of Buschke
Scleredema diabeticorum

Workup

Laboratory Studies

  • The role of the immune system in the pathogenesis of systemic sclerosis remains unclear; however, patients have specific humoral and cell-mediated immunity abnormalities.
  • Antinuclear antibodies are present in about 95% of affected patients, usually with a speckled or centromere pattern. A nucleolar pattern, although less common, is more specific for systemic sclerosis.
  • Cell-mediated abnormalities involve lymphocytes, mononuclear phagocytes, and mast cells.
  • Topoisomerase I antibodies (also known as Scl-70) are present in approximately 30% of patients with diffuse disease (absent in limited disease) and are associated with pulmonary fibrosis.11
  • Anticentromere antibodies are present in about 80-90% of patients with limited disease and are rare in patients with diffuse disease.
  • Fibrillarin antibodies and antibodies to U3 ribonucleoprotein (RNP) may be present. Anti-U3RNP is present mostly in patients with diffuse disease with overlap syndromes. In addition, these antibodies are more common in patients with skeletal muscle involvement and pulmonary disease.
  • Anti-ThRNP is present mostly in limited disease and is associated with more extensive visceral disease.
  • Anti-PM-Scl is present in limited and overlap states and is associated with myositis and renal involvement.
  • A microangiopathic hematologic picture may precede renal crisis.
  • Recent studies have reported new autoantibodies in systemic sclerosis that may play a role in its pathogenesis;12 these autoantibodies include antiendothelial cell (AECA), anti-fibrillin (FBN1), anti–matrix metalloproteinase (MMP)–1 and anti–MMP-3, and anti–platelet-derived growth factor receptor (PDGFr).13

Imaging Studies

  • CT scan: HRCT scan is required to evaluate pulmonary involvement. Imaging may reveal a ground-glass appearance, possibly indicating active alveolitis. Ground-glass appearance on HRCT scan is the first abnormality observed during the development of lung fibrosis and is subsequently replaced by honeycombing and traction bronchiectasis or bronchiolectasis. HRCT scanning should be performed every 6 months if active alveolitis or interstitial pulmonary fibrosis is present and every year if these abnormalities are not present.
  • Radiography: Chest radiography is an insensitive imaging procedure that shows only late findings of pulmonary fibrosis, such as increased interstitial markings. Extremity radiography should be performed to reveal calcinosis and resorption of the distal tufts of the digits.
  • Echocardiography: Conduct this test to evaluate the patient's pulmonary artery pressure and to assess septal fibrosis or pericardial effusions. Roughly 30% of patients have asymptomatic pericardial effusions (see Image 6).
  • Right-heart catheterization: This remains the standard criterion for diagnosing pulmonary hypertension and is performed after an elevated pulmonary artery pressure is found on echocardiographic screening.7
  • Esophagraphy: Perform this test to document esophageal dysmotility and an incompetent LES.

Other Tests

  • Pulmonary function testing (every 6 mo)
    • Conduct this test to evaluate the DLCO, FVC, and TLC.
    • This is a very sensitive technique for detecting early fibrotic changes, alveolitis, and pulmonary hypertension.
    • An isolated reduction in the DLCO is the best predictor of pulmonary hypertension. A FVC/DLCO or TLC/DLCO of greater than 1.6 is highly suggestive of pulmonary hypertension.
  • Serum N-terminal pro-brain natriuretic peptide (NT-proBNP): Elevation of NT-proBNP levels may correlate with early pulmonary hypertension.14
  • Cardiac rhythm monitoring: Perform 24-hour ambulatory Holter monitoring to evaluate arrhythmias and serious conduction defects.
  • Esophagogastroduodenoscopy, esophageal manometry, and pH monitoring studies: Conduct these studies to survey and evaluate the upper GI system.

Procedures

  • Nail-fold capillary microscopy: This study demonstrates fewer capillaries than normal (ie, capillary loop drop [see Image 5]) and numerous dilated capillary loops.
  • Bronchoscopy with bronchoalveolar lavage: This is used to assess for active lung inflammation.15

Histologic Findings

Systemic sclerosis is characterized by excessive fibrosis in the skin and other affected organs (see Images 9-10). The skin and lungs also show prominent T-lymphocyte infiltration. A severe fibroproliferative vasculopathy that affects small arteries and arterioles is universally present in affected organs. Platelet microthrombi are often found in the lumen of the narrowed vessels.

More on Scleroderma

Overview: Scleroderma
Differential Diagnoses & Workup: Scleroderma
Treatment & Medication: Scleroderma
Follow-up: Scleroderma
Multimedia: Scleroderma
References
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Further Reading

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Keywords

scleroderma, systemic sclerosis, proximal scleroderma, diffuse cutaneous scleroderma, limited cutaneous scleroderma, diffuse systemic scleroderma, progressive systemic sclerosis, hard skin, skin tightening, skin tightness, sclerodactyly, bibasilar pulmonary fibrosis, Raynaud phenomenon, Raynaud's phenomenon, pulmonary hypertension, calcinosis, esophageal dysmotility, telangiectasias, CREST, juvenile-onset systemic sclerosis

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Contributor Information and Disclosures

Author

Sergio A Jimenez, MD, Director, The Scleroderma Center; Co-Director, Jefferson Institute of Molecular Medicine; Director, Division of Connective Tissue Diseases; Professor of Medicine, Department of Dermatology and Cutaneous Biology, Thomas Jefferson University
Sergio A Jimenez, MD 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 Koenig, 
Andrew Koenig 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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

Alex J Mechaber, MD, FACP, 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, Professor of Medicine, Temple University 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
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