The Many Faces of Scleroderma

doi:10.1016/j.rdc.2007.12.001

Rheumatic Disease Clinics of North America

Volume 34, Issue 1, February 2008, Pages 1-15

https://doi.org/10.1016/j.rdc.2007.12.001 Get rights and content

Scleroderma autoantibodies are associated with very specific demographic, clinical, organ system, and survival features. The use of scleroderma autoantibodies may be very helpful in determining the prognosis, as well as monitoring and treatment of scleroderma patients. There are many faces of scleroderma that seem to be closely associated with scleroderma autoantibodies. These antibodies should be used in performing clinical trials and in doing genetic and basic research. Hopefully, these scleroderma antibodies will lead to a better understanding of the pathogenesis of scleroderma.

Section snippets

Limited scleroderma

Traditionally, systemic sclerosis has been divided into limited and diffuse scleroderma because of the major differences in the extent of cutaneous disease and the type of organ systems that are involved in these subsets. However, this was based on the classic old terms “CREST” and “progressive systemic sclerosis.” Patients with limited scleroderma can have one of four of the scleroderma-specific antibodies. Table 1 describes some of the major differences between these autoantibody subsets.

Anti-topoisomerase antibody

The autoantibodies in patients with diffuse cutaneous scleroderma are associated with distinctive and discriminating features (Table 2). The antinuclear pattern is either speckled or homogeneous. Occasionally it may be speckled. Patients with anti-TOPO have classic “diffuse” scleroderma. Thirty percent of African Americans with scleroderma have this autoantibody. Raynaud's is usually the first symptom and there is a variable time range before the onset of other symptoms. Most develop hand

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The Prevalence of systemic sclerosis (SSc) and different clinical subsets varies across the world. Few data have been published on SSc patients in North Africa. Our objective was to describe a SSc cohort in south of Tunisia and to compare clinical findings, disease subsets and antibodies with other international SSc populations.
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The Many Faces of Scleroderma

Section snippets

Limited scleroderma

Anti-topoisomerase antibody

Semin Arthritis Rheum

J Biol Chem

Am J Med

Hum Immunol

Hum Immunol

Gastroenterology

Rheum Dis Clin North Am

J Invest Dermatol

Respir Med

J Hepatol

Semin Arthritis Rheum

Rheum Dis Clin North Am

J Autoimmun

Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis

Arthritis Rheum

The ‘CREST’ syndrome. Comparison with systemic sclerosis (scleroderma)

Arch Intern Med

Preliminary criteria for the classification of systemic sclerosis (scleroderma)

Arthritis Rheum

Serum antinuclear antibodies in progressive systemic sclerosis (scleroderma)

Arthritis Rheum

Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma)

Ann Intern Med

Autoantibody to centromere (kinetochore) in scleroderma sera

Proc Natl Acad Sci U S A

Scleroderma (systemic sclerosis): classification, subsets and pathogenesis

J Rheumatol

Diversity of antinuclear antibodies in progressive systemic sclerosis. Anti-centromere antibody and its relationship to CREST syndrome

Arthritis Rheum

Bibliographical study of the concurrent existence of anticentromere and antitopoisomerase I antibodies

Clin Rheumatol

Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched for sex, age, and disease duration

Ann Rheum Dis

Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody

J Rheumatol

Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis

Mod Rheumatol

Pulmonary involvement in systemic sclerosis (scleroderma)

Arthritis Rheum

Serum autoantibody to the nucleolar antigen PM-Scl. Clinical and immunogenetic associations

Arthritis Rheum

Autoantibody to Th ribonucleoprotein (nucleolar 7-2 RNA protein particle) in patients with systemic sclerosis

Arthritis Rheum

Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis

Arthritis Rheum

Autoantibody reactive with RNA polymerase III in systemic sclerosis

Ann Intern Med

Studies of HLA-DR and DQ alleles in systemic sclerosis patients with autoantibodies to RNA polymerases and U3-RNP (fibrillarin)

J Rheumatol

HLA and clinical associations in systemic sclerosis patients with anti- Th/To antibodies

Arthritis Rheum

The genetics of systemic sclerosis

Curr Rheumatol Rep

Clinical, immunologic, and genetic features of familial systemic sclerosis

Arthritis Rheum

Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies

Arthritis Rheum

Influence of ethnic background on clinical and serologic features in patients with systemic sclerosis and anti-DNA topoisomerase I antibody

Arthritis Rheum

A study of the prevalence of systemic sclerosis in northeast England

Rheumatology (Oxford)

Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population

Arthritis Rheum

Disease subsets, antinuclear antibody profile, and clinical features in 127 French and 247 US adult patients with systemic sclerosis

J Rheumatol

Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients

Medicine (Baltimore)