The Many Faces 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
References (80)
- et al.
The early history and nomenclature of scleroderma and of its differentiation from sclerema neonatorum and scleroedema
Semin Arthritis Rheum
(1982) - et al.
Identification of a nuclear protein (Scl-70) as a unique target of human antinuclear antibodies in scleroderma
J Biol Chem
(1979) - et al.
Factors predicting development of renal involvement in progressive systemic sclerosis
Am J Med
(1984) - et al.
HLA and ethnic associations among systemic sclerosis patients with anticentromere antibodies
Hum Immunol
(1995) - et al.
Severe systemic sclerosis with anti-topoisomerase I antibodies is associated with an HLA-DRw11 allele
Hum Immunol
(1994) - et al.
Antibody-mediated gastrointestinal dysmotility in scleroderma
Gastroenterology
(2002) Antinuclear antibody in systemic sclerosis (scleroderma)
Rheum Dis Clin North Am
(1996)- et al.
Function blocking autoantibodies against matrix metalloproteinase-1 in patients with systemic sclerosis
J Invest Dermatol
(2003) - et al.
Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status
Respir Med
(1996) - et al.
Antinuclear antibody (ANA) patterns in hepatic and extrahepatic autoimmune disease
J Hepatol
(1999)
Autoantibodies in systemic sclerosis
Semin Arthritis Rheum
Does mixed connective tissue disease exist? Yes
Rheum Dis Clin North Am
Kinetics of anti-fibrillin-1 autoantibodies in MCTD and CREST syndrome
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)
Cited by (88)
Red and white lesion of the tongue in a patient with cutaneous findings
2023, Journal of the American Dental AssociationRacial Disparities in Systemic Sclerosis
2020, Rheumatic Disease Clinics of North AmericaCitation Excerpt :Canadians of Chinese descent are more likely to be ATA positive than those of European descent.17 Anticentromere antibody (ACA) is associated with limited cutaneous systemic sclerosis (lcSSc) and pulmonary arterial hypertension (PAH).18 ACA is less prevalent among African Americans,13,14 Afro-Brazilians,20 and black patients of the EUSTAR cohort15 relative to white patients.
High serum levels of silica nanoparticles in systemic sclerosis patients with occupational exposure: Possible pathogenetic role in disease phenotypes
2018, Seminars in Arthritis and RheumatismCitation Excerpt :Different molecular and cellular requirements may be involved in two distinct pathological processes leading to inflammasome activation and fibrosis production responsible for tissue damage [11; Fig. 3]. The same pathogenetic mechanisms might be operative in the setting of SSc with silica exposure and specific genetic susceptibility; in this respect, the natural course of the disease may reproduce both pathological processes above-mentioned [1–3]. In particular, typical inflammatory manifestations, i.e. puffy fingers and/or lung alveolitis, often characterize the early stages of the disease that very frequently may progress to overt fibrosis of the skin and visceral organs of advanced scleroderma (Fig. 4).