Antibodies in Systemic Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
ANCA and Anti-PR3
DNA, Topoisomerase I
ESR (Sed Rate)
PmScl and dsDNA
TNF and IL-13

Overview of Antibodies in Systemic Scleroderma

Abstract PaintingDozens of antibodies can occur in systemic scleroderma patients. They can be useful for diagnosing, categorizing and predicting the likelihood of certain complications.

However, scleroderma is always a clinical diagnosis, which means that it is based upon symptoms and not bloodwork. This is because some people with scleroderma never develop antibodies, and also because entirely healthy people can have antibodies but never develop scleroderma or any other autoimmune disease. (Also see Overview of Antibodies, What is Scleroderma?, Types of Scleroderma and Systemic Symptoms)

Antibodies That Attack Inflammatory Immune Cells May Work to Treat Scleroderma. Antibodies against a protein selectively expressed on certain types of immune system cells were seen to prevented fibrosis in rodent models of scleroderma and lung fibrosis. Scleroderma News, 09/27/2016.

Autoantibodies Present in Scleroderma, Myositis Explored in New Review. These specific autoantibodies play a role in muscle damage, their detection in an early disease stage may help predict the disease course and prognosis. Scleroderma News, 05/27/2015.

Examination of autoantibody status and clinical features that associate with cancer risk and cancer-associated scleroderma. Increased age at scleroderma onset is strongly associated with cancer risk overall. PubMed, Arthritis Rheumatol, 01/20/2015. (Also see Cancer and Scleroderma)

Novel autoantibodies in inflammatory myopathies and systemic sclerosis. The scope of this article is to review the diagnostic and prognostic value of antibodies in inflammatory myopathies and systemic sclerosis. PubMed, Rev Med Suisse, 2015 Jan 14;11(456-457):25-9. (Also see Myopathy and Myositis)

Early systemic sclerosis (SSc): Analysis of the disease course in patients with marker autoantibody or capillaroscopic positivity or both. The data demonstrate faster progression of SSc in autoantibody–positive patients, particularly in those with preclinical internal organ involvement at baseline, than in autoantibody–negative patients. PubMed, Arthritis Care Res (Hoboken), 2014 Feb 10. (Also see Capillaroscopy)

ACA: Anticentromere Antibodies

Predicting cardiopulmonary involvement in patients with systemic sclerosis: complementary value of nailfold videocapillaroscopy (NVC) patterns and disease–specific autoantibodies. All SSc–specific auto–antibodies were found, with ACA and anti-Scl-70 being the most prevalent and the association between NVC–pattern and heart/lung involvement was independent of specific anti-ENA antibodies, which might indicate microangiopathy is an important cause of organ involvement. PubMed, Rheumatology (Oxford), 12/10/2016. (Also see Nailfold Videocapillaroscopy and Scleroderma Cardiac (Heart) Involvement)

Increased risk of digital vascular events in scleroderma patients who have both anti–centromere (CENP) and anti–nterferon–inducible protein 16 (IF–16) antibodies. This study provides further evidence that anti–CENP and anti–IFI–16 antibodies are disease biomarkers that may be used for risk stratification of vascular events in scleroderma. PubMed, Arthritis Care Res (Hoboken), 07/07/2016. (Also see Prognosis and Mortality)

Anti-centromere protein A antibodies in systemic sclerosis (SSc): Significance and origin. Preliminary evidence for a possible role of forkhead box protein E3 in SSc pathogenesis is discussed and for the association of different subsets of anti-CENP-A antibodies. PubMed, Autoimmun Rev, 10/09/2015.

Serology Helps Pinpoint Type of Scleroderma. Serologic status should be added to extent of skin involvement for more accurate classification of disease phenotype in systemic sclerosis. Medpage Today, 04/23/2015.

Impact of anti-centromere antibodies (ACA) on pulmonary function test (PFT) results in patients with systemic sclerosis without established or suspected pulmonary disease. Patients with ACA, without established or suspected pulmonary complications, have PFT abnormalities consistent with indolent increased pulmonary vascular resistance despite the majority of such patients not subsequently developing PAH. PubMed, Clin Rheumatol, 2014 Apr 22. (Also see Pulmonary Hypertension Diagnosis)

Centromere Antibody, IgG. Centromere antibody is present in 80-90% of individuals with CREST variant scleroderma. This antibody is also seen in 30% of Raynaud patients, 12% of patients with mixed connective-tissue disease, diffuse scleroderma, interstitial pulmonary fibrosis, primary biliary cirrhosis, and in a smaller percent of patients with systemic lupus erythematosus (SLE) and RA. ARUP Laboratories.

AECA: Agonistic anti-ICAM-1 Antibodies

Agonistic anti-ICAM-1 antibodies in scleroderma (SSc): Activation of endothelial pro-inflammatory cascades. Anti-endothelial cell antibodies (AECA) from SSc patients target specific endothelial antigens including ICAM-1, and cause pro-inflammatory activation of human endothelial cells, suggesting that they are not only a marker of disease but that they contribute to its progression. PubMed, Vascul Pharmacol. (Also see Causes of Scleroderma: Endothelin)

AFA: Antifibroblast Antibodies

Antifibroblast growth factor receptor 3 antibodies identify a subgroup of patients with sensory neuropathy. These antibodies identify a subgroup of patients with SN in whom an underlying autoimmune disorder affecting sensory neurons in the dorsal root and trigeminal nerve ganglia is suspected. PubMed, J Neurol Neurosurg Psychiatry, 2015 Jan 27.

ANA: Antinuclear Antibodies

Definition of Antinuclear antibody. Antinuclear antibodies (ANAs) are found in patients whose immune system is predisposed to cause inflammation against their own body tissues.

Antinuclear antibody negative systemic sclerosis. SSc patients who are ANA negative constitute a distinct subset of SSc with less vasculopathy, a greater proportion of males and possibly, more frequent lower gastrointestinal involvement. Seminars in Arthritis and Rheumatism, 11/28/2014.

ANCA and Anti-PR3

Incidence of PR3- and MPO-ANCA autoantibody specificity changes in ANCA-associated vasculitis. Antibody specificity changes in AAV are rareand monitoring only the initial antibody specificity would have missed clinical events but rising C–reactive protein presaged relapse in these cases. PubMed, Ann Clin Biochem, 2015 Mar;52(Pt 2):297-301.

ANoA: Antinucleolar Antibodies

A new immunoprecipitation-real time quantitative PCR assay for anti-Th/To and anti-U3RNP antibody detection in systemic sclerosis. Our new method readily detects these two clinically important antibodies in SSc. Making tests for anti-Th/To and -U3RNP antibodies widely available to clinicians should be helpful in the diagnosis and follow-up of SSc patients. Arthritis Research and Therapy.

APA: Antiphospholipid Antibodies

Antiphospholipid Antibodies (APS) and Systemic Scleroderma. APS is being increasingly recognized as an important cause of renal damage due to thrombosis at any location within the renal vasculature. Turk J Haematol.

AT1R and ETAR Antibodies

Angiotensin receptor type 1 (AT1R) and endothelin receptor type A (ETAR) on immune cells mediate migration and the expression of IL-8 and CCL18 when stimulated by autoantibodies from systemic sclerosis (SSc) patients. PubMed, Arthritis Res Ther, 2014 Mar 11;16(2):R65. (Also see Causes of Scleroderma: Endothelin)

BPI Antibodies

BPI Antibodies: Bactericidal/Permeability-Increasing Protein and Cathepsin G Are the Major Antigenic Targets of Antineutrophil Cytoplasmic Autoantibodies in Systemic Sclerosis. The study included 33 patients with diffuse and 35 with limited SSc. Patients with antibodies to BPI (bactericidal/permeability-increasing protein) had lower skin scores. J Rheumatol.

Caspase-3 Antibody

Autoantibody (Ab) against caspase-3, an executioner of apoptosis, in patients with systemic sclerosis (SSc). These results suggest that autoantibody against caspase-3 is generated in SSc and that this Ab is related to the severity of pulmonary fibrosis, vascular damage, and inflammation. Shihoko Okazaki (SpringerLink) Rheumatology International.

CCP: Anti-Cyclic Citrullinated Peptide Antibodies

Anticyclic citrullinated peptide antibodies in rheumatoid and nonrheumatoid rheumatic disorders: experience with 1162 patients. Anti-CCP are a hallmark of RA, but may be observed in other inflammatory, systemic, or mechanical diseases. PubMed, J Rheumatol, 2014 Dec;41(12):2395-402.

CRP: C-Reactive Protein

Being Bullied Is Bad for Your Health. Being bullied raises the blood's level of C-Reactive Protein or CRP a marker of systemic inflammation and a risk factor for cardiovascular and other diseases. New York Times, 05/12/2014. (Also see CRP: C-Reactive Protein)

DNA: Anti-DNA and Anti-Topoisomerase I Antibodies

Clinical differences between Thai systemic sclerosis (SSc) patients with positive versus negative anti-topoisomerase l (ATA). A high prevalence of ATA positivity was found among Thai SSc patients and this was associated with a high frequency of hand deformity, anti-centromere negativity, a short duration of pulmonary fibrosis in Diffuse SSc and a lower frequency of Raynaud's phenomenon in Limited SSc. PubMed, Int J Rheum Dis, 10/08/2014.


Eosinophilia in rheumatologic diseases: a prospective study of 1000 cases. Eosinophilia can be seen in various rheumatologic conditions but, as corticosteroids are one of the most common medications used in collagen tissue diseases, the eosinophil numbers found may be lower than expected and eosinophilia may be more frequent than reported. PubMed, Rheumatol Int.

IgA Antibodies

Reversible IgA deficiency after severe Gram-negative bacteria infection in a patient with systemic sclerosis. Although the mechanism of secondary IgAD is still vague, its association with autoimmune diseases including SSc and also with bacterial infection is discussed. (Springerlink) Masato Yagita. (Also see Bacterial Infections)

IgG Antibodies

Immunoglobulins. Antibodies attach to the foreign substances so the immune system can destroy them.IgG antibodies are found in all body fluids. They are the smallest but most common antibody (75% to 80%) of all the antibodies in the body. WebMD.

Epitope specificity determines pathogenicity and detectability of anti-PDGFRa autoantibodies in systemic sclerosis. Agonistic anti-PDGFRa autoantibodies are enriched in SSc sera and recognize specific conformational epitopes, that can be used to discriminate agonistic from nonagonistic antibodies and block PDGFRa signaling in SSc. PubMed, Arthritis Rheumatol, 03/25/2015.


Immunodeficiency disorders. Immunodeficiency disorders occur when the body's immune response is reduced or absent. When the immune system detects an antigen, it responds by producing proteins called antibodies that destroy the harmful substances. MedlinePlus.

KU: Anti-KU Antibodies

Ku, Rare and Specific Antigen in Scleroderma, May Be Linked to Lung Disease. Few scleroderma patients have antibodies directed specifically against the Ku antigen and have them as the only autoantibody present, are more likely to have interstitial lung disease and higher levels of a muscle degradation factor. Scleroderma News, 09/06/2016.

LPL: Anti-lipoprotein Lipase Antibody

Anti-lipoprotein lipase antibody in systemic sclerosis: association with elevated serum triglyceride concentrations. The presence of IgG anti-LPL antibody was associated with elevated serum triglyceride levels, greater extent of skin fibrosis, and more frequent presence of lung fibrosis, heart involvement, and anti-topoisomerase I antibodies. J Rheumatol. 2005 Apr;32(4):629-36. (Also see Skin Fibrosis, Pulmonary Fibrosis, and Cardiac Involvement)


Pathogenic autoantibodies: Emerging insights into tissue injury. Accumulating evidence is emerging that B lymphocytes and autoantibodies are critical in the development of autoimmune disease. Studies of autoantibodies penetrating living cells suggest a dosage effect in generating a biological outcome in vivo. PubMed, 02-28-06. (Also see Causes of Scleroderma: B and T Cells)

MMP-3 Antibodies

Autoantibody against matrix metalloproteinase-3 in patients with systemic sclerosis. These results suggest that anti-MMP-3 antibody is a serological marker that reflects the severity of SSc and also suggest that it may contribute to the development of fibrosis by inhibiting MMP-3 activity and reducing the ECM (extracellular matrix) turnover. PubMed, Clin Exp Immunol. 2004 Nov;138(2):357-63. (Also see Skin Fibrosis)

Nucleolar Antibodies

Pulmonary Arterial Hypertension (PAH) and Severe Pulmonary Fibrosis in Systemic Sclerosis Patients with a Nucleolar Antibody. Scleroderma-specific autoantibodies and the FVC%/DLCO% ratio are helpful in determining whether a patient has PAH alone, PAH along with pulmonary fibrosis, or secondary PAH from chronic hypoxia with severe pulmonary fibrosis. J Rheumatol 2007;34:2230-5. (Also see Pulmonary Hypertension, Pulmonary Fibrosis)

PM/Scl: Anti-PM/SCL-100

Anti-PM/Scl antibodies are found in Japanese patients with various systemic autoimmune conditions besides myositis and scleroderma (SSc). In Japanese patients, anti-PM/Scl antibodies are only very rarely found, and they are not always specific for dermatomyositis (DM) or SSc. Arthritis Research and Therapy, 03/11/2015.

PmScl and dsDNA

Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody. Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile. Seminars in Arthritis and Rheumatism, 07/15/2014. (Also see Pulmonary Fibrosis Prognosis)

RNA-Polymerases Antibodies

Systematic autoantigen analysis identifies a distinct subtype of scleroderma with coincident cancer. Strong evidence was found for both intra and intermolecular epitope spreading in patients with RNA polymerase III (POLR3) and the minor spliceosome specificities. PNAS, 11/07/2016.

Specific autoantibody profiles and disease subgroups correlate with circulating micro-RNA (miRNA) in systemic sclerosis. Circulating miRNA profiles differ between limited and diffuse scleroderma patients and between patients with different autoantibodies. PubMed, Rheumatology (Oxford), 07/10/2015.

Demographic and clinical features of systemic sclerosis (SSc) patients with anti-RNA polymerase III antibodies (RNAP). Measurement of RNAP in SSc patients is useful for the diagnosis and risk stratification of severe manifestation, such as renal crisis and severe skin sclerosis. PubMed, J Dermatol, 12/06/2014.

SCL-70 Antibodies

Fever of unknown origin secondary to type I crescentic glomerulonephritis (CGN) and anti-SCl 70 antibodies without clinical manifestations of systemic sclerosis. Anti-Scl 70 antibodies are highly specific for scleroderma and are seldom present in other diseases. As far as we are aware there are no published cases of the association of type 1 CGN with anti-Scl 70 antibodies. Vega Stieb J. (PubMed) Clin Exp Nephrol. 2008 Oct;12(5):388-92. (Also see Renal Involvement)

Clinical risk assessment of organ manifestations in systemic sclerosis. Diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. PubMed, Ann Rheum Dis. 2007 Feb 1. (Also see Types of Scleroderma)

TAA: Tumor-associated antigens

Tumor-associated antigens (TAAs) in systemic sclerosis and systemic lupus erythematosus: Associations with organ manifestations, immunolaboratory markers and disease activity indices. The production of some TAAs may also be increased in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and other connective tissue diseases. E Szekanecz. PubMed, Journal of Autoimmunity.

TNF and IL-13: Tumor Necrosis Factor and Interleukin-13

Elevated serum levels of a proliferation-inducing ligand in patients with systemic sclerosis: Possible association with myositis? Our preliminary results suggest increased serum a proliferation-inducing ligand (APRIL) levels in systemic sclerosis patients, particularly in those associated with myositis and hypergammaglobinemia. IS Bassyouni. Joint Bone Spine. (Also see Tumor Necrosis Factor)

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