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Antibodies in Systemic Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
ACA
AECA
AFA
ANA
ANCA and Anti-PR3
ANoA
APA/aPL
ATA
AT1R and ETAR
BPI
Caspase-3
CCP
CRP
DNA, Topoisomerase I
Eosinophilia
ESR (Sed Rate)
IgA
IgG
Immunodeficiency
Ku
Lipoprotein
Lymphocytes
MMP-3
Nucleolar
PM/Scl
PmScl and dsDNA
RNA-Polymerases
SCL-70
TAA
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)

Autoantibodies are present before the clinical diagnosis of systemic sclerosis (SSc). Our findings demonstrate that relevant seropositive autoantibodies often precede the clinical diagnosis of Ssc/no scleroderma renal crisis (SRC) and SSc/SRC. PubMed, PLoS One, 2019 Mar 26;14(3):e0214202.

Antinuclear Antibodies in Systemic Sclerosis: an Update. Autoantibodies specific for systemic sclerosis have been linked to distinct clinical features, so therefore, detecting a particular antibody type is important in predicting a possible organ involvement and prognosis. PubMed, Clin Rev Allergy Immunol, 01/03/2019.

Prevalence of auto–antibodies associated to pulmonary arterial hypertension in scleroderma – A review. The available evidence points in the direction of a strong association between auto–immune mechanisms and pulmonary hypertension in the setting of scleroderma. PubMed, Autoimmun Rev, 10/11/2018. (Also see Pulmonary Hypertension)

Autoantibodies and scleroderma phenotype define subgroups at high–risk and low–risk for cancer. Autoantibody specificity and disease subtype are biologically meaningful filters that may inform cancer risk stratification in patients with scleroderma. PubMed, Ann Rheum Dis, 2018 Aug;77(8):1179-1186. (Also see Cancer and Scleroderma)

Immune complexes (ICs) containing scleroderma (SSc)–specific autoantibodies induce a profibrotic and proinflammatory phenotype in skin fibroblasts. These data provide the first demonstration of the proinflammatory and profibrotic effects of SSc–ICs on fibroblasts, suggesting the potential pathogenicity of SSc autoantibodies. PubMed, Arthritis Res Ther, 2018 Aug 29;20(1):187. (Also see Fibroblasts)

Disease–related autoantibody (autoABs) profile in patients with systemic sclerosis (SSc). Anti-Topo I, anti-CENP, and anti-RNA pol III are the most prevalent autoAbs in SSc. PubMed, Autoimmunity, 2017 Jul 27:1-8.

New tools can potentially play key role in early diagnosis of systemic sclerosis. The results of two studies presented today at the Annual European Congress of Rheumatology (EULAR) 2017 press conference highlight the use of two new tools. News Medical, 06/14/2017. (Also see Nailfold Capillaroscopy)

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.

ACA: Anticentromere Antibodies

The limited cutaneous form of systemic sclerosis (SSc) is associated with urinary incontinence (UI): an international multicentre study. Self–reported UI is frequent in SSc and disproportionally affects the limited cutaneous form of the disease and patients positive for Anticentromere Antibodies. PubMed, Rheumatology (Oxford), 08/01/2017. (Also see Interstitial Cystitis)

Risk of Digital Vascular Events in Scleroderma Patients Who Have Both Anticentromere and Anti-Interferon-Inducible Protein 16 Antibodies. Scleroderma patients who are double–positive for antibodies recognizing CENP and IFI-16 are significantly more likely to have significant digital vascular events during the course of their disease. PubMed, Arthritis Care Res (Hoboken), 2017 Jun;69(6):922-926. (Also see Vascular Involvement and Digital Ulcers)

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)

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

Anti–endothelial cell antibodies (AECA) do not correlate with disease activity in systemic sclerosis (Ssc). AECA are not associated with the activity of SSc, although the presence of AECA might be an indicator of vascular complications development in Ssc. PubMed, Postepy Dermatol Alergol, 2018 Apr;35(2):185-191.

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

FGFR3 Antibodies in Neuropathy: What to Do With Them? Our case series highlights the variability and inconsistency in FGFR3 antibody titers through enzyme–linked immunosorbent assay testing. PubMed, J Clin Neuromuscul Dis, 2018 Sep;20(1):35-40.

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. MedicineNet.com

Circulating Anti–Nuclear Antibodies (ANA) in Systemic Sclerosis (SSc): Utility in Diagnosis and Disease Subsetting. Since these autoantibodies are specifically detected in SSc patients they are widely used in routine clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvements and prognosis. PubMed, J Nippon Med Sch, 2017;84(2):56-63.

Scleroderma ANA and Antibody Testing Basics This technical article, which has just been updated, discusses issues related to ANA and antibody testing for patients that have or might have Scleroderma and is one of the "ANA and Antibody Series" by The Scleroderma Education Project. SclerodermaInfo.org, 03/19/2017.

Case Report: Sero–Negative Systemic Sclerosis: A Rare Presentation. We report a rare case of this rare disease where patient was ANA, Antitopoisomerase I (anti-Scl-70), Anticenteromere antibody negative. PubMed, J Clin Diagn Res.

ANCA and Anti-PR3

Clinical and prognostic features of Korean patients with MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis (AAV). Clinical manifestations varied AAV categories, and neither MPO-ANCA nor PR3-ANCA significantly affected relapse of AAV. PubMed, Clin Exp Rheumatol, 2017 Mar-Apr;35 Suppl 103(1):111-118.

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.

ATA: Antithyroid Antibodies

High Prevalence of Antithyroid Antibodies (ATAs) in a New Zealand Cohort of Patients With Systemic Sclerosis (SSc). There is a higher prevalence of ATAs in SSc and Ssc– overlap syndrome compared with the general population and screening these patients for ATAs is a reasonable measure. PubMed, J Clin Rheumatol, 2018 Aug;24(5):264-271. (Also see Thyroid Diseases)

AT1R and ETAR Antibodies

Anti–Angiotensin II Type 1 Receptor and Anti–Endothelial Cell Antibodies: A Cross–Sectional Analysis of Pathological Findings in Allograft Biopsies. The data show an association between non-HLA antibodies detected in the ECXM and AT1R ELISA and microvascular injury observed in antibody mediated rejection. PubMed, Transplantation, 2017 Mar;101(3):608-615. (Also see Causes of Scleroderma: Endothelin)

BPI Antibodies

(Expired Article) 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

Anti-CCP antibodies and rheumatoid factor (RF) in systemic sclerosis: Prevalence and relationships with joint manifestations. The prevalence of RF and anti-CCP antibodies is relatively high in SSc, and joint involvement occurs frequently. PubMed, Adv Clin Exp Med, 07/19/2018. (Also see Rheumatoid Arthritis in Overlap)

HLA-DRB1 Analysis Identified a Genetically Unique Subset within Rheumatoid Arthritis (RA) and Distinct Genetic Background of Rheumatoid Factor (RF) Levels from Anticyclic Citrullinated Peptide Antibodies (ACPA). The seroconversion group was shown to have distinct genetic characteristics and the genetic architecture of RF levels is different from that of ACPA. PubMed, J Rheumatol, 02/01/2018.

CRP: C-Reactive Protein

Moderate use of alcohol is associated with lower levels of C reactive protein (CRP) but not with less severe joint inflammation: a cross-sectional study in early Rheumatoid Arthritis (RA) and healthy volunteers. Despite the fact that moderate alcohol consumption has been shown protective against RA, and our data confirm a J-shaped association of alcohol consumption with CRP levels in RA, alcohol was not associated with the severity of joint inflammation. PubMed, RMD Open, 2018 Jan 7;4(1):e000577. (Also see Causes of Rheumatoid Arthritis)

DNA: Anti-DNA and Anti-Topoisomerase I Antibodies

Survival and organ involvement in patients with limited cutaneous systemic sclerosis (LcSSc) and anti-topoisomerase-I antibodies (ATA): determined by skin subtype or auto-antibody subtype? LcSSc patients who are ATA-positive are more likely to develop dcSSc than lcSSc patients who are ATA negative. PubMed, Rheumatology (Oxford), 2016 Nov;55(11):2001-2008.

Eosinophilia

Peripheral blood eosinophilia is associated with the presence of skin ulcers in patients with systemic sclerosis (SSc). These results suggest that eosinophils are involved in the pathogenesis of vascular dysfunction of SSc. PubMed, J Dermatol, 02/04/2019. (Also see Digital Ulcers)

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.

Metastability Gap in the Phase Diagram of Monoclonal IgG Antibody. Crystallization of IgG antibodies has important applications in the fields of structural biology, biotechnology, and biopharmaceutics. PubMed, Biophys J, 2017 Oct 17;113(8):1750-1756.

Immunodeficiency

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

Single–specificity anti-Ku antibodies in an international cohort of 2140 systemic sclerosis (SSc) subjects: clinical associations. This is the largest cohort to date focusing on the prevalence and disease characteristics of single–specificity anti-Ku antibodies in subjects with SSc. PubMed, Medicine (Baltimore). 2016 Aug;95(35):e4713. (Also see Ethnicity, Race and Geographical Regions)

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. Research Gate, J Rheumatol.

Lymphocytes

CD57 in human natural killer cells and T-lymphocytes. Functional modulation of senescent CD57(pos) T-cells and mature CD57(pos) NK cells may therefore represent innovative strategies for protection against human immunological aging and/or various chronic diseases. PubMed, Cancer Immunol Immunother, 2016 Apr;65(4):441-52. (Also see Natural Killer Cells)

MMP-3 Antibodies

Matrix metalloproteinase (MMP) gene polymorphisms and susceptibility to systemic sclerosis. These results suggest that MMP polymorphisms are not associated with SSc susceptibility, although MMP1 and MMP3 variants are associated with specific SSc clinical and laboratory features. PubMed, Genet Mol Res, 2016 Dec 19;15(4).

Cysteine-rich 61 (Cyr61) participates in the pathogenesis of rheumatoid arthritis (RA) via promoting MMP-3 expression by fibroblast-like synoviocytes. This study provides new evidence that Cyr61 participates in RA pathogenesis not only as a pro–inflammatory factor but also plays a key role in bone erosion via promoting MMP-3 expression. PubMed, Mod Rheumatol, 2016 Sep 1:1-10. (Also see Treatments for Rheumatoid Arthritis)

Nucleolar Antibodies

Presence of anti-eukaryotic initiation factor-2B, anti-RuvBL1/2 and anti-synthetase antibodies in patients with anti-nuclear antibody negative systemic sclerosis (SSc). Anti-synthetase autoantibodies, and other recently discovered SSc-specific antibodies such as anti-RuvBL1/2, can also be identified in ANA-negative SSc. PubMed, Rheumatology (Oxford), 2018 Apr 1;57(4):712-717.

PM/Scl: Anti-PM/SCL-100

Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies. Anti-PM/Scl-positive patients have weaker arm abductors than hip flexors and also have the most extensive extramuscular features. PubMed, Neurology. 2018 Jun 5;90(23):e2068-e2076. (Also see Dermatomyositis and Polymyositis)

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. PubMed, Arthritis Res Ther. (Also see Ethnicity, Race and Geographical Regions)

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. (Also see Pulmonary Fibrosis Prognosis)

RNA-Polymerases Antibodies

Anti-RNPC3 (U11/U12) antibodies in systemic sclerosis are associated with moderate to severe gastrointestinal dysmotility. After adjusting for relevant covariates and potential confounders, moderate to severe GI disease was associated with anti-RNPC3 antibodies. PubMed, Arthritis Care Res (Hoboken), 09/22/2018. (Also see Dysmotility)

Circulating Anti-Nuclear Antibodies in Systemic Sclerosis: Utility in Diagnosis and Disease Subsetting. Application of circulating SSc-specific ANA measurement to clinical practice has greatly improved patient care, but utility of the autoantibody testing could be maximized by combining other clinical information, such as degree and extent of skin thickness and disease duration. PubMed, J Nippon Med Sch, 2017;84(2):56-63.

Cancers associated with systemic sclerosis (SSc) involving anti-RNA polymerase III antibodies. Anti-RNA polymerase III antibodies are useful for SSc diagnosis in patients without anti-centromere or anti-Scl70 antibodies and their presence must lead physicians to screen for associated cancer, even in the absence of clinical signs. PubMed, Ann Dermatol Venereol, 09/13/2017. (Also see Cancer)

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.

SCL-70 Antibodies

Analysis of anti-topoisomerase I antibodies (Abs) in patients with systemic sclerosis before and after autologous stem cell transplantation (aSCTrans). The presence of anti-topo489-573 Abs before aSCTrans may indicate a less favourable course after aSCTrans. PubMed, Rheumatology (Oxford), 12/10/2016. (Also see Stem Cell Transplantation)

Oral manifestations of Systemic Sclerosis and Correlation with anti-Topoisomerase I Antibodies (SCL-70). Oral symptoms have been frequent in patients with Scleroderma, SCL -70 positive but not statistically significant difference. PubMed, Med Arch. (Also see Dental Involvement)

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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