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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 targeting telomere-associated proteins in systemic sclerosis. Autoantibodies targeting telomere-associated proteins in a subset of patients with SSc are associated with short lymphocyte telomere length and lung disease. PubMed, Ann Rheum Dis, 2021 Jan 25;annrheumdis-2020-218918.

Scleroderma-specific autoantibodies embedded in immune complexes (ICs) mediate endothelial damage: an early event in the pathogenesis of systemic sclerosis. These data provide the first demonstration of the pathogenicity of ICs from scleroderma patients with different autoantibodies on the endothelium. PubMed, Arthritis Res Ther, 2020 Nov 9;22(1):265.

Putative functional pathogenic autoantibodies in systemic sclerosis. Several novel interesting autoantibodies, targeting antigens within the extracellular matrix or on the cell surface have been recently described in patients with SSc. PubMed, Eur J Rheumatol, 2020 Oct;7(Suppl 3):S181-S186.

Antigen-specific (Ab) humoral responses against Helicobacter pylori (Hp) in patients with systemic sclerosis (SSc). Anti-Hp Abs are neither more frequent nor elevated in SSc compared with healthy population and these results suggest that Hp is unlikely to be involved in the development of SSc. PubMed, Immunol Res, 04/06/2020.

A comprehensive analysis of antigen-specific antibody responses against human cytomegalovirus (HCMV) in patients with systemic sclerosis (SSc). More prevalent and/or stronger antigen-specific HCMV responses are noted in SSc compared to controls, implying a role of these viral responses in SSc development. PubMed, Clin Immunol, 07/29/2019.

Clinical Manifestation and Incidence of Cardiopulmonary Complications in Early Systemic Sclerosis (SSc) Patients with Different Antibody Profiles. It was found that the presence of SSc-specific autoantibodies was associated with a distinctive clinical presentation and cumulative internal organ involvement, even in the early phase of the disease. PubMed, J Clin Med Res, 2019 Jul;11(7):524-531. (Also see Research about Scleroderma Cardiac (Heart) Involvement)

Antibodies against specific extractable nuclear antigens (ENAs) as diagnostic and prognostic tools and inducers of a profibrotic phenotype in cultured human skin fibroblasts: are they functional? This study suggests a pathogenic role of scleroderma-specific autoantibodies to directly induce pro-fibrotic activation in human dermal fibroblasts. PubMed, Arthritis Res Ther, 2019 Jun 24;21(1):152. (Also see Fibroblasts)

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)

ACA: Anticentromere Antibodies

Characteristics of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis and Anticentriole Autoantibodies (ACA). ACA-positive SSc-PAH rapidly deteriorates, so careful hemodynamic observation and timely aggressive use of pulmonary vasodilators should be considered. PubMed, Int Heart J, 2020;61(2):413-418. (Also see Pulmonary Hypertension Symptoms and Complications)

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study. This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting. PubMed, Rheumatology (Oxford), 2020 May 1;59(5):1051-1058. (Also see Nailfold Videocapillaroscopy and Scleroderma Cardiac (Heart) Involvement)

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

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.

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

Double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane antibody could predict end-stage renal disease in ANCA-associated vasculitis (AAV): a monocentric pilot study. Double positivity for ANCA and anti-GBM at diagnosis were observed in 7.3% of AAV patients, and it could predict end-stage renal disease occurrence during follow-up in Korean patients with AAV. PubMed, Clin Rheumatol, 2020 Mar;39(3):831-840.

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

Prevalence of Angiotensin II Type 1 Receptor Antibodies (AT1RAb) in Persons With Hypertension and Relation to Blood Pressure (BP) and Medication. AT1RAb was prevalent among hypertensive adults and was associated with higher BP among persons on anangiotensin receptor blocker. PubMed, Am J Hypertens, 2020 Aug 4;33(8):734-740. (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

Smoking and systemic sclerosis (SSc): influence on microangiopathy and expression of anti-topoisomerase I antibodies (ATA) in a monocentric cohort. We observed a significant association between smoking history and positivity of ATA and we outlined the idea of a different effect of smoking on autoantibody expression between men and women. PubMed, Clin Exp Rheumatol, 03/25/2020. (Also see Preventive Care for Pulmonary Involvement)

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

Beta–2–glycoprotein–I IgA antibodies predict coronary plaque progression in rheumatoid arthritis. Anti-β2GPI IgA addition to clinical risk models improved prediction accuracy of coronary artery calcium, plaque progression and transition to extensive/obstructive disease. Seminars in Arthritis and Rheumatism, 12/17/2020. (Also see Symptoms and Complications of Rheumatoid Arthritis)

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.

Monoclonal antibodies divide overwhelmed Covid doctors. Some doctors are clamoring for the treatment, which, officials say, in some places is sitting unused. NBC Health News, 12/20/2020.

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

Anti-Ku antibodies: important points to consider. We emphasise that defining the clinical phenotype associated with anti-Ku antibodies is challenging and should be based on a systemic study considering all the aforementioned points raised. BMJ Journals, Ann Rheum Dis, 11/20/2019.

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). (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

Human Cytomegalovirus Antigen Presentation (HCMV) by HLA-DR+ NKG2C+ Adaptive Natural Killer (NK) Cells Specifically Activates Polyfunctional Effector Memory CD4+ T Lymphocytes. Our data discloses the capacity of NKG2C+ adaptive NK cells to process and present HCMV antigens to memory CD4+ cytotoxic T cells, directly regulating their response to the viral infection. PubMed, Front Immunol, 2019 Apr 3;10:687. (Also see Natural Killer Cells)

MMP-3 Antibodies

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. (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)

SCL-70 Antibodies

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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