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Antibodies in Systemic Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
ANA
ANoA
ACA
DNA, Topoisomerase I
AECA
AFA
ANCA and Anti-PR3
APA/aPL
AT1R and ETAR
BPI
Caspase-3
CRP
Eosinophilia
ESR (Sed Rate)
IgA
IgG
Immunodeficiency
Ku
Lipoprotein
Lymphocytes
MMP-3
Nucleolar
PM/Scl
PmScl and dsDNA
Proteins
RNA-Polymerases
RNP
SCL-70
TAA
TNF and IL-13

Overview of Antibodies in Systemic Scleroderma

Abstract by Janet Paulmenn, ISN ArtistDozens 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)

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)
Early systemic sclerosis (SSc): marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers. Autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Arthritis Research & Therapy, 29 May 2013, 15:R63 (Also see: Nailfold Videocapillaroscopy)
Systemic sclerosis without antinuclear antibodies (ANA) or Raynaud's phenomenon (RP): a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients. PubMed, Rheumatology. 2013 Mar;52(3):560-7. (Also see: What is Scleroderma? and Raynaud's)
Autoantibodies in systemic sclerosis. With the advent of array technologies, there is now an unprecedented capability to detect multiple autoantibodies in an individual serum and this long held tenet of clinical diagnostic immunology is being reexamined. Autoimmun Rev. 2012 Jun 25.
Specific anti-nuclear antibodies (ANA) in systemic sclerosis patients with and without skin involvement: an extended methodological approach. Standard algorithms for ANA identification lack sensitivity for the detection of SSc-associated ANA and should be supplemented with additional assays, especially in a clinical environment that has particular interest in SSc. The spectrum of SSc-associated ANA differs according to the presence or absence of skin involvement. Rheumatology, 2011 50(7): 1302-1309.
Involvement of functional autoantibodies against vascular receptors in systemic sclerosis (SSc). Functional autoimmunity directed at angiotensin II type 1 receptor (AT1R) and endothelin-1 type A receptor (ETAR) is common in patients with SSc. AT1R and ETAR autoantibodies could contribute to disease pathogenesis and may serve as biomarkers for risk assessment of disease progression. Gabriela Riemekasten, Annals of Rheumatic Diseases, 15 November 2010.
Autoantibodies as predictive tools in systemic sclerosis. Strong links exist between autoantibody specificities and disease presentation and outcome, which make autoantibodies essential assessment tools in patients with SSc, although up to 11% of patients with SSc can test negative for antinuclear antibodies. SL Nihtyanova. Nat Rev Rheumatol. Feb. 2010.

Antinuclear Antibodies (ANA)

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
Specific anti-nuclear antibodies (ANA) in systemic sclerosis patients with and without skin involvement: an extended methodological approach. Standard algorithms for ANA identification lack sensitivity for the detection of SSc-associated ANA and should be supplemented with additional assays, especially in a clinical environment that has particular interest in SSc. The spectrum of SSc-associated ANA differs according to the presence or absence of skin involvement. Rheumatology, February 17, 2011.
Risk factors for ANA positivity in healthy persons. Risks for ANA positivity include female gender and organ-specific autoimmunity. Upregulation of skin-specific autoantibodies may indicate that early events in the break of tolerance take place in cutaneous structures. Arthritis Research &Therapy 2011, 13:R38.
New research finds pattern for detecting 'false-positive' ANA results. The presence of antinuclear antibodies (ANA) indicates the possibility of autoimmunity. However, studies have shown that a "false-positive" ANA test occurs in up to 13% of healthy individuals. The Medical News. 01/04/2011.

ANoA: Antinucleolar Antibodies

The clinical significance of antinucleolar antibodies (ANoA) . Neither the presence nor subtype of ANoA is specific for systemic sclerosis. Laboratory comments appended to results should reflect this fact. (PubMed) J Clin Pathol. 2008 Mar;61(3):283-6.

ACA: Anticentromere Antibodies

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.

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)
Autoantibodies to angiotensin (AT1R) and endothelin (ETAR) receptors in systemic sclerosis (SSc) induce cellular and systemic events associated with disease pathogenesis. Anti-AT1R and anti-ETAR autoantibodies could provide novel targets for therapeutic intervention in the treatment of SSc. PubMed, Arthritis Res Ther, 2014 Jan 28;16(1):R29.

CCP: Anti-Cyclic Citrullinated Peptide Antibodies

Use of antibodies recognizing cyclic citrullinated peptide in the differential diagnosis of joint involvement in systemic sclerosis. Anti-CCP antibodies can be detected in patients with SSc, but less commonly present than in adults with rheumatoid arthritis. PubMed. Clin Rheumatol. 2006 May 3. (Also see: Skeletal Involvement and Rheumatoid Arthritis)

DNA: Anti-DNA and Anti-Topoisomerase I Antibodies

Diagnostic value of anti-topoisomerase I antibodies in a large monocentric cohort. Diagnosis and risk assessment of scleroderma patients can be supported by the detection of anti-topo I antibodies. Signal intensities as obtained by LIA or ELISA can be used as a surrogate marker for fibrosis, active disease and worse prognosis. K. Hanke. Arthritis Research & Therapy 2009, 11:R28.

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, 2013 May 16.S1537-1891(13)00071-2. (Also see: Causes of Scleroderma: Endothelin)

AFA: Antifibroblast Antibodies

Identification of Target Antigens of Antifibroblast Antibodies(AFAs) in Pulmonary Arterial Hypertension (PAH). AFAs detected in patients with PAH recognize cellular targets playing key roles in cell biology and maintenance of homeostasis. B. Terrier. American Journal of Respiratory and Critical Care Medicine Vol 177. pp. 1128-1134, (2008). (Also see: PAH)

ANCA and Anti-PR3

Antineutrophil Cytoplasmic Antibody (ANCA) -Positive Crescentic Glomerulonephritis in Scleroderma (SSc) - A Different Kind of Renal Crisis. The presence of (ANCA and anti-MPO defines a subset of patients with SSc who are susceptible to crescentic glomerulonephritis. These patients may present in a manner identical to scleroderma renal crisis, yet treatment requirements differ significantly. PubMed. J Rheumatol. 2006 Jul 1. (Also see: Renal Involvement)

APA: Antiphospholipid Antibodies

Neurological manifestations of antiphospholipid syndrome. This review summarizes the latest data regarding the clinical aspects, radiological and therapeutic of major neurologic manifestations associated with antiphospholipid antibodies. Rodrigues CE. (PubMed) Eur J Clin Invest. 2010 Apr;40(4):350-9. Epub 2010 Feb 19.

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 NO. 6 JUNE 2003;30:1248-52.

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. July 28, 2009.

CRP: C-Reactive Protein

Autoantibodies Against C-Reactive Protein: Clinical Associations in Systemic Lupus Erythematosus and Primary Antiphospholipid Syndrome. We observed that the presence of these antibodies was associated with lupus nephritis and with clinical features of the APS in patients with lupus and non-lupus patients. J Rheumatol 2006;33:1980-6. (Also see: Lupus and APS)

Eosinophilia

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. 2004 Apr 6.

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. Modern Rheumatology, 27 September 2010. (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.
High IgG Signals Autoimmunity in Kids. High levels of immunoglobulin G (IgG) in children — and particularly girls — were associated with the development of autoimmune disease. Med Page Today, 11/11/2013.

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: Clinical, genetic and diagnostic insights. Anti-Ku antibodies are reported in various connective tissue diseases and the Ku complex can be responsible for a very strong autoimmune answer in autoimmune disease. It is postulated that these antibodies could be found in 55% overlap polymyositis/systemic sclerosis patients. Belizna C. (PubMed) Autoimmun Rev. 2010 Jun 4. (Also see: Polymyositis)

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)

Lymphocytes

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: Pulmonoary Hypertension, Pulmonary Fibrosis)

PM/Scl: Anti-PM/SCL-100

Anti-PM/Scl-100 and anti-RNA-polymerase III antibodies in scleroderma (SSc). At high levels, anti-PM/Scl-100 antibodies were associated with SSc, polymyositis, and dermatomyositis, L Maes. (PubMed) Clin Chim Acta. 2010 Jul 4;411(13-14):965-71. (Also See: Antibodies in Scleroderma)

PmScl and dsDNA

Antibodies against PM/Scl-75 and PM/Scl-100 are independent markers for different subsets of systemic sclerosis patients. Anti-PM/Scl antibodies are common in distinct SSc subsets and are associated with several clinical symptoms. They are mainly directed to the PM/Scl-75 antigen. K. Hanke. Arthritis Research & Therapy 2009, 11:R22.

Proteins

The human exosome and disease. The highest frequency of autoantibodies to components of the exosome complex is found in polymyositis-scleroderma (PM/Scl) overlap patients and therefore the exosome is termed PM/Scl autoantigen in the autoimmune field. Stalls RH. Adv Exp Med Biol. 2011;702:132-42. (Also see: Polymositis).

RNA-Polymerases Antibodies

Malignancies in Italian Patients with Systemic Sclerosis (SSc) Positive for Anti-RNA Polymerase III Antibodies. In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc. Paolo Airo. The Journal of Rheumatology jrheum.101144. (Also see: Cancer and Scleroderma)
Researchers Point to Possible Scleroderma-Cancer Link. Patients with a certain type of scleroderma may get cancer and scleroderma simultaneously, Johns Hopkins researchers have found, suggesting that in some diseases, autoimmunity and cancer may be linked. Those with antibodies called anti-RNA polymerase I/III had the most closely related onset of cancer and scleroderma. EurekAlert! 06/08/10. (Also see: Scleroderma and Cancer)
Association of RNA Polymerase III Antibodies with Scleroderma Renal Crisis (SRC). The authors report that the majority of the SRC patients had anti-topoisomerase I (anti-topo I) antibody (30/46, 65%), whereas a minority of patients (7/46, 15%) had anti-RNA polymerase III (RNAP). Binh Nguyen, MD. Journal of Rheumatology. Vol. 27. No. 5. May 2010. (Also see: Renal Involvement)

RNP Antibodies

Anti-hnRNP and other autoantibodies in systemic sclerosis with joint involvement. These parameters might suggest that autoantibody to both hnRNP antigens might become a non-specific but useful marker for joint involvement in SSc patients and identify SSc patients prone to develop joint damage. Sergio Generini. Rheumatology Advanced Access. May 29, 2009. (Also see: Skeletal Involvement)

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 Vol 31, Issue 4, Dec 2008, Pp 372-376.

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. 2010 Jul 2. (Also see: Tumor Necrosis Factor)
 
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