| Autoantibodies in Scleroderma |
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| Antibodies in Systemic Scleroderma |
| This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer. |
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| Overview of Antibodies in Systemic Scleroderma |
| Plasma cytokine profiles in systemic sclerosis (SSc): associations with autoantibody subsets and clinical manifestations. Anti-centromere antibodies (ACA), anti-topoisomerase antibodies (ATA), and anti-RNA polymerase III antibodies (ARA) are three mutually exclusive SSc-associated autoantibodies that correlate with distinct clinical subsets characterized by extent of cutaneous involvement and pattern of organ involvement. Pravitt Gourh. Arthritis Research and Therapy (7th Space) 10/02/09. |
| Autoantibodies (autoAbs) in systemic sclerosis: clinical interest and diagnosis approach. Despite the diagnosis of scleroderma is mainly clinical, these different autoAbs constitute a diagnosis and prognosis tools by defining immuno-clinical subsets of the disease. B. Admou. Ann Biol Clin (Paris). 2009 May-Jun. |
| Autoantibodies in systemic sclerosis (scleroderma): clues for clinical evaluation, prognosis and pathogenesis. Autoantibodies against topoisomerase (Scl-70), centromere-associated proteins, and nucleolar antigens are important for the diagnosis of the disease and give clues for its clinical manifestations and prognosis (prognostic autoantibodies). (PubMed) Wien Med Wochenschr. 2008 Jan;158(1-2):19-28. |
| Systemic Sclerosis (SSc) Risk 60-Fold Higher in Raynaud's with SSc Antibodies, Nailfold Capillary Microscopy Changes. Positive antinuclear antibodies (ANAs), positive SSc autoantibodies, and characteristic nailfold capillary microscopy patterns can help identify patients with Raynaud's phenomenon who will progress to definite SSc. J. Kelly. Musculoskeletal Report. 12/08/09. (Also see: Raynaud’s) |
| Systemic Sclerosis Patients Have Activating Antibodies Targeting Both Endothelin Receptor Type A And Angiotensin Ii Type 1 Receptor Predicting Worse Prognosis. Anti-AT1R and anti-ETAR antibodies are a biomarker for severe disease and worse prognosis and could explain pathogenic features found in systemic sclerosis. The detection of these antibodies could identify SSc patients that might benefit from a receptor blockade or from a specific modulation of the antibody-receptor interaction. G. Riemekasten OP0162 EULAR 2007. (Also see: Causes of Scleroderma: Endothelin) |
| Is scleroderma an autoantibody mediated disease? In the past year, several provocative studies have presented evidence that autoantibodies may actually cause the vascular damage and fibrosis characteristic of systemic sclerosis. PubMed. Curr Opin Rheumatol. 2006 Nov;18(6):579-81. |
| Prognostic markers for systemic sclerosis. The risk of death is directly related to the autoantibody pattern. Other serum markers for organ involvement are under study, although their predictive performance remains to be evaluated. PubMed. Joint Bone Spine. 2006 Jun 2. |
| Antinuclear Antibodies and Systemic Scleroderma |
| Disease Subsets, Antinuclear Antibody Profile, and Clinical Features in 127 French and 247 US Adult Patients with Systemic Sclerosis. There are disease classification and SSc-related serum autoantibody differences between French and American patients with SSc. These differences help to explain variations in clinical features reported from different geographic regions. J Rheumatol 2007 January;34:104–9. (Also see: Causes of Scleroderma: Ethnicity ) |
| Anti-Th/To-Positivity in a Cohort of Patients with Idiopathic Pulmonary Fibrosis. Our findings indicate that a nucleolar-staining ANA is a common finding in patients with IPF, and that antibodies against Th/To are responsible for the majority of these. The Journal of Rheumatology. Vol 33: NO. 8 Aug 2006. |
| Scleroderma associated autoantibodies - clinical and diagnostic relevance. In more than 95% of patients, antinuclear antibodies or other autoantibodies can be detected. In about 90% of SSc patients with antinuclear antibodies, scleroderma associated autoantibodies highly specific for systemic sclerosis are found. PubMed. Z Rheumatol. 2006 Jun 21. |
| 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 |
| Scleroderma - A Simple Blood Test Could Save Lives. “Diagnosis can be helped by an anti nuclear antibody (ANA) blood test. If positive then early treatment may prevent the condition from progressing, especially if the lungs are involved. Once a diagnosis of scleroderma is made, patients can be closely followed in specialist centres so that complications are detected as soon as possible.” Professor Christopher Denton. Medical News Today. 11/06/09. (Also see: Antibodies) |
| Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients. The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). C. C. Vaz. Clinical Rheumatology. 24 April 2009 (Also see: Undifferentiated Connective Tissue Disease) |
| Clinical interpretation of antinuclear antibody tests in systemic rheumatic diseases. Autoantibody tests have been used extensively in diagnosis and follow-up of patients in rheumatology clinics. Despite a long history of routine tests and attempts to standardize such assays, there are still limitations and problems that clinicians need to be aware of. Minoru Satoh. Modern Rheumatology. March 11, 2009. |
| The Cutoff Points of Antinuclear Antibody (ANA) with High Negative and Positive Predictive Values. Analysis of 5655 Cases. The cutoff point of ANA titer 160 is appropriate to exclude SLE (Lupus), MCTD (Mixed Connective Tissue Disease), SSc (Systemic Scleroderma) in most of the clinical setting. ANA 640 or higher deserves further investigation such as disease specific autoantibodies even without characteristic clinical findings, especially after proper exams for chronic liver and thyroid diseases and RA (Rheumatoid Arthritis). Hisanori Shimizu. 1515/129. ACR 2007. (Also see: RA, Lupus,and MCTD) |
| Measurement of Antinuclear Antibodies by Multiplex Immunoassay: A Prospective, Multicenter Clinical Evaluation. These results suggest that patterns of autoantibodies detected by multiplex immunoassay testing, when analyzed by an interpretative algorithm, are useful in the evaluation of patients with CTD in situations of high disease prevalence. The Journal of Rheumatology. Vol 34 No 5 May 2007. |
| Antinucleolar Antibodies (ANoA) |
| 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. |
| Anticentromere Antibodies (ACA) |
| Centromere Antibody. Autoantibodies to centromere antigens are found in 22% of patients with progressive systemic sclerosis (PSS, or diffuse scleroderma) and in 90% of patients with the subset of scleroderma known as the CREST syndrome. Clinlab Navigator. |
| Anticentromere Antibodies (ACA) Identify Patients with Sjögren's Syndrome (SS) and Autoimmune Overlap Syndrome. The presence of ACA among patients with SS allows identification of a subset of patients with "SS overlap syndrome," who show a wide diversity of autoimmunity, encompassing but not limited to limited cutaneous sclerosis (SSc). J Rheumatol 2007;34:2253-8 (Also see: Sjogren's Syndrome and Scleroderma in Overlap) |
| Clinical and Serological Heterogeneity in Patients with Anticentromere Antibodies (ACA). ACA were positive mostly in patients with SSc with CREST features and partly in other rheumatic disorders. The high levels of ACA may be necessary for the development of CREST features, and frequent concurrence of other disease marker ANA may contribute to the development of heterogeneous clinical characteristics, including overlap syndrome, in patients with ACA. J Rheumatol. 2005 August;32:1488-94. (Also see: CREST Syndrome) |
| Anti-Cyclic Citrullinated Peptide Antibodies (CCP) |
| 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) |
| 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. |
| Clinical subsets, skin thickness progression rate (STPR), and serum antibody levels in systemic sclerosis (SSc) patients with anti-topoisomerase I antibody. Anti-topo I antibody-positive patients with SSc with a rapid STPR have reduced survival rates, primarily due to early and often fatal renal and cardiac involvement. (Wiley InterScience) Arthritis & Rheumatism.Volume 56, Issue 8, Pages 2740 - 2746. July 30 2007. (Also see: Diffuse Scleroderma) |
| Unstabilized DNA breaks in lymphocytes of patients with systemic sclerosis (SSc). Our results indicate that in SSc patients there is an interference in the protective cellular mechanisms, normally stabilizing DNA breaks. PubMed. Eur J Dermatol. 2006 May-Jun;16(3):258-61. (Also see: Causes of Scleroderma: DNA) |
| Antifibroblast Antibodies (AFAs) |
| 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) |
| Exposure to silica and risk of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Long-term silica exposure may be one of the exogenous factors contributing to ANCA production, however, silica exposure alone, without typical silicosis, was not associated with ANCA positivity. PubMed. Am J Ind Med. 2006 May 11. (Also see: Vasculitis,and Causes of Scleroderma: Silica) |
| Anti-lipoprotein Lipase Antibody (Anti-LPL) |
| 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) |
| APA, aPL (Antiphospholipid Antibodies) |
| Antiphospholipid antibodies and kidney involvement in patients with systemic sclerosis (SSc). Antiphospholipid (aPL) antibodies are often detected in systemic autoimmune diseases. Positivity for IgG aCL and IgG a-B2GPI in patients with SSc without secondary antiphospholipid syndrome seems to be connected with decrease of glomerular filtration. Ewa Wielosz Clinical Rheumatology 13 May 2009. (Also see: Kidney (Renal) Involvement) |
| Antiphospholipid Antibodies (aPL) And Vascular Endothelial Growth Factor In Systemic Lupus Erythematosus, Rheumatoid Arthritis And Systemic Sclerosis. The frequency of aPL was high in rheumatic diseases. The titres of aPL were higher in rheumatic diseases than controls and in patients with APS manifestations than without. N. A. Fathi AB0974-AHP. EULAR 2008. (Also see: Blood Tests) |
| Antiphospholipid antibody syndrome and autoimmune diseases. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases. (PubMed) Hematol Oncol Clin North Am. 2008 Feb; 22(1):53-65. (Also see: Lupus, PH,and Cardiac) |
| 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. |
| 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. |
| IgG Antibodies |
| Removing a sugar turns protective antibodies into attackers. Researcher shows that the ability of the IgG family of antibodies to efficiently trigger inflammation depends on a specific sugar molecule at the stem of the Y-shaped antibody structure. Years of prior research have confirmed that patients with autoimmune diseases have variations in the sugar patterns on their antibodies. RU Newswire. 07/04/07. (Also see: Autoimmune Diseases) |
| Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens. PubMed. Eur Respir J. 2006 Oct;28(4):799-807. (Also see: Pulmonary Hypertension) |
| Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. IgG antibodies from patients with idiopathic or SSc associated PAH express distinct reactivity profiles with macrovascular and microvascular endothelial cell antigens. PubMed. Thorax. 2005 Sep;60(9):765-772. (Also see: Pulmonary Hypertension) |
| Immunodeficiency |
| Autoimmune disease in primary antibody deficiencies. Immunodeficiency and autoimmune phenomena may occur concomitantly in the same individual. For early detection and appropriate treatment, autoimmune disease should be suspected in patients with immunodeficiency. PubMed. Allergol Immunopathol (Madr). 2005 Mar-Apr;33(2):69-73. |
| 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) |
| 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. |
| Dyspnoea in Systemic Sclerosis. Diffuse systemic sclerosis/myositis overlap is a known entity. Anti PmScl antibodies are seen in 40-50% of patients and are associated with positive HLA-DR3. Consider muscle disease as the cause for dyspnoea and dysphagia in systemic sclerosis. N. R. Priddee. SAT0228 EULAR 2006. (Also see: Pulmonary Hypertension and Dermatomyositis) |
| Anti PM-Scl antibodies. Study of prevalence and of meaning. Low prevalence and possible association with an overlap autoimmune syndrome of quite good prognosis are reported with anti PM-Scl antibodies. PubMed. Rev Med Interne. 2006 Jun 12. (Also see: Overlap Syndrome) |
| Proteins |
| Cannabinoids inhibit fibrogenesis in diffuse systemic sclerosis fibroblasts. Our preliminary findings suggest that cannabinoids are provided with an anti-fibrotic activity, thereby possibly representing a new class of agents targeting fibrosis diseases. Estrella Garcia-Gonzalez. Rheumatology. July 9, 2009. |
| Disturbed angiogenesis in systemic sclerosis(SSc) high levels of soluble endoglin (sENG). The aim of this study was to investigate, in a cross-sectional study, sENG levels together with other serum vascular markers. This study shows increased values of sENG in a large SSc cohort and a relevant association with a vascular phenotype. J. Wipff. Rheumatology Advance Access. May 13, 2008 (Also see: Vasculitis) |
| C1D is a major autoantibody target in patients with the polymyositis-scleroderma overlap syndrome. Our results demonstrate that the recently identified exosome-associated protein C1D is a major autoantigen in patients with the PM-scleroderma overlap syndrome and suggest that the use of recombinant C1D as an autoantibody target may aid in diagnosis of the PM-scleroderma overlap syndrome. Arthritis and Rheumatism. Vol 56, Issue 7, Pp 2449 - 2454. (Also see: Polymyositis, and Overlap Syndrome) |
| RNA-Polymerases Antibodies |
| A longitudinal study of anti-RNA polymerase III antibody (ARA) levels in systemic sclerosis. The pathogenic significance of ARA is unclear. Despite the very strong association of ARA with scleroderma renal crisis, we could not show the clinically significant association between absolute levels of antibody and development of internal organ complications, which makes repeated measurements of ARA levels unnecessary. However, changes in ARA level over time occur and may reflect changes in skin score. Svetlana I. Nihtyanova. Rheumatology. August 20, 2009. |
| Antibodies to RNA Polymerase III in Systemic Sclerosis Detected by ELISA. Anti-RNAP-III autoantibodies were found in nearly 20% of SSc patients but in less than 1% of controls, thus detection of this antibody is a useful marker to help diagnose SSc. As well, this antibody has prognostic utility, since it is associated with scleroderma renal crisis and the diffuse cutaneous form of SSc. J Rheumatol 2007 July;34:1528-34. (Also see: Renal Involvement, and Diffuse Scleroderma) |
| A case of renal crisis in a Korean scleroderma patient with anti-RNA polymerase I and III antibodies. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. PubMed. J Korean Med Sci. 2006 Dec;21(6):1121-3. (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 - a report from the EULAR Scleroderma Trials And Research (EUSTAR) group data base. 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) |
| Anti-scl-70. Evidence-based guidelines suggest that anti-Scl-70 antibodies are very useful in the diagnosis and clinical management of SSc patients and also to establish prognosis in these patients, particularly those with diffuse skin involvement. PubMed. Autoimmunity. 2005 Feb;38(1):65-72. |
| Relationship of Pulmonary Hypertension with Level of Skin Involvement and Autoantibody Profile in Systemic Sclerosis. Positive anti Scl-70 antibodies were significantly associated with signs of pulmonary hypertension in our patients. However, there was no correlation among extension of skin involvement and presence of ACA on one side, and signs of pulmonary hypertension and restrictive lung disease on the other. M. B. Zlatanovic. FRI0116 EULAR 2005. (Also see: Pulmonary Hypertension) |
| Tumor-associated antigens (TAAs) |
| 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. (ScienceDirect) Journal of Autoimmunity Vol 31, Issue 4, Dec 2008, Pp 372-376. |
| TNF (Tumor Necrosis Factor) and IL-13 (Interleukin-13) |
| Immune system can alter body's clock. The sudden sleepiness that accompanies the onset of many illnesses occurs when the immune system interferes with the body's circadian clocks, Swiss researchers report on Monday. ChinaDaily. 07/17/07. (Also see: Sleep and Autoimmunity) |
| Off-Label Dermatologic Uses of Anti-TNF-a Therapies. Reports suggest that anti-TNF-a therapies may be effective in the treatment of numerous inflammatory skin diseases outside their currently approved indications. PubMed. J Cutan Med Surg. 2006 May 25. (Also see: Causes of Scleroderma: Interleukins, Medications, and Skin Fibrosis) |
| The TNF-863A allele strongly associates with anticentromere antibody positivity in scleroderma. We believe these findings may have importance both for the directional pathogenesis of scleroderma progression and for the treatment of scleroderma with anti-TNF agents. PubMed. Arthritis Rheum. 2004 Feb;50(2):558-64. |
