| Autoantibodies
in Scleroderma |
| This page was written
by Shelley Ensz and
has not yet been medically edited. See disclaimer. |
|
| Overview of Lab Tests |
 Specialized
blood tests such as antinuclear antibody (ANA) tests are sometimes
useful in the diagnosis or categorization of both localized
and systemic scleroderma, as well as overlapping or other autoimmune
diseases. |
| Patient information: Antinuclear antibodies (ANA). Testing for antinuclear antibodies (ANA) test is commonly used when evaluating patients who are suspected of having an autoimmune or connective tissue disorder. Antibodies are proteins that are made as part of an immune response. UpToDate Patient Information. |
| Antibody
Patterns. To learn about antibodies associated with scleroderma and other rheumatic diseases, click on "Rheumatic" in the navigation bar. Antibody Patterns. |
| Antibodies and Complement in Myopathies and Neuromuscular Disorders. Very technical medical resource. Neuromuscular Disease Center, Washington University. |
| Comprehensive Scleroderma Antibodies Panel. Introducing a comprehensive serologic assessment for Systemic Sclerosis (Scleroderma – SSc). RDL Reference Laboratory. |
| Quantitation
of autoantibodies in systemic autoimmune diseases: clinically
useful? Treatment based on changes in levels of the respective
autoantibodies only seems at present not justified, in view
of the toxicity of currently available immunosuppressive
regimens. (Sage Journals OnLine) Lupus, Vol. 15, No. 7, 397-402 (2006) (Also see: Lupus) |
| Autoimmune
Disease? Get Paid for Your Antibodies! |
| Access
Biologicals’ Specialty Antibody Donors. If you have been
diagnosed with and are being treated for any autoimmune disease,
you may be eligible to receive up to $500 per plasma donation,
as long as your antibody levels are high. For U.S. residents
who weigh at least 110 lbs and are at least 18 years of age. Access
Biologicals. Posted 06-12-07. |
| Overview of Antibody
Testing |
| Effective
use of autoantibody tests in the diagnosis of systemic autoimmune
disease. New technology enabling screening for multiple
autoantibodies may further enhance the clinical usefulness
of autoantibody testing, making it possible to diagnose autoimmune
disease in its earliest stages and to intervene before serious
end organ damage occurs. PubMed. Ann N Y Acad Sci. 2005
Jun;1050:217-28. |
| Evaluating
inflammatory joint disease: how and when can autoantibodies
help? In a patient with inflammatory joint disease, the
diagnosis can be sought by assaying a limited number of autoantibodies
according to a decision tree. PubMed. Joint Bone Spine.
2003 Dec;70(6):433-47. (Also see: Skeletal
Involvement) |
| Autoantibodies
as predictors of disease. Autoantibodies might not be
directly responsible for many of the manifestations of autoimmune
disease, but they are markers of future disease in presently
healthy individuals. PubMed. Lancet. 2004 May 8;363(9420):1544-6. |
| Laboratory
testing in autoimmune rheumatic diseases. The detection
and quantification of autoantibodies has become an important
component in the diagnosis and management of autoimmune rheumatic
diseases such as rheumatoid arthritis, systemic lupus erythematosus,
the systemic vasculitides and systemic sclerosis. Each of
these diseases is associated with a particular autoantibody
or group of autoantibodies. PubMed. Best Pract Res Clin
Rheumatol. 2004 Jun;18(3):249-69. |
| Antinuclear
Antibody Test (ANA). MedicineNet. |
| Antinuclear
antibody testing in a regional immunopathology laboratory. Antinuclear
antibody detection and characterization for systemic immune
disorders can provide the clinician with useful diagnostic
and prognostic information; it is important that the laboratory
results are relevant, timely, accurate and precise. PubMed.
Immunol Cell Biol. 2003 Oct;81(5):409-12. |
| A
Critical Evaluation of Enzyme Immunoassay Kits for Detection
of Antinuclear Autoantibodies of Defined Specificities. III.
Comparative Performance Characteristics of Academic and Manufacturers'
Laboratories. We found a disconcertingly large range
of performance characteristics in the various laboratories,
which could be quite detrimental in routine utilization of
EIA ANA kits. J Rheumatol. Volume 30: No. 11 November
2003;30:2374-81. |
| Outcome
of Positive Antinuclear Antibodies in Individuals Without
Connective Tissue Disease (CTD). Patients tended to remain
ANA positive on repeat testing. Three out of 53 patients
had developed CTD, reflecting the more sensitive but less
specific nature of ANA testing. Another common condition
associated with ANA positivity was hypothyroidism. Continued
longterm followup with larger cohorts is needed. The Journal
of Rheumatology VOLUME 30: NO. 4 APRIL 2003. |
| Laboratory
assessment in musculoskeletal disorders. Autoimmune-mediated
musculoskeletal disorders feature the presence and pathogenic
role of circulating autoantibodies and autoreactive T cells.
Determination of these autoantibodies provides crucial information
to establish the diagnosis of these diseases. PubMed.
Best Pract Res Clin Rheumatol. 2003 Jun;17(3):475-94. (Also
see: Skeletal
Involvement) |
| Antibodies in Systemic
Scleroderma |
| 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 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, and Prognosis) |
| Studies
of scleroderma at The Alfred Hospital, Melbourne. All
types have a high incidence of autoantibodies, but these
are generally not related to the severity of the disease
and do not occur in relatives or spouses. PubMed. Intern
Med J. 2006 Aug;36(8):513-8. (Also see: Types
of Scleroderma) |
| The clinical relevance of autoantibodies in scleroderma. Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Arthritis Res Ther. 2003; 5(2): 80–93. |
| 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. |
| 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. |
| 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. |
| The
Level of Circulating Immune Complexes (CIC) in Sera of Patients
with Systemic Sclerosis in Different Stages of Cutaneous
Changes. Our results suggest that patients in edematous
and indurative phases of SSc have higher values of CIC implicating
their pathogenic role in earlier phases of SSc. These results
also suggest that concentration of CIC can be used as a useful,
objective, laboratory index of disease activity in patients
with SSc but, always in conjunction with overall clinical
context of disease. J. M. Nedovic. AB0188 EULAR 2005. |
| Autoantibodies
in systemic sclerosis. Scleroderma autoantibodies are
associated with very specific demographic, clinical, organ
system, and survival features. The determination of scleroderma
autoantibodies may be helpful in assessing the prognosis,
monitoring, and treatment of scleroderma patients. PubMed.
Semin Arthritis Rheum. 2005 Aug;35(1):35-42. |
| Antinuclear
Antibody (ANA) Profiles and Organ System Involvements in
127 French and 247 U.S. Adult Systemic Sclerosis (SSc) Patients. ANA
profiles differed between these two SSc populations. Both
similarities and differences in the frequency of clinical
features can be explained, in part, by variations in autoantibody
frequencies. It is not surprising that cohorts of SSc patients
reported from different geographic regions have variations
in demographic and clinical features. Olivier C. Meyer.
1684/507. ACR 2004. |
| The
association of antinuclear antibodies with organ involvement
and survival in systemic sclerosis. AHA and a nucleolar
HEp-2 cell pattern may indicate critical organ involvement
and predict a reduced survival in SSc patients. PubMed.
Rheumatology (Oxford) 2003 Apr;42(4):534-40. |
| The
clinical relevance of autoantibodies in scleroderma. Scleroderma
is associated with several autoantibodies, each of which
is useful in the diagnosis of affected patients and in determining
their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70
antibodies are very useful in distinguishing patients with
systemic sclerosis from healthy controls, from patients with
other connective tissue disease, and from unaffected family
members. PubMed. Arthritis Res Ther 2003;5(2):80-93. |
| Non
organ based laboratory markers in systemic sclerosis. Autoantibody
measurement and general inflammatory markers were considered
the minimum requirements, with other markers needing further
evaluation. PubMed. Clin Exp Rheumatol. 2003;21(3 Suppl
29):S32-8. |
| Antibodies
in scleroderma: direct pathogenicity and phenotypic associations. Several
autoantibodies present in the sera of patients with scleroderma,
may directly contribute to disease pathogenesis. Scleroderma
also is characterized by the presence of antinuclear and
antinucleolar antibodies, which correlate with particular
phenotypes. PubMed. Curr Rheumatol Rep. 2004 Apr;6(2):156-63. |
| Autoantibodies
in systemic sclerosis and fibrosing syndromes: clinical indications
and relevance. Autoantibodies in systemic sclerosis provide
important and prognostic information and are useful in defining
clinical subsets of the disease. When used appropriately,
they can be a useful instrument in the management of scleroderma. PubMed.
Curr Opin Rheumatol. 2004 Nov;16(6):723-32. |
| Stability
of Autoantibody Repertoires in Systemic Sclerosis (SSc) Patients
Before and After High Dose Chemotherapy and Autologous Stem
Cell Transplantation. Autoantibody repertoires from SSc
patients undergoing high dose CYC and autologous stem cell
transplantation or pulse CYC therapy remains stable and independent
from response to treatment. Mathieu C. Tamby. 1662/484.
ACR 2004. (Also see: Stem
Cell Transfusions) |
| Nuclear
auto-antibodies: a useful tool for the diagnosis, the classification
and the prognosis of systemic sclerosis. Systemic sclerosis
(SSc) like other connective tissue diseases is characterized
by the occurrence of antinuclear auto-antibodies (ANA). PubMed.
Rev Med Interne. 2004 Jun;25(6):442-447. |
| The
Immunoglobulin Fraction of Scleroderma Sera Activates Pathways
of Inflammation, Fibrosis, and Apoptosis in Adult Human Dermal
Endothelial Cells. The distinct pathways of inflammation,
apoptosis, and ECM regulation induced in HDEC by SSc-sera
Ig (containing either ACA or anti-Scl70 autoantibodies) suggest
that these autoantibodies may be responsible for endothelial
pathology and the subsequent differences in clinical phenotypes
associated with centromere and topoisomerase I autoantibodies
in systemic sclerosis. S. Sohail Ahmed. ACR Conference
Oct. 2003. (Also see: Skin
Fibrosis) |
| Autoimmunity
in scleroderma: the origin, pathogenetic role, and clinical
significance of autoantibodies. Novel autoantibody and
phenotype associations discovered within the past year underscore
the clinical utility of systemic sclerosis-associated autoantibodies. PubMed.
Curr Opin Rheumatol. 2003 Nov;15(6):778-84. |
| Current
methods for the generation of human antibodies for the treatment
of autoimmune diseases. Some autoimmune diseases, such
as rheumatoid arthritis, are currently benefiting from antibody
treatment and new and existing technologies for antibody
generation could facilitate the production of effective human
antibodies as future drug candidates for other autoimmune
diseases. PubMed. Drug Discov Today. 2003 Sep 15;8(18):845-51. |
| The Clinical Relevance of Autoantibodies in Scleroderma. Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. (pdf file) Arthritis Research & Therapy Vol 5 No 2. |
| Nailfold
Videocapillaroscopy Patterns and Specific Autoantibodies
in Scleroderma. This transversal study confirms on a
large number of systemic scleroderma (SSc) patients that
the identification of distinct NVC patterns might be useful
to evaluate the progression of SSc microangiopathy. Specific
SSc autoantibodies might influence and/or modulate the rate
of progression of the SSc vascular damage, including possible
effects on the expression of the limited or diffuse skin
involvement. A. Sulli. FRI0103 EULAR 2003. (Also see: Types
of Scleroderma) |
| ADAMTS-13: Von WIllebrand
Factor |
| ADAMTS-13:
Von Willebrand factor cleaving protease (ADAMTS-13) in 123
patients with connective tissue diseases (systemic lupus
erythematosus and systemic sclerosis). Systemic connective
tissue diseases are other conditions besides TTP (thrombotic
thrombocytopenic purpura) that are associated in some instances
with low but detectable levels of ADAMTS-13. PubMed. Haematologica.
2003 Aug;88(8):914-8. |
| 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 |
| 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: SSc, Lupus, MCTD, and RA) |
| 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. |
| Long
Term Follow Up of a Cohort of Patients With Positive Antinuclear
Antibodies (ANA) and Raynaud’s Phenomenon (RP) in the
Absence of Connective Tissue Disease at Initial Evaluation. Initial
evaluation indicates that there is a subset of patients who
remain with only positive ANA and RP and do not develop a
defined CTD. Marie S. O'Brien. 1881/553 ACR 2006. (Also
see: Raynaud's) |
| Antinuclear
Antibodies and Non-SSc Autoimmune Diseases in First-Degree
Relatives of Scleroderma Patients: Data from the Scleroderma
Family Registry and DNA Repository. This is the largest
scleroderma family study to date. The results demonstrate
no significant difference between the frequencies of ANA
positivity in first-degree relatives compared to controls.
In our population, a positive ANA was frequently associated
with a diagnosed autoimmune disease. Jo Elle G. Peterson.
1556/332. ACR 2005. (Also see: Causes
of Scleroderma: Genetics) |
| Profile
of Antinuclear Antibodies (ANA) in 153 French Patients with
Systemic Sclerosis (SSc). Comparison with a Series of 247
US SSc Patients. ANA profile is very similar between
SSc patients from French and US centres with a major difference
for anti-RNP polymerase III antibodies. As anti-RNA polymerase
III antibodies have been described in SSc patients with extensive
cutaneous [3] and a high frequency of renal involvement,
clinical and epidemiological studies are in progress to explore
this difference. O. C. Meyer. FRI0317 EULAR 2004. (Also
see:Causes
of Scleroderma: Genetics) |
| Dental
amalgam as one of the risk factors in autoimmune diseases. Results
imply that, in some patients with thyroiditis, mercury from
dental amalgam can stimulate the production of antinuclear
antibodies. Dental amalgam may be a risk factor in some
patients with autoimmune disease. PubMed. Neuroendocrinol
Lett 2003 Feb-Apr;24(1/2):65-67 (Also see: Causes
of Scleroderma: Mercury, Thyroiditis,
and Dental
Involvement) |
| 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) |
| 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) |
| Morbidity
of Digital Tip Ulcerations in Scleroderma. Persistent
digital tip ulcerations and severe digital tip ulcerations
are more frequent in patients with anticentromere (ACA) and
antitopoisomerase I (TOPO) antibodies, and these patients
had significantly more functional disability than those who
had never had an ulcer. Kristin M. Ingraham. F58/578 ACR
2006. (Also see: Digital
Ulcers) |
| Clinical
and Serological Heterogeneity in Patients with Anticentromere
Antibodies. 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) |
| Anticentromere
Antibodies in Systemic Connective Inflammatory Disease. ACA
are very specific of scleroderma, in particular localized
cutaneous systemic sclerosis, but the positive predictive
value is low. ACA are detected only in 23 % of the patients
with localized cutaneous systemic sclerosis and can appear
in other systemic connective inflammatory disease. M.
Montoro Álvarez. AB0116 EULAR 2004. (Also see: Limited
Scleroderma) |
| Improvement
of skin sclerosis after occurrence of anticentromere antibody
in a patient with diffuse cutaneous systemic sclerosis. This
case suggests that the presence of ACA closely correlates
with clinical features and also suggests that clinical features
may change during the clinical course with the appearance
of another specific ANA. This case is very rare because such
a case was not reported previously. PubMed. Clin Rheumatol.
2004 Aug;23(4):345-7. Epub 2004 Apr 09. |
| Anticentromere
Antibodies in Connective Tissue Diseases. ACA are very
specific of scleroderma (95.3%), in particular of lcSSc (98.5%),
but the PPV (positive predictive value) is low (51.3%). Indeed,
only 23% of patients with lcSSc had positive ACA. Our data
show that ACA may be detected in other CTD at a lower percentage. Carlos
M. González. 1686/509. ACR 2004. (Also see Limited
Scleroderma) |
| 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 |
| 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. This information is important for managing physicians and researchers planning clinical trials involving patients with early dcSSc. (Wiley InterScience) Arthritis & Rheumatism.Volume 56, Issue 8 , Pages 2740 - 2746. July 30 2007. (Also see: Diffuse Scleroderma) |
| Morbidity
of Digital Tip Ulcerations in Scleroderma. Persistent
digital tip ulcerations and severe digital tip ulcerations
are more frequent in patients with anticentromere (ACA) and
antitopoisomerase I (TOPO) antibodies, and these patients
had significantly more functional disability than those who
had never had an ulcer. Kristin M. Ingraham. F58/578 ACR
2006. (Also see: Digital
Ulcers) |
| Unstabilized
DNA breaks in lymphocytes of patients with systemic sclerosis. 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) |
| The
Pathogenic Role of Autoantibodies to Nuclear Autoantigens
in Systemic Sclerosis (Scleroderma). A growing body of
compelling evidence is now pointing to a pathogenic role
for certain SSc aAb such as anti-topo I. J Rheumatol.
2005 September;32:1643. Editorial. |
| The
Incidence of Anti-DNA Antibodies in Patients with Anti-Topoisomerase
I Antibodies. We have shown that anti-dsDNA antibodies
were rarely detected in sera positive for anti-topo I antibodies.
The associated presence of anti-dsDNA and anti-topo I antibodies
were detected in only 3 patients and two of them had some
manifestations of systemic sclerosis (SSc). Our results confirmed
that the anti-topo I antibodies are highly specific for SSc. L.
Srejic. AB0114 EULAR 2004. |
| Anti-DNA
Topoisomerase II Alpha Autoantibodies in Localized Scleroderma. The
present results indicate that anti-topo II alpha Ab is a
major autoantibody in LSc, which is distinct from anti-topo
I Ab in SSc. Ikuko Hayakawa. ACR Conference Oct. 2003
(Also see: Localized
Scleroderma: Morphea and Linear) |
| 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. RedOrbit. 05/16/08. (Also see: PAH) |
| ANCA and Anti-PR3 |
| Antineutrophil
Cytoplasmic Antibody-Positive Crescentic Glomerulonephritis
in Scleroderma - A Different Kind of Renal Crisis. The
presence of antineutrophil cytoplasmic antibodies (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) |
| Review
of: "Antineutrophil cytoplasmic antibodies (ANCA) in
scleroderma patients: first report of a case with anti-proteinase
3 antibodies and review of the literature" The low
incidence of anti-neutrophil cytoplasmic antibodies in scleroderma
patients has been confirmed in a study in Italy. Doctor's
Guide 6-10-02. |
| 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 (Antiphospholipid Antibodies)
|
| 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) |
| Antiphospholipid
antibody in localised scleroderma. These results suggest
that aCL (antibodies against cardiolipin) and LAC (lupus
anticoagulant) are the major autoantibodies in patients with
generalised morphoea. PubMed. Ann Rheum Dis. 2003 Aug;62(8):771-4.
(Also see: Antiphospholipid
Syndrome and Morphea) |
| Autoantibodies after Stem
Cell Transplant for Scleroderma |
| 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. |
| Stability
of Autoantibody Repertoires Before and After High Dose Chemotherapy
and Autologous Stem Cell Transplantation in Systemic Sclerosis. Autoantibody
repertoires from SSc patients undergoing high dose CYC and
autologous stem cell transplantation or pulse CYC therapy
remains stable and independent from response to treatment. M.
C. Tamby. FRI0339 EULAR 2004. (Also see: Stem
Cell Transfusions) |
| 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. |
| Cancer and Scleroderma
Antibodies |
| Autoantibodies
in Patients with Systemic Sclerosis and Cancer: A Case Control
Study. From our data we can conclude that autoantibodies
are unlikely to be a risk factor for the occurrence of cancer
in patients with systemic sclerosis. C. T. Derk. FRI0074
EULAR 2003. |
| Autoantibodies
in Patients with Systemic Sclerosis and Cancer: A Case-Control
Study. In contrast to previous studies, in our case-control
study we were not able to detect a significant difference
in autoantibody frequency or patterns among SSc patients
with and without a diagnosis of cancer. These results refute
the conclusion made previously that certain autoantibodies
may represent risk factors for the development of cancer
in patients with SSc. J Rheumatol. Volume 30: NO. 9 September
2003;30:1994-6. (Also see: Associated
Diseases: Cancer) |
| 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) |
| Increased
C Reactive Protein and Aminoterminal Propeptide of Type III
Procollagen Levels are Unfavourable Predictors of Survival
in Systemic Sclerosis (Scleroderma). The increased C
reactive protein level or elevated serum aminoterminal type
III procollagen peptide are unfavourable prognostic signs
by univariate analysis. Z. Nagy. FRI0319 EULAR 2004. |
| CA11 (Anti-Carbonic Anhydrase
II) |
| Anti-carbonic
anhydrase II antibodies in systemic sclerosis: association
with lung involvement. These findings suggest both a
possible pathogenic role of anti-CAII in the development
of lung damage and a potential clinical utility as serological
marker of pulmonary involvement in SSc patients. PubMed.
Autoimmunity. 2003 Mar;36(2):85-9. (Also see: Pulmonary
Involvement) |
| 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. |
| ESR: Erythrocyte Sedimentation
Rate |
| ESR:
Erythrocyte Sedimentation Rate. There are different methods
of obtaining the SED rate--such as Westergren, Cutler, Wintrobe,
and Smith--and what is considered normal will depend on the
method used. Medline Plus. |
| 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) |
| IgG
antibodies to human cytomegalovirus late protein UL94 in
patients with systemic sclerosis. These results show
that increased levels of antibodies to the HCMV late protein
UL94 are associated with SSc and they may be a marker for
the severity of the disease. PubMed. Autoimmunity. 2004
May;37(3):241-4. |
| Abnormalities
of serum antielastin antibodies in connective tissue diseases. Abnormal
variations in elastin metabolism may be detected in several
connective tissue diseases by measuring ratios of alpha-
and tropoelastin IgG Abs as markers of elastin degradation
and synthesis. PubMed. J Investig Med 2003 Mar;51(2):104-9. |
| Factors
Associated with Disease Course in Systemic Sclerosis Patients
with Anti-Topoisomerase-I Antibodies. A rapid increase
of TSS (total skin score) and high IgG anti-Topo-I titer
are associated with increased frequency of internal organ
involvement and reduced survival. The anti-Topo-I IgG titer
may be a useful laboratory marker of SSc severity and progression. Achini
Perera. ACR Conference Oct. 2003. |
| Rheumatoid
factor isotypes and anti-agalactosyl IgG antibodies in systemic
sclerosis. IgM-, IgG-, IgA-RF and anti-AG IgG can be
serum indicators of specific clinical manifestations in SSc
patients. PubMed. Br J Dermatol. 2004 Oct;151(4):803-8. (Also
see: Antibodies) |
| Anti-lactoferrin
antibodies in patients with connective tissue diseases. Patients
with connective tissue diseases are known to develop multiple
auto-antibodies; anti-lactoferrin antibodies mainly of IgG1
isotype can also be found in patients with rheumatoid arthritis
and more often in patients with systemic lupus erythematosus. PubMed.
Folia Med (Plovdiv). 2003;45(4):25-30. (Also see: Rheumatoid
Arthritis and Lupus) |
| 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 ) |
| Lymphocytes:
Analysis of lymphocyte subpopulations in systemic sclerosis. It
has become clear that the activated cellular-immune system
plays a central role in the pathogenesis of SSc. These results
suggest that T-, B-, and NK-cell alterations may be involved
in the onset of the disease, and/or in the perpetuation of
disease, and may eventually be useful as a prognostic indicator
in selected patient subgroups. PubMed. J Investig Allergol
Clin Immunol. 2003;13(2):87-93. |
| 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) |
| Autoantigenicity
of nucleolar complexes. Autoantibodies targeting nucleolar
autoantigens (ANoA) are most frequently found in sera from
patients with systemic sclerosis (SSc, also designated scleroderma)
or with SSc overlap syndromes. PubMed. Autoimmun Rev.
2003 Oct;2(6):313-21. (Also see: Types
of Scleroderma and Overlap
Syndrome) |
| p-ANCA Antibodies |
| p-ANCA:
Correspondence: Antineutrophil Cytoplasmic Antibodies in
Patients with Systemic Sclerosis. The relationship between
atypical p-ANCA staining and antibodies to other neutrophil
antigens and well defined clinical features in scleroderma
patients needs further investigation if the significance
of this particular ANCA fluorescence pattern is to be determined.
(This item is two thirds down the linked webpage) J Rheumatol.
VOLUME 30: NO. 9 SEPTEMBER 2003. |
| PmScl and dsDNA |
| 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) |
| Patients
with Antibodies to Both PmScl and dsDNA. Antibodies to
PmScl are associated with scleroderma and myositis when dsDNA
antibodies are not present. In the presence of dsDNA antibodies,
PmScl antibodies do not appear to have clinical relevance. J
Rheumatol. November 2004;31:2169-74. |
| PM-SCL
autoantibody positive scleroderma with polymyositis (mechanic's
hand: clinical aid in the diagnosis). The recently observed
overlapping syndrome is characterized by the presence of
specific autoantibodies, HLA-type association and benign
course. A new skin symptom ('mechanic's hands') predicts
the disease, in particular the interstitial lung pathology,
which is its most relevant internal manifestation. PubMed.
J Eur Acad Dermatol Venereol. 2004 May;18(3):356-9. (Also
see: Overlap
Syndrome, Polymyositis,
and Pulmonary
Fibrosis ) |
| Proteins |
| 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. Volume 56, Issue 7, Pages 2449 - 2454.
(Also see: Polymyositis, and Overlap Syndrome) |
| RNA-Polymerases Antibodies |
| 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) |
| (RNA-polymerases)
Autoantibodies to RNA-polymerases in Italian patients with
systemic sclerosis. Anti-RNA-polymerase antibodies appear
to be less frequent in Italian patients than in Caucasian
patients from the United Kingdom or USA. This might be associated
with the lower frequency of scleroderma renal crisis. PubMed.
Clin Exp Rheumatol. 2003 May-Jun;21(3):301-6. |
| Ro/SSA Antibodies |
| Ro/SSA
autoantibodies directly bind cardiomyocytes, disturb calcium
homeostasis, and mediate congenital heart block. Congenital
heart block develops in fetuses after placental transferof
Ro/SSA autoantibodies from rheumatic mothers. The condition
is often fatal and the majority of live-born children require
a pacemaker at an early age. These findings suggest that
passive transfer of maternal p200 autoantibodies causes congenital
heart block by dysregulating Ca2+ homeostasis
and inducing death in affected cells. JEM, Volume 201,
Number 1, 11-17, 3 January 2005. (Also see: Scleroderma
and Pregnancy) |
| SCL-70 Antibodies |
| 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) |
| SCL
70: The Predictive and Diagnostic Value of Antibodies against
Topoisomerase 1 (Scl 70) in Systemic Sclerosis. High
titre of Scl 70 could suggest oesophageal abnormality but
we have not found correlation with other clinical manifestations.
The question remains whether the high titre of Scl 70 occurs
coincidentally with organ damage or does it appear earlier
and could serve as predictor of characteristic involvement
in systemic sclerosis. D. Martinovic. AB0328 EULAR 2003. |
| Combining
EL4-B5-based B-cell stimulation and phage display technology
for the successful isolation of human anti-Scl-70 autoantibody
fragments. Scl-70 is the major antigen recognised by
autoantibodies in the sera of patients with systemic sclerosis
(SSc). The autoantibodies that specifically react with Scl-70
are highly characteristic of the disease and represent valuable
markers for the diagnosis of SSc. PubMed. J Immunol Methods.
2003 Jul 1;278(1-2):249-59. |
| Nailfold
videocapillaroscopy in systemic sclerosis: diagnostic and
follow-up parameters and correlation with both specific serum
autoantibodies and subsets of skin involvement. The presence
of the Scl70 antibodies seems related to a more rapid progression
of the SS microangiopathy. On the contrary, the presence
of the ACA seems to be related to a slower progression of
the SS microvascular damage. PubMed. Reumatismo. 2004;56(1):36-45. |
| Nailfold
videocapillaroscopic (NVC) patterns and serum autoantibodies
in systemic sclerosis. NVC is an appropriate tool for
differential diagnosis between primary and secondary Raynaud's
(RP) through the clear recognition of the early NVC scleroderma
pattern. The presence of anti-Scl70 antibodies seems be related
to earlier expression of the active and late NVC patterns
of SSc microvascular damage. The presence of ACA seems to
be related to delayed expression of the late NVC pattern. PubMed.
Rheumatology (Oxford). 2004 Mar 16. (Also see: Raynaud's) |
| 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. |
| TNF
and IL-13: Serum levels of tumor necrosis factor and interleukin-13
are elevated in patients with localized scleroderma. These
results suggest that TNF and IL-13 may be associated with
the development of LSc. PubMed. Dermatology. 2003;207(2):141-7.
(Also see: Linear and Morphea) |
| Antinuclear Antibodies
and Localized Scleroderma: Generalized Morphea |
| Novel
Autoantibody to Cu/Zn Superoxide Dismutase in Patients with
Localized Scleroderma. IgG or IgM anti-Cu/Zn SOD antibody
was detected in the serum of 89% of localized scleroderma
patients, especially 100% of patients with generalized morphea,
the severest form of localized scleroderma, but was positive
only in the serum of less than 15% of patients with other
autoimmune disorders, including systemic sclerosis, systemic
lupus erythematosus, dermatomyositis, and autoimmune bullous
disorders. Minoru Hasegawa. 1687/510. ACR 2004. (Also
see: Localized
Scleroderma and Generalized
Morphea) |
| Antibodies in Localized
Scleroderma (Linear and Morphea) |
| Serum
Autoantibodies and their Clinical Associations in Patients
with Childhood and Adult Onset Linear Scleroderma. Childhood
onset Linear Scleroderma is similar to adult onset disease
in regard to the frequency of serum autoantibodies. Thaschawee
Arkachaisri. 289/289 ACR 2006. (Also see: Linear
Scleroderma) |
| Serum
Autoantibodies and their Clinical Associations in Patients
with Childhood and Adult Onset Linear Scleroderma (LScl). Childhood
onset LScl is similar to adult onset disease in regard to
the frequency of serum aAbs. Over two thirds of LScl patients
had ANA. Thaschawee Arkachaisri. 289/289 ACR 2006. (Also
see: Linear
Scleroderma) |
| Antihistone
antibodies (AHAs) in linear scleroderma variants. AHAs,
which traditionally are markers for drug-induced lupus, may
also be linked to linear scleroderma. The AHA titers may
be related to the extent of involvement as well as disease
activity. PubMed. Int J Dermatol. 2006 Nov;45(11):1296-9.
(Also see: Linear
Scleroderma) |
| Antinucleosome |
| Antinucleosome
antibody is a major autoantibody in localized scleroderma. Although
antinucleosome antibody was not specific to localized scleroderma,
its high prevalence in localized scleroderma indicates that
antinucleosome antibody is a major autoantibody in this disease. PubMed.
Br J Dermatol. 2004 Dec;151(6):1182-8. (Also see: Localized
Scleroderma) |
| Antibodies in Juvenile
Scleroderma |
| Is
Routine Testing of Anti-topoisomerase Antibody (Scl-70) and
Anti-centromere Antibody (ACA) Warranted in a Pediatric Population? This
study suggests that routine screening of Scl-70 or ACA in
the pediatric populaton with scleroderma-like disease has
little utility. The majority of patients with PSS had negative
Scl-70 and ACA serologies but evidence of clinical disease.
When present in related diseases, they had no clinical correlation
and did not alter treatment. Margalit E. Rosenkranz. ACR
Conference Oct. 2003. (Also see: Types
of Scleroderma) |
| Autoantibodies
to Beta Adrenoreceptors in Patients with Juvenile Scleroderma. Further
follow up investigations are necessary to find out whether
ABâ-1 could serve as an early predictor of scleroderma
cardiac lesions in the pre-clinical stage of the disease. M.
K. Osminina. THU0095 EULAR 2004. (Also see: Cardiac
Involvement, and Juvenile
Scleroderma) |
| Lupus and Antibodies |
| Antibodies
in Lupus. There are over 100 antibodies associated with
lupus. ISN. |
| Sjogren's Syndrome
and Antibodies |
| Sjogren's
Syndrome Diagnosis. ISN. |
| Thyroid Disease and
Antibodies |
| Thyroid
Disease and Antibodies. ISN. |
