Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis.
Specimen Sources
Blood, ArterialBlood, CapillaryBlood, Central LineBlood, Venous
Specimen Types
Blood
Specimen Volume
Container
Gold Top Tube
Collection Instructions
Container/Tube: Gold Top Tube Specimen: 0.5 mL serum ( 0.3 mL min) Transport Temperature: Room Temperature
Transport Instructions
Specimen Stability
7 Days Room Temperature 14 Days Refrigerated 21 Days Frozen
Methodology
Line Blot This test was developed and its analytical performance characteristics have been determined by Quest Diagnostics. It has not been cleared or approved by FDA. This assay has been validated pursuant to the CLIA regulations and is used for clinical purposes.
Days Performed
TAT: 4-6 Days
Performing Laboratories
Quest Diagnostics Nichols Institute- San Juan Capistrano, CA
CPT
86235 x 10 84182 x 2
PDM
225705
Results
Component Name
Base Name
Common Name
External Name
SCL-70
SCL70
SCL 70
Scl-70
CENP-A
CENPA
CENP A
CENP-A
CENP-B
CENPB
CENP B
CENP-B
RP11
RP11
RP11
RP11
RP155
RP155
RP155
RP155
U1-SNRNP RNP A
U1SNRNPA
U1 SNRNP RNP A
U1-snRNP RNP A
U1-SNRNP RNP C
U1SNRNPC
U1 SNRNP RNP C
U1-snRNP RNP C
U1-SNRNP RNP-70KD
UQSNRNP70KD
U1 SNRNP RNP 70KD
U1-snRNP RNP-70kd
FIBRILLARIN
FIBRILLARIN
FIBRILLARIN
Fibrillarin
TH/TO
THTO
TH TO
Th/To
PM/SCL-100
PMSCL100
PM SCL 100
PM/Scl-100
PM/SCL-75
PMSCL75
PM SCL 75
PM/Scl-75
Result Interpretation
See Report
Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis. Some antibodies are considered highly specific for SSc, including Scl-70 (anti-topoisomerase 1) antibody, anti-centromere (CENP) antibodies, and anti-RNA polymerase III antibody and are classification criteria for disease defined by the American College of Rheumatology. Centromere antibodies are found in patients with a limited form of cutaneous systemic sclerosis, "CREST" syndrome, a complex of subcutaneous calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. RNA polymerase III antibodies target RNAP III epitopes 11 and 155. Antibodies to three major components of U1-snRNP (snRNP RNP A, U1-snRNP RNP C, U1-snRNP RNP-70kd): U1- are associated with scleroderma and inflammatory myopathy overlap syndromes. Anti-fibrillarin (anti-U3RNP) antibodies are associated with diffuse cutaneous SSc, frequent visceral involvement, and especially renal and cardiac involvement. In African-American patients, anti-fibrillarin antibodies are associated with severe pulmonary disease, pulmonary hypertension, severe small bowel involvement, and a poor prognosis. Anti-Th/To antibodies primarily bind to two proteins of the mitochondrial RNA processing (MRP) and the ribonuclease P complexes, are present in 1-13% of SSc patients, and are rarely found in other diseases. Anti-Th/To antibodies are primarily associated with localized cutaneous scleroderma, and associated with pericarditis, interstitial lung disease and a high frequency of “intrinsic pulmonary hypertension, and a poorer prognosis. Autoantibodies to PM/Scl, the human exosome complex, are found in polymyositis/scleroderma overlap syndromes. The majority of anti-PM/Scl reactivity is directed to one of two proteins: PM/Scl100 and/or PM-Scl75.
