Systemic Sclerosis 12 Ab Pnl 2 SYSSCLER

Synonyms

Allscripts (AEHR) Order Name

Systemic Sclerosis 12 Ab Pnl 2

Sunrise Clinical Manager (SCM) Order Name

Systemic Sclerosis 12 Ab Pnl 2

EPIC Order Name

Systemic Sclerosis 12 Ab Panel 2

Clinical Info

Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis.

Specimen Type

Blood

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 Laboratory

Quest Diagnostics Nichols Institute- San Juan Capistrano, CA

CPT

86235 x 10
84182 x 2

PDM

225705

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.

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