Rheumatic Disease Serology (EN) Codes and Comments Tables

Immunology
Rheumatic Disease Serology (EN) Codes and Comments Tables
Table 1
Code
ENA
Code
ENA
A
B
C
J
L
M
N
SS-A
SS-B
PCNA
Jo1
PmSc
Sm
Neg
O
P
Q
R
S
U
Ribosomal P
Pos
Ro52
RNP
Scl-70
UPL
Table 2
1. dsDNA This antibody is highly specific for SLE.
2. RNP This antibody is seen in the majority of patients with Mixed Connective Tissue disease but
may be associated with other connective tissue disorders such as SLE.
3. SSA This antibody is associated with SLE, cutaneous lupus, primary Sjogren’s syndrome and
neonatal lupus/heart block.
4. SSA, SSB These antibodies in combination are relatively specific for primary Sjogren’s syndrome.
5. Scl-70 This antibody is seen in the diffuse variant of scleroderma.
6. Jo-1 This antibody is seen in about 30% of patients with autoimmune myositis, often associated
with interstitial lung disease.
7. The clinical significance of this antibody is unknown.
8. Absence of dsDNA does not exclude the diagnosis of SLE.
9. PM-Scl This antibody is suggestive of polymyositis/scleroderma overlap syndrome.
10. Ribosomal P This antibody is seen in SLE.
11. UPL The clinical significance of detection of a UPL is uncertain.
12. Negative for specificities detailed below.
16. Ro52 Ro52 antibodies are found in a number of autoimmune diseases including SLE, Primary
Sjogren’s Syndrome and inflammatory myositis. They may occur in many inflammatory disease
states.
17. Nucleosome Nucleosome antibodies typically segregate with systemic lupus erythematosis.
18. Sm This antibody is highly specific for SLE.
Immunology
Rheumatic Disease Serology (EN) Codes and Comments Tables
Table 3
Comment Code
1.
ACL IgG and IgM negative
A small proportion of patients with antiphospholipid antibody syndrome have undetectable
anticardiolipin antibodies. Testing for lupus anticoagulants and anti-B2GP1 is recommended if clinically
indicated.
2.
ACL IgM positive
IgM anticardiolipin antibodies are less sensitive and specific than IgG anticardiolipin antibodies for the
diagnosis of antiphospholipid syndrome, and have a weaker association with thrombotic events and/or
recurrent pregnancy loss in patients with antiphospholipid syndrome. Repeat testing to assess antibody
persistence is recommended, ideally after 12 weeks.
3.
ACL IgG and IgM positive OR IgG ACL positive alone
Moderate to high levels of IgG anticardiolipin antibodies are suggestive of the presence of the
antiphospholipid syndrome. Their presence is also associated with an increased risk of a thrombotic
event or recurrent pregnancy loss in patients with antiphospholipid syndrome. This risk appears to
increase with the level of aCL positivity . Transient and/or low level antibodies have a much lower
predictive value for antiphospholipid syndrome and may be found in a range of other inflammatory,
infectious and malignant disorders. Repeat testing to assess antibody persistence is recommended,
ideally after 12 weeks.
4.
Anti-B2GP1 pos
Positive IgG anti-B2GP1 antibodies are suggestive of the presence of the antiphospholipid syndrome,
and are associated with an increased risk of a thrombotic event or recurrent pregnancy loss in patients
with antiphospholipid syndrome. The combination of positive ACL, anti-B2GPI and lupus anticoagulant
results is reported to be associated with an increased risk of thrombosis and/or pregnancy
complications.
Repeat testing to assess antibody persistence is recommended, ideally after 12 weeks.
5.
Anti-B2GPI neg
A proportion of patients with antiphospholipid antibody syndrome are negative for antibodies to B2GP1
but are positive for anticardiolipin antibodies due to the lower sensitivity of the anti-B2GP1 assay
compared with the ACL assay. Testing for lupus anticoagulants and anticardiolipin antibodies is
recommended if clinically indicated.
6.
Anti-B2Gp1 pos and ACL pos
The combination of positive ACL, anti-B2GPI and lupus anticoagulant results in combination with
appropriate clinical features, is suggestive of the presence of the antiphospholipid syndrome. This
combination of positive assay results is associated with an increased risk of thrombosis and/or
pregnancy complications. Repeat testing to assess antibody persistence is recommended, ideally after
12 weeks.