Autoantibodies and Autoimmunity
465
Tab. 1
Examples of clinical diagnostic speciFcity of autoantibodies.
Autoantibody specifcity
a
Molecular specifcity
Clinical association
Organ-specifc autoimmune
diseases
Antiacetylcholine receptor*
Acetylcholine receptor
Myasthenia gravis
Anti-TSH receptor*
TSH receptor
Graves’ disease
Antithyroglobulin*
Thyroglobulin
Chronic thyroiditis
Antithyroid peroxidase*
Thyroid peroxidase
Chronic thyroiditis
Antimitochondria*
Pyruvate dehydrogenase complex
Primary biliary cirrhosis
Antikeratinoctye*
Desmoplakin I homologue
Bullous pemphigoid
Antikeratinoctye*
Desmoglien
Pemphigus foliaceus
Multisystem autoimmune
diseases
Anti–double-stranded DNA*
B-form of DNA
SLE
Anti-Sm*
B, B
0
,D
,andEprote
insofU1
,U2,
U4–U6 snRNP
SLE
Anti-nRNP
70 kDa, A and C proteins of
U1-snRNP
MCTD, SLE
Anti-SS-A/Ro
60 and 52 kDa proteins associated
with hY1-Y5 RNP complex
SS, neonatal lupus, SLE
Anti-SS-B/La
47 kDa phosphoprotein complexed
with RNA polymerase III
transcripts
SS, neonatal lupus, SLE
Anti-Jo-1*
Histidyl tRNA synthetase
Polymyositis
AntiFbrillarin*
34 kDa protein of box C/D
containing snoRNP (U3, U8, etc.)
Scleroderma
Anti-RNA polymerase 1*
Subunits of RNA polymerase 1
complex
Scleroderma
Anti-DNA topoisomerase 1
(anti-Scl-70)*
100 kDa DNA topoisomerase I
Scleroderma
Anti-centromere*
Centromeric proteins CENP-A, B, C
CREST (limited Scleroderma)
Canca
Serine proteinase (proteinase 3)
Wegener’s vasculitis
a
Disease-speciFc diagnostic marker antibodies indicated by an asterisk.
Notes
: SLE: systemic lupus erythematosus; MCTD: mixed connective tissue disease; SS: Sj
..
ogren’s
syndrome; cANCA: cytoplasmic antineutrophil cytoplasmic antibody; TSH: thyroid-stimulating
hormone; CREST: c
alcinosis, R
aynaud’s phenomenon, e
sophageal dysmotility, s
clerodactyly,
t
elangiectasia.
Autoantibody specifcities may occur
at
diFFerent
Frequencies
in
a
variety
oF
diseases,
and
the
resultant
profle
consisting
oF
distinct
groups
oF
autoantibodies in diFFerent diseases can
have diagnostic use. In some cases, the
grouping
oF
autoantibody
specifcities,
such as the preponderance oF antinucleolar
autoantibodies in scleroderma (Table 1),
provides
provocative
but
as
yet
little-
understood
relationships
with
clinical
diagnosis. Unlike SLE, where a single
patient may have multiple autoantibody
specifcities to a number oF unrelated
nuclear autoantigens (e.g. DNA, Sm, SS-
A/Ro), scleroderma patients inFrequently
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