Περίληψη:
The presence of autoantibodies is the hallmark of systemic autoimmune
diseases. During the past 30 years, intense clinical and basic research
have dissected the clinical value of autoantibodies in many autoimmune
diseases and offered new insights into a better understanding of the
molecular and functional properties of the targeted autoantigens.
Unraveling the immunologic mechanisms underlying the autoimmune tissue
injury, provided useful conclusions on the generation of autoantibodies
and the perpetuation of the autoimmune response. Primary Sjogren’s
syndrome (pSS) is characterized by the presence of autoantibodies
binding on a vast array of organ and non-organ specific autoantigens.
The most common autoantibodies are those targeting the Ro/La RNP
complex, and they serve as disease markers, as they are included in the
European-American Diagnostic Criteria for pSS. Other autoantibodies are
associated with particular disease manifestations, such as
anti-centromere antibodies with Raynaud’s phenomenon, anti-carbonic
anhydrase II with distal renal tubular acidosis, anti-mitochondrial
antibodies with liver pathology, and cryoglobulins with the evolution to
non-Hodgkin’s lymphoma. Finally, autoantibodies against autoantigens
such as alpha- and beta-fodrin, islet cell autoantigen, poly(ADP)ribose
polymerase (PARP), NuMA, Golgins, and NOR-90 are found in a
subpopulation of SS patients without disease specificity, and their
utility remains to be elucidated. In this review, the molecular and
clinical characteristics (divided according to their clinical utility)
of the autoantigens and autoantibodies associated with pSS are
discussed.
