Pathogenesis and diagnosis of Sjögren’s syndrome

Abstract

Sjögren's syndrome is a common autoimmune disorder. Several genetic risk factors such as STAT-4, ILT6 and the haplotype HLA-B8/DR3 have been identified. In addition, there are environmental risk factors, possibly chronic viral infections. In the pathophysiology of Sjögren's syndrome T and B cells infiltrate the salivary and lacrimal glands. As a consequence of the destruction of glandular cells by cytotoxic T cells, production of cytokines and autoantibodies inhibiting glandular function, the production of saliva and tears is decreased. The feeling of dry eyes and mouth is frequently not noticed by the patients. Therefore, Sjögren's syndrome should also be considered when extraglandular manifestations such as vasculitis, polyneuropathy or arthritis occur, even when the patients do not complain of dry eyes and mouth. Establishing the diagnosis of Sjögren's syndrome requires verification of reduced glandular function, for example using Schirmer's test and the Saxon test. The confirmation of Sjögren's syndrome as a cause of sicca syndrome is subsequently performed by the detection of autoantibodies against Ro (SS-A) and La (SS-B) and/or by a salivary gland biopsy.

Serologic features of primary Sjögren’s syndrome: clinical and prognostic correlation

“…Genetic risk factors such as STAT-4 , IL-T6 , haplotype HLA DRw52-DR2-DR3-B8 , the heterozygosity of the HLA-DQ genetic locus [12] and, more recently, IRF5 , have been identified [13]. The association between HLA and SS is restricted to patients with anti-Ro/or anti-La antibodies [14,15].…”

Labordiagnostik bei Kollagenosen und Vaskulitiden

“…Während beim SLE der gleichzeitige Nachweis mehrerer typischer Autoantikörper die Regel ist, treten die SSc-typischen Autoantikörper bei individuellen Patienten sehr selten gemeinsam auf [15,16]. Möglicherweise handelt es sich bei den durch diese Antikörper charakterisierten Subsets um unabhängige, nur klinisch ähnliche Erkrankungen unterschiedlicher Ätiologie.…”

Labordiagnostik bei Kollagenosen und Vaskulitiden

“…Weitere beim Sjögren-Syndrom häufi g gefundene Autoantikörper sind Rheumafaktoren, die aber aufgrund ihrer mangelnden Spezifi tät hier nicht diagnostisch richtungsweisend sind. Der Vorschlag, als zusätzlichen Marker-Antikörper den Nachweis von anti-α-Fodrin einzusetzen [ 21 ] , hat sich bislang nicht allgemein durchgesetzt.…”