The Spectrum of Extraglandular Manifestations in Primary Sjögren’s Syndrome

Abstract

Extraglandular manifestations (EGMs) in primary Sjogren’s syndrome (pSS) represent the clinical expression of the systemic involvement in this disease. EGMs are characterized by a wide heterogeneity; virtually any organ or system can be affected, with various degrees of dysfunction. The existing gaps of knowledge in this complex domain of extraglandular extension in pSS need to be overcome in order to increase the diagnostic accuracy of EGMs in pSS. The timely identification of EGMs, as early as from subclinical stages, can be facilitated using highly specific biomarkers, thus preventing decompensated disease and severe complications. To date, there is no general consensus on the diagnostic criteria for the wide range of extraglandular involvement in pSS, which associates important underdiagnosing of EGMs, subsequent undertreatment and progression to severe organ dysfunction in these patients. This review article presents the most recent basic and clinical science research conducted to investigate pathogenic mechanisms leading to EGMs in pSS patients. In addition, it presents the current diagnostic and treatment recommendations and the trends for future therapeutic strategies based on personalized treatment, as well as the latest research in the field of diagnostic and prognostic biomarkers for extraglandular involvement in pSS.

Zielgerichtete Immuntherapien beim primären Sjögren-Syndrom

“…Das Sjögren-Syndrom ist eine chronische autoimmune Erkrankung, die mit einer Sicca-Symptomatik als Folge einer chronisch-entzündlichen Destruktion der exokrinen Drüsen einhergeht. Es können aber auch extraglanduläre Manifestationen auftreten [1]. Weiterhin besteht bei Patient*innen mit entsprechenden Risikofaktoren, wie zum Beispiel Komplementverbrauch, Schwellung der Parotis, Nachweis hoher ANA-Titer und dem Nachweis von SSA/ Ro-Antikörpern ein erhöhtes Lymphomrisiko von etwa 5 % [2].…”

Intracranial Hypertension as a rare presentation of Sjögren's Syndrome: a case report

“…The most common neurological manifestation of SS is peripheral neuropathy, and most common CNS involvement are demyelinating lesions 6 . In this case-report, we describe an atypical CNS manifestation of SS.…”