Sjögren’s syndrome - immunological mechanisms, clinical presentation and management

master's thesis

• Title (croatian): Sjögrenov sindrom - imunološki mehanizmi, kliničko očitovanje i liječenje
• Author: Julia Anastasia Raith
• Mentor: Jasenka Markeljević (mentor)
• Committee member: Jasenka Markeljević (predsjednik povjerenstva)
• Committee member: Jadranka Morović-Vergles (član povjerenstva)
• Committee member: Martina Lovrić Benčić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Internal Medicine); Zagreb
• Defense date and country: 2021-07-16, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Internal Medicine
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Clinical Immunology

Abstract

Sjögren Syndrome (SS) is a chronic systemic autoimmune disease of unknown etiology, primarily affecting the exocrine glands. Clinically, this manifests as decreased secretion of lacrimal glands and salivary glands with a feeling of dry eyes (xerophthalmia) and a dry oral mucosa (xerostomia), as well as dryness of the mucous membranes of the respiratory, gastrointestinal, and urogenital systems. In addition to the glandular manifestations, extra glandular manifestations can also occur. These involve the musculoskeletal system, the interstitium of the lungs, internal organs, and the peripheral and central nervous systems. Primarily affecting women in their 5th and 6th decades of life, Sjögren’s syndrome is the most common autoimmune disease after rheumatoid arthritis (RA). The Immunopathogenesis of SS includes a dysregulation in the interaction of endogenous and exogenous factors at systemic and local levels in genetically predisposed individuals. Sjögren’s syndrome may manifest as primary Sjögrens syndrome (pSS) or secondary if associated with other autoimmune diseases. The diagnostic procedure is based on the diagnostic criteria established by the American-European Consensus Group in 2002, which have been revised several times since then and include a variety of diagnostic tests, including the Schirmer test, serology, biopsy, and imaging. In the past, the treatment of Sjögren’s syndrome has mainly focused on relieving the symptoms and suppressing the immune system, more recently the subject of research has been immunotherapy, biological therapy, and stem cell therapy. As an alternative therapy, Chinese medicine has shown a positive effect on patients with Sjögren Syndrome.

Abstract (croatian)

Sjoegrenov sindrom (SS) je kronična sustavna autoimuna bolest (AIB) nepoznate etiologije koja primarno zahvaća egzokrine žlijezde. Klinicki dominira smanjena sekrecija suznih žlijezda i žlijezda slinovnica s osjećajem suhoće očiju (kseroftalmija) i sluznice usne šupljine (kserostomija) ali i suhoca sluznice respiratornog, gastrointestinalnog i urogenitalnog sustava. Sjogrenov sindrom se očituje glandularnim ili extraglanularnim oblikom sa zahvaćanjem koštano-mišićnog sustava, intersticija pluća, unutrašnjih organa, perifernog i središnjeg živčanog sustava. Primarno oboljevaju žene u 5. i 6. desetljeću života, a nakon reumatoidnog artritisa (RA) najčešća je autoimuna bolest. U imunopatogenezi dolazi do poremećene
regulacije u interakciji endogenih i egzogenih čimbenika. na sistemskoj i lokalnoj razini u genetski predisponiranih osoba. Sjoegrenov sindrom se može očitovati kao primarni Sjoegrenov sindrom (pSS),ili sekundarni ako je pridružen drugim AIB Imunohistološka i serološka obilježja bolesti se očituju limfocitnom infiltracijom žlijezda slinovnica sa stvaranjem ektopičnih zametnih centara, kao i prisutnost Anti-Ro i Anti-La autoantitijela u serumu. Postupak dijagnostike temelji se na dijagnostičkim kriterijima Američko-europske skupina za konsenzus 2002. godine, a koje su od tada nekoliko puta revidirane, a uključuje mnoštvo dijagnostičkih testova, poput Schirmerovog testa, serologije, biopsije i snimanja. U prošlosti
se liječenje Sjögrenovog sindroma uglavnom fokusiralo na ublažavanje simptoma i supresiju imunološkog sustava. U zadnje vrijeme, predmet istraživanja je imunoterapija, biološka terapija i terapija s matičnim stanicama, kao alternativna terapija temeljena na drevnoj kineskoj medicini za koju je opaženo Povoljan učinak na simptome u bolesnika sa Sjögrenovim sindromom.

• Keywords: Sjögren Syndrome; sicca symptoms; autoimmune disease; immunity
• Keywords (croatian): Sjögrenov sindrom; simptomi sicce; autoimuna bolest; imunitet
• Language: english
• URN:NBN: urn:nbn:hr:105:426277
• Study programme: Title: Medicine (in English language); Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2021-12-21 12:37:37