Sindrome di Sjögren: sintomi e cause
Table of Contents
Che cos’è la sindrome di Sjögren?
La Sindrome di Sjögren è una malattia autoimmune sistemica cronica progressiva, caratterizzata da disfunzioni delle ghiandole salivari e lacrimali, con diminuita secrezione lacrimale, salivare con xerostomia (bocca secca), cheratocongiuntivite secca e ingrandimento delle parotidi. Il coinvolgimento delle ghiandole a secrezione esocrina è generalizzato; si parla di esocrinopatia autoimmune. La Sindrome di Sjögren può esistere isolatamente o in associazione con altre malattie immunopatologiche (circa il 60% dei casi), prevalentemente con l’artrite reumatoide, il lupus eritematoso sistemico e la sclerosi sistemica.
Epidemiologia
Colpisce soprattutto le donne di mezza età; l’età di incidenza presenta due picchi: tra 20 e 40 anni e dopo la menopausa.
- Primitiva: si sviluppa in persone che non hanno altre malattie autoimmuni.
- Secondaria: quando si manifestano associazioni con altre malattie autoimmuni.
Quali sono i sintomi della sindrome di Sjögren?
L’esordio è in genere insidioso; possono passare anni dalla comparsa dei primi sintomi alla diagnosi. Le manifestazioni più frequenti sono:
- Xeroftalmia: la diminuzione o l’assenza di lacrime provoca una sensazione di bruciore oculare, si può osservare una cheratocongiuntivite secca o filamentosa. I pazienti lamentano soprattutto una sensazione di corpo estraneo (come sabbia) negli occhi. Altri sintomi riferiti all’occhio comprendono ridotta lacrimazione, prurito e visione offuscata; è spesso presente iperemia congiuntivale.
- Xerostomia: la diminuzione della secrezione salivare provoca una secchezza della bocca, una difficoltà a parlare e ad inghiottire, una stomatite e carie dentali; inoltre si palpa una carotide ingrandita in un terzo dei casi.
- Poliartrite reumatoide
Altre manifestazioni
- La diminuzione della secrezione di altre ghiandole può manifestarsi con secchezza del naso (rinite), della gola, dei bronchi (tracheobronchite secca) della cute (atrofia delle ghiandole sebacee e sudoripare); si osserva spesso un ipocloridria gastrica.
- Altre manifestazioni rare: vasculite, porpora (non trombopenica), acidosi renale tubulare, nefrite interstiziale.
- Complicazione linfoproliferative:comparsa di linfomi maligni.
Diagnosi
- Anamnesi esame obiettivo: particolare attenzione a cavità orale, occhi e sistema linfatico e alla presenza di altre malattie autoimmuni
- Presenza di autoanticorpi quali anticorpi antinucleo (ANA), anticorpi anti ENA (anti-Ro/SSA e
anti-La/SSB), Fattore Reumatoide;
– aumento gli indici di infiammazione (VES e PCR), alterazioni nell’esame emocromocitometrico
(anemia e/o una diminuzione dei globuli bianchi e/o diminuzione delle piastrine), aumento delle
gammaglobuline ed una riduzione dei livelli di complemento (C3 e C4). - Test oculari per diagnosticare e quantificare la cheratongiuntivite secca. Schirmer test (<5 mm in 5 min). Rosa Bengala positivo.
Come si cura la Sindrome di Sjögren?
- Monitoraggio periodico dal dentista e dall’ oftalmologo.
- Lacrime artificiali, collirio lubrificante.
- Xerostomia: frequenti sorsi d’acqua e caramelle senza zucchero.
- Per i dolori articolari possono essere consigliate piccole dosi di cortisone, anti-infiammatori e plaquenil.
- Per la tosse secca, da secchezza tracheo-bronchiale, sono utili i mucolitici.
- Nei casi più gravi si può utilizzare la pilocarpina per stimolare la secrezione della saliva.
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