Zespół sjögrena
Etiologia i przyczyny

Zespół Sjögrena to przewlekła choroba autoimmunologiczna charakteryzująca się nieprawidłową odpowiedzią immunologiczną skierowaną przeciwko gruczołom wydzielającym łzy i ślinę, prowadzącą do suchości oczu i jamy ustnej. Etiologia jest wieloczynnikowa, obejmująca predyspozycje genetyczne (m.in. mutacje w genach RF5, STAT4, BLK, IL12A, TNIP1, CXCR5 oraz silne powiązania z allelami HLA klasy II, np. HLA-DRB1*0301 u rasy białej i HLA-DRB1*08032 u Azjatów), czynniki infekcyjne (wirusy EBV, HCV, HIV, CMV, HTLV-1, HHV-6, Coxsackie) oraz hormonalne (istotna rola estrogenów, zwłaszcza w okresie menopauzy). Mechanizmy patogenetyczne obejmują aktywację komórek nabłonkowych gruczołów ślinowych przez receptory Toll-podobne, nadekspresję genów indukowanych przez interferon oraz przewagę limfocytów B i T, co skutkuje przewlekłym stanem zapalnym i produkcją autoprzeciwciał (ANA, RF, anty-Ro/SSA, anty-La/SSB). Ryzyko rozwoju chłoniaka nieziarniczego jest 44-krotnie wyższe niż w populacji ogólnej, co podkreśla znaczenie monitorowania powikłań hematologicznych.

Etiologia Zespołu Sjögrena

Zespół Sjögrena jest przewlekłą chorobą autoimmunologiczną, w której układ odpornościowy organizmu błędnie atakuje własne komórki i tkanki, szczególnie gruczoły wydzielające łzy i ślinę, powodując charakterystyczne objawy suchości oczu i jamy ustnej.123 Dokładna przyczyna tej choroby nie jest w pełni poznana, jednak badacze sugerują, że w patogenezie odgrywają rolę czynniki genetyczne, środowiskowe, infekcyjne oraz hormonalne, które wspólnie przyczyniają się do rozwoju choroby.45

Czynniki genetyczne

Badania wykazują, że predyspozycje genetyczne odgrywają istotną rolę w rozwoju zespołu Sjögrena. Obecność specyficznych genów może zwiększać podatność na wystąpienie nieprawidłowej odpowiedzi immunologicznej po ekspozycji na czynniki środowiskowe.67 Zidentyfikowano kilka mutacji genowych potencjalnie związanych z rozwojem tej choroby, w tym RF5, STAT4, BLK, IL12A, TNIP1 i CXCR5.8

Wykazano silny związek między antygenami układu zgodności tkankowej (HLA) klasy II, szczególnie allelami HLA-DR i HLA-DQ, a zespołem Sjögrena. Korelacje te różnią się w zależności od pochodzenia etnicznego pacjenta.9 Na przykład, u osób rasy białej z Kalifornii z pierwotnym zespołem Sjögrena stwierdzono związek z haplotypem HLA-DRB1*0301-DRB3*0101-DQA1*0501-DQB1*0201, natomiast u kobiet z Japonii i Chin zaobserwowano większą częstość występowania haplotypu HLA-DRB1*08032/DQA1*0103/DQB1*0601, DRB1*08032 oraz alleli HLA-DRB1*0405-DRB4*0101-DQA1*0301-DQB1*0401.10

U pacjentów z pierwotnym zespołem Sjögrena częstość występowania HLA-DR52 szacuje się na 87%, ale jest ona również znacząco podwyższona we wtórnym zespole Sjögrena, który występuje w przebiegu reumatoidalnego zapalenia stawów lub tocznia rumieniowatego układowego.11

Warto zauważyć, że ryzyko rozwoju zespołu Sjögrena jest 44 razy wyższe u osób mających krewnych z tą chorobą, co dodatkowo wskazuje na komponent genetyczny.12 Około 12% pacjentów z zespołem Sjögrena ma jednego lub więcej krewnych z tą chorobą.13

Czynniki infekcyjne

Infekcje wirusowe i bakteryjne są uważane za potencjalne czynniki inicjujące rozwój zespołu Sjögrena u osób predysponowanych genetycznie.1415 Wirusy są najbardziej prawdopodobnymi kandydatami jako czynniki środowiskowe wyzwalające chorobę, chociaż dowód przyczynowy pozostaje nieuchwytny, a z pewnością żaden pojedynczy wirus nie został jednoznacznie zidentyfikowany.16

Wśród potencjalnych patogenów wirusowych związanych z zespołem Sjögrena wymienia się:1718

  • Wirus Epsteina-Barr (EBV)
  • Wirus zapalenia wątroby typu C (HCV)
  • HIV
  • Cytomegalowirus (CMV)
  • Ludzki wirus T-limfotropowy typu 1 (HTLV-1)
  • Ludzki herpeswirus 6 (HHV-6)
  • Wirus Coxsackie

19

Wirusy te mogą wywołać zespół Sjögrena poprzez zakażanie gruczołów ślinowych, prowadząc do uszkodzenia narządów i wywoływania autoimmunizacji poprzez molekularną mimikrę, co skutkuje uszkodzeniem tkanek.2021 W szczególności, przewlekłe zakażenie gruczołów ślinowych może prowadzić do uszkodzenia organów i zapoczątkować proces autoimmunologiczny.22

Mechanizm działania czynników infekcyjnych polega prawdopodobnie na aktywacji komórek nabłonkowych gruczołów ślinowych i receptorów Toll-podobnych (TLRs). Dzięki rozpoznawaniu wzorców związanych z patogenami, komórki te ulegają aktywacji i zaczynają produkować cytokiny, chemokiny i cząsteczki adhezyjne, co prowadzi do procesu zapalnego.23

Czynniki hormonalne

Znacząca przewaga zachorowań na zespół Sjögrena wśród kobiet (kobiety chorują około 10 razy częściej niż mężczyźni) sugeruje ważną rolę czynników hormonalnych w patogenezie choroby.2425 Uważa się, że estrogen, główny hormon żeński, może wpływać na odpowiedź immunologiczną zarówno humoralną, jak i komórkową, zmieniając podatność na zespół Sjögrena.26

Istnieją sprzeczne teorie dotyczące roli estrogenu:27

  • Niektóre badania sugerują, że estrogen chroni przed zespołem Sjögrena, a spadające poziomy tego hormonu (np. podczas menopauzy) mogą zmieniać funkcję immunologiczną i wywoływać chorobę. To czyni menopauzę głównym czynnikiem ryzyka dla zespołu Sjögrena.
  • Badania na zwierzętach wykazały, że myszy niezdolne do wytwarzania estrogenu z powodu inaktywowanych genów aromatazy doświadczały objawów autoimmunologicznych podobnych do zespołu Sjögrena.
  • Jednakże badania na ludziach przyniosły sprzeczne wyniki dotyczące roli poziomów hormonów w zespole Sjögrena.

28

W niedawnym badaniu kliniczno-kontrolnym wykazano, że pierwotny zespół Sjögrena u kobiet był związany z niższą ekspozycją na estrogeny i krótszym skumulowanym czasem cykli menstruacyjnych w porównaniu z grupą kontrolną z objawami suchości.29

Mikrochimeryzm komórek płodowych (obecność komórek limfoidalnych potomstwa w krążeniu matki) może również generować autoimmunizację u kobiet, które wcześniej były w ciąży, co stanowi kolejny potencjalny mechanizm związany z płcią.30

Czynniki środowiskowe

Poza infekcjami wirusowymi i bakteryjnymi, inne czynniki środowiskowe mogą również przyczyniać się do rozwoju zespołu Sjögrena. Badanie kohortowe populacji tajwańskiej wykazało, że narażenie na tlenek węgla (CO), tlenek azotu (NO) i metan (CH4) było związane z wyższym ryzykiem rozwoju pierwotnego zespołu Sjögrena.31

Dodatkowo, takie czynniki jak przewlekły stres, zanieczyszczenie środowiska oraz narażenie na toksyny mogą potencjalnie wyzwalać chorobę u osób predysponowanych genetycznie.3233 Zmiany epigenetyczne, w tym metylacja DNA, mikro RNA, koliste mRNA i długie niekodujące RNA, są również ściśle związane z rozwojem choroby.34

Pierwotny i wtórny zespół Sjögrena

Zespół Sjögrena klasyfikuje się jako pierwotny, gdy występuje samodzielnie bez związku z inną chorobą autoimmunologiczną, oraz jako wtórny, gdy towarzyszy innym chorobom autoimmunologicznym.3536

Wtórny zespół Sjögrena najczęściej współistnieje z:3738

  • Reumatoidalnym zapaleniem stawów (RZS)
  • Toczniem rumieniowatym układowym (SLE)
  • Twardziną
  • Zapaleniem wielomięśniowym
  • Łuszczycowym zapaleniem stawów

39

Około połowa wszystkich przypadków zespołu Sjögrena występuje wraz z inną chorobą autoimmunologiczną.40 Posiadanie jednej choroby autoimmunologicznej zwiększa ryzyko rozwoju zespołu Sjögrena, co sugeruje wspólne mechanizmy patogenetyczne.41

Mechanizmy patogenetyczne zespołu Sjögrena

Mechanizmy patogenetyczne zespołu Sjögrena nie zostały w pełni wyjaśnione, co przekłada się na niepełne zrozumienie patofizjologii tej autoimmunologicznej egzokrinopatii.42 Jednak badania rzucają światło na kluczowe procesy immunologiczne zaangażowane w rozwój choroby.

Zaburzenia odpowiedzi immunologicznej

Zespół Sjögrena charakteryzuje się ciągłą interakcją między wrodzonym a nabytym układem odpornościowym.43 W obecności podatnego podłoża genetycznego, zarówno czynniki środowiskowe, jak i hormonalne mogą wywołać infiltrację limfocytów, w szczególności limfocytów T CD4+, limfocytów B i komórek plazmatycznych, powodując dysfunkcję gruczołów w gruczołach ślinowych i łzowych.44

Kluczowe zaburzenia immunologiczne w zespole Sjögrena obejmują:45

  • Dysfunkcja komórek nabłonkowych – obecnie uważa się je za głównych graczy w patogenezie zespołu Sjögrena, do tego stopnia, że termin „zapalenie nabłonka autoimmunologiczne” jest coraz częściej używany do opisania tego stanu.
  • Nadekspresja genów indukowanych przez interferon (tzw. „sygnatura interferonowa”) została wykazana u pacjentów z zespołem Sjögrena, zarówno we krwi obwodowej, jak i w gruczołach ślinowych.
  • Zaangażowanie komórek NK (Natural Killer) w patogenezę choroby.
  • Limfocyty B są uważane za głównych graczy w patogenezie zespołu Sjögrena i jego głównym powikłaniu (mianowicie chłoniaku).
  • Limfocyty T również znacząco przyczyniają się do patogenezy zespołu Sjögrena.

W obecnym modelu patofizjologicznym zespołu Sjögrena, czynniki środowiskowe, w tym infekcje wirusowe, prowadzą do aktywacji komórek nabłonkowych gruczołów ślinowych i receptorów Toll-podobnych. Aktywacja tych komórek przez rozpoznanie wzorców związanych z patogenami powoduje produkcję cytokin, chemokin i cząsteczek adhezyjnych, co inicjuje i podtrzymuje proces zapalny.46

Dysregulacja odpowiedzi limfocytów B

Zespół Sjögrena reprezentuje złożoną, wieloaspektową aktywację układu odpornościowego, w której dysregulacja i nadaktywność limfocytów B odgrywają główną rolę.47 Komórki B są odpowiedzialne za produkcję wielu autoprzeciwciał charakterystycznych dla zespołu Sjögrena, w tym:

  • Przeciwciała przeciwjądrowe (ANA)
  • Czynnik reumatoidalny (RF)
  • Specyficzne przeciwciała Ro/SSA i La/SSB

48

Obecność tych autoprzeciwciał, szczególnie przeciwciał anty-Ro i anty-La, jest charakterystyczna dla zespołu Sjögrena. Około dwie trzecie pacjentów z pierwotnym zespołem Sjögrena ma przeciwciała anty-Ro i/lub anty-La.49

Nadmierna aktywacja limfocytów B może również przyczyniać się do zwiększonego ryzyka rozwoju chłoniaka u pacjentów z zespołem Sjögrena. Osoby z zespołem Sjögrena mają 44 razy wyższe ryzyko wystąpienia chłoniaka w porównaniu do populacji ogólnej.5051

Czynniki ryzyka zespołu Sjögrena

Oprócz genetycznych, środowiskowych i hormonalnych czynników przyczyniających się do rozwoju zespołu Sjögrena, zidentyfikowano kilka czynników ryzyka zwiększających prawdopodobieństwo zachorowania:

Wiek i płeć

Zespół Sjögrena występuje znacznie częściej u kobiet niż u mężczyzn, z proporcją około 9:1.52 Choroba najczęściej dotyka osób w wieku 40-60 lat, chociaż może wystąpić w każdym wieku, w tym u młodszych dorosłych i dzieci.5354

Menopauza jest istotnym czynnikiem ryzyka, co prawdopodobnie wiąże się ze spadkiem poziomu estrogenu.55 Bycie kobietą po menopauzie znacząco zwiększa ryzyko rozwoju zespołu Sjögrena.56

Współistniejące choroby autoimmunologiczne

Osoby z innymi chorobami autoimmunologicznymi mają zwiększone ryzyko rozwoju zespołu Sjögrena.57 Około połowa pacjentów z zespołem Sjögrena ma jednocześnie inną chorobę autoimmunologiczną, taką jak reumatoidalne zapalenie stawów, toczeń rumieniowaty układowy lub twardzina.58

Historia rodzinna

Posiadanie krewnych z chorobami autoimmunologicznymi zwiększa ryzyko rozwoju zespołu Sjögrena.59 Badania wykazują, że ryzyko jest 12 razy wyższe wśród krewnych pacjentów z zespołem Sjögrena niż w populacji ogólnej.60

Inne czynniki ryzyka

Do dodatkowych czynników ryzyka zespołu Sjögrena można zaliczyć:61

  • Przewlekły stres
  • Ekspozycja na zanieczyszczenia środowiska
  • Niedawne infekcje wirusowe

Warto zauważyć, że sam fakt posiadania czynników ryzyka nie gwarantuje rozwoju zespołu Sjögrena. Choroba prawdopodobnie wymaga zarówno genetycznej podatności, jak i co najmniej jednego czynnika wyzwalającego do pełnego rozwoju.62

Powikłania i choroby współistniejące

Zespół Sjögrena jest chorobą ogólnoustrojową, która może prowadzić do różnych powikłań i jest związana z różnymi chorobami współistniejącymi:

Powikłania choroby

Pacjenci z zespołem Sjögrena mają podwyższone ryzyko rozwoju:6364

  • Chłoniaka – ryzyko rozwoju chłoniaka nieziarniczego jest 44 razy wyższe niż w populacji ogólnej.
  • Makroglobulinemii Waldenströma – rzadkiego typu chłoniaka.
  • Zapalenia naczyń małych naczyń
  • Neuropatii – zespół Sjögrena jest jednym z najczęstszych typów autoimmunologicznych ataków na komórki nerwowe.

65

Choroby współistniejące

Zespół Sjögrena często współistnieje z innymi schorzeniami, w tym:66

  • Inne choroby autoimmunologiczne, takie jak reumatoidalne zapalenie stawów, toczeń rumieniowaty układowy, twardzina, zapalenie wielomięśniowe, choroba przeszczep przeciwko gospodarzowi, a w rzadkich przypadkach, choroba Behçeta.
  • Choroby tarczycy, szczególnie autoimmunologiczne zapalenie tarczycy Hashimoto.
  • Pierwotna marskość żółciowa wątroby
  • Przewlekłe autoimmunologiczne zapalenie wątroby

67

Objawy ogólnoustrojowej autoimmunizacji związanej z zespołem Sjögrena mogą również obejmować suchą skórę, zmęczenie, niewielką gorączkę, zaparcia, bóle mięśniowe i bóle stawów. Inne stany, które mogą wystąpić, to zjawisko Raynauda, objawy płucne, zapalenie nerek i niedoczynność tarczycy.68

Diagnostyka i różnicowanie przyczyn

Diagnostyka zespołu Sjögrena jest złożona i wymaga wykluczenia innych przyczyn objawów suchości oraz potwierdzenia autoimmunologicznego charakteru choroby.69

Różnicowanie przyczyn suchości

Najczęstszymi przyczynami suchości oczu i suchości jamy ustnej (objawów sicca) są:70

  • Starzenie się
  • Leki, w tym antyhistaminowe, niektóre rodzaje leków przeciwdepresyjnych oraz leki przeciwnadciśnieniowe
  • Wirusowe zapalenie wątroby typu C
  • Zakażenie HIV
  • Bulimia
  • Sarkoidoza
  • Choroba IgG4-zależna – szczególnie u pacjentów z powiększeniem gruczołów podżuchwowych i historią zapalenia trzustki
  • Napromieniowanie głowy i szyi
  • Cukrzyca

71

Kryteria diagnostyczne zespołu Sjögrena ewoluowały i były wielokrotnie rewidowane od 1965 roku. W 2016 roku Amerykańskie Kolegium Reumatologiczne (ACR) i Europejska Liga Przeciwreumatyczna (EULAR) opracowały i zwalidowały międzynarodowy konsensus dotyczący kryteriów klasyfikacji pierwotnego zespołu Sjögrena, które zostały ustanowione do stosowania u osób z objawami sugerującymi zespół Sjögrena.72

Należy zachować ostrożność przy rozważaniu biopsji wargi jako metody diagnostycznej. Fałszywie ujemne wyniki mogą wystąpić u pacjentów przyjmujących lub niedawno przyjmujących leki immunosupresyjne, w tym kortykosteroidy, u pacjentów, którzy mieli zespół Sjögrena wystarczająco długo, aby gruczoły ślinowe całkowicie zdegenerowały, oraz u palaczy papierosów. Fałszywie dodatnie wyniki mogą wystąpić u osób starszych, po urazie lub u osób z wirusowym zapaleniem wątroby typu C, chłoniakiem lub sarkoidozą.73

Biopsje gruczołów ślinowych są ważnym narzędziem diagnostycznym zespołu Sjögrena u osób z ujemnymi wynikami badań krwi i bez suchości jamy ustnej lub oczu, ale z innymi objawami podobnymi do zespołu Sjögrena.74

Podsumowanie etiologii zespołu Sjögrena

Etiologia zespołu Sjögrena pozostaje nie w pełni poznana, jednak obecny stan wiedzy wskazuje na złożoną interakcję między czynnikami genetycznymi, środowiskowymi, infekcyjnymi i hormonalnymi. Choroba charakteryzuje się nieprawidłową reakcją układu odpornościowego, która prowadzi do przewlekłego stanu zapalnego, uszkadzającego gruczoły wydzielające łzy i ślinę, a także potencjalnie inne narządy.75

Wzrost zrozumienia mechanizmów patogenetycznych zespołu Sjögrena może prowadzić do rozwoju bardziej skutecznych metod leczenia i potencjalnie do opracowania strategii zapobiegania chorobie u osób z grupy ryzyka. Badania nad zespołem Sjögrena są aktywnym obszarem immunologii, a wiele nowych terapii może być dostępnych w niedalekiej przyszłości.76

Badania sugerują, że rituximab (Rituxan) może być korzystny dla wielu objawów zespołu Sjögrena, co wskazuje na potencjał terapii ukierunkowanych na limfocyty B.77 Jednak leki modyfikujące przebieg choroby były stosowane w leczeniu niektórych manifestacji ogólnoustrojowych, ale poziom dowodów jest niski i potrzebne są badania na większą skalę nad najbardziej obiecującymi terapiami.78

Biorąc pod uwagę złożoność i wieloczynnikową naturę zespołu Sjögrena, zrozumienie jego etiologii ma kluczowe znaczenie dla opracowania skutecznych strategii terapeutycznych i poprawy jakości życia pacjentów dotkniętych tą przewlekłą chorobą autoimmunologiczną.79

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Sjogren’s syndrome – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/symptoms-causes/syc-20353216
    Sjogren’s syndrome is an autoimmune disorder. Your immune system mistakenly attacks your body’s own cells and tissues. […] Scientists aren’t certain why some people develop Sjogren’s syndrome. Certain genes put people at higher risk of the disorder, but it appears that a triggering mechanism such as infection with a particular virus or strain of bacteria is also necessary. […] In Sjogren’s syndrome, your immune system first targets the glands that make tears and saliva.
  • #2 Sjögren’s Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/4929-sjogrens-syndrome
    Sjgrens syndrome is an autoimmune disease that happens when your immune system damages the glands that produce and control moisture in your body. […] Sjgrens syndrome is an autoimmune disease that makes your glands produce less moisture than they should. […] Sjgrens syndrome is an autoimmune disease. Autoimmune diseases happen when your immune system mistakenly damages your body instead of protecting it. Experts arent sure what makes your immune system attack your glands and cause Sjgrens syndrome. […] Primary Sjgrens syndrome happens with no known trigger or cause. […] Other health conditions trigger secondary Sjgrens syndrome, especially other autoimmune diseases and some viral infections. […] Any autoimmune disease can trigger secondary Sjgrens syndrome. Some autoimmune diseases that are related to Sjgrens syndrome include: Rheumatoid arthritis, Psoriatic arthritis, Lupus. […] Even though studies have linked Sjgrens syndrome to other conditions, theres no guarantee youll develop it if you have these conditions.
  • #3 Sjögren’s Syndrome Causes, Symptoms, Diagnosis, Treatment | National Institute of Dental and Craniofacial Research
    https://www.nidcr.nih.gov/health-info/sjogrens-disease
    Sjgren’s disease is an autoimmune disorder in which immune cells mistakenly attack and destroy healthy cells in the glands that produce tears and saliva. […] Scientists think Sjgren’s disease is caused by a combination of genetic and environmental factors. Several genes appear to be involved in the development of Sjgren’s disease. […] Some scientists think that Sjgren’s disease may happen because of a previous infection with a virus or bacteria.
  • #4 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacterium. […] The cause of Sjgren’s disease is unknown, but it may be the influence of a combination of genetic, environmental, and other factors, as is the case with many other autoimmune disorders. […] The observation of high rates of autoimmune disorders in families with a history of Sjgren’s disease is linked with a genetic predisposition to the disease. […] Since Sjgren’s disease is associated with a high prevalence in women, sex hormones, especially estrogen, are believed to affect humoral and cell-mediated immune responses affecting susceptibility to the disease. […] Microchimerism of fetal cells (offspring lymphoid cells in maternal circulation) may generate autoimmunity in women who have previously been pregnant.
  • #5 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
    https://www.mdpi.com/2077-0383/9/7/2299
    Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. […] The aetiology of the “autoimmune epithelitis” still remains unknown, but certainly involves genetic, environmental and hormonal factors. […] SS is considered as a multifactorial process originating from the interaction between genetic factors and exogenous and endogenous agents able to trigger an abnormal autoimmune response mediated in particular by T and B lymphocytes. […] In SS pathogenesis, a trigger phase is induced by environmental factors such as viral infections combined with genetic predisposition, epigenetic factors and sex hormonal regulation.
  • #6 Sjögren syndrome: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/sjogren-syndrome/
    Sjögren syndrome is thought to result from a combination of genetic and environmental factors; however, no associations between specific genetic changes and the development of Sjögren syndrome have been confirmed. […] Researchers believe that variations in many genes affect the risk of developing Sjögren syndrome, but that development of the condition may be triggered by something in the environment. […] In particular, viral or bacterial infections, which activate the immune system, may have the potential to encourage the development of Sjögren syndrome in susceptible individuals. […] The genetic variations that increase susceptibility may reduce the body’s ability to turn off the immune response when it is no longer needed.
  • #7 Sjögren’s Disease: Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/secondary-sjogrens-syndrome-4692187
    Sjgren’s disease is one of the most common autoimmune disorders affecting as many as four million people in the United States. […] The cause of Sjgren’s disease is unknown. For reasons not entirely clear, the immune system will suddenly attack moisture-producing glands and tissues with chronic inflammation. […] Genetics are thought to predispose certain people to Sjgren’s, with six gene mutations currently identified: RF5, STAT4, BLK, IL12A, TNIP1, and CXCR5. […] Having these gene mutations does not mean you will automatically get Sjgren’s disease; other factors are thought to „trigger” the disease, including infection with the Epstein-Barr virus and hepatitis C virus and hormonal abnormalities like estrogen deficiency (which starts to decline steeply in females after the age of 40). […] Having another autoimmune disease also increases your risk of Sjgren’s disease.
  • #8 Sjögren’s Disease: Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/secondary-sjogrens-syndrome-4692187
    Sjgren’s disease is one of the most common autoimmune disorders affecting as many as four million people in the United States. […] The cause of Sjgren’s disease is unknown. For reasons not entirely clear, the immune system will suddenly attack moisture-producing glands and tissues with chronic inflammation. […] Genetics are thought to predispose certain people to Sjgren’s, with six gene mutations currently identified: RF5, STAT4, BLK, IL12A, TNIP1, and CXCR5. […] Having these gene mutations does not mean you will automatically get Sjgren’s disease; other factors are thought to „trigger” the disease, including infection with the Epstein-Barr virus and hepatitis C virus and hormonal abnormalities like estrogen deficiency (which starts to decline steeply in females after the age of 40). […] Having another autoimmune disease also increases your risk of Sjgren’s disease.
  • #9 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Certain viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus are proposed as possible inducers of Sjgrens syndrome given that they may cause persistent infection of the salivary glands, leading to organ damage. […] Alleles in the major histocompatibility complex (MHC) class II gene region, particularly the human leukocyte antigen (HLA)-DR and HLA-DQ alleles, are linked to Sjgrens syndrome. These gene correlations differ depending on a patients ethnic origin. For example, the haplotype HLA-DRB1*0301-DRB3*0101-DQA1*0501-DQB1*0201 was found to be associated with the development of primary Sjgrens syndrome in White individuals from California. Similarly, women from Japan and China had a higher prevalence of the haplotype HLA-DRB1*08032/DQA1*0103/DQB1*0601, DRB1*08032, and HLA-DRB1*0405-DRB4*0101-DQA1*0301-DQB1*0401 alleles associated with Sjgrens syndrome.
  • #10 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Certain viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus are proposed as possible inducers of Sjgrens syndrome given that they may cause persistent infection of the salivary glands, leading to organ damage. […] Alleles in the major histocompatibility complex (MHC) class II gene region, particularly the human leukocyte antigen (HLA)-DR and HLA-DQ alleles, are linked to Sjgrens syndrome. These gene correlations differ depending on a patients ethnic origin. For example, the haplotype HLA-DRB1*0301-DRB3*0101-DQA1*0501-DQB1*0201 was found to be associated with the development of primary Sjgrens syndrome in White individuals from California. Similarly, women from Japan and China had a higher prevalence of the haplotype HLA-DRB1*08032/DQA1*0103/DQB1*0601, DRB1*08032, and HLA-DRB1*0405-DRB4*0101-DQA1*0301-DQB1*0401 alleles associated with Sjgrens syndrome.
  • #11 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    The etiology of Sjgren syndrome is not well understood. The presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules and the identification of inherited susceptibility markers suggest that environmental or endogenous antigens trigger a self-perpetuating inflammatory response in susceptible individuals. In addition, the continuing presence of active interferon pathways in Sjgren syndrome suggests ongoing activation of the innate immune system. […] Together, these findings suggest an ongoing interaction between the innate and acquired immune systems in Sjgren syndrome. […] The frequency of HLA-DR52 in patients with primary Sjgren syndrome is estimated to be 87%, but it is also significantly increased in secondary Sjgren syndrome that occurs with rheumatoid arthritis or systemic lupus erythematosus.
  • #12 Causes of Sjögren’s Syndrome | Sjogren’s Syndrome NewsEnvelope icon
    https://sjogrenssyndromenews.com/causes-of-sjogrens-syndrome/
    Sjögren’s syndrome is an autoimmune disorder primarily characterized by inflammation of the tear and salivary glands, leading to the condition’s signature symptoms of dry eyes and mouth. […] Experts don’t know for sure what causes Sjögren’s syndrome, but some believe that a combination of genetics and an infection causes the immune system to start mistakenly attacking the body’s own cells. […] It is thought that Sjögren’s arises from a complex mix of genetic and environmental factors. […] The disease is 12 times more common among relatives of Sjögren’s patients than in the general population, pointing to a genetic component. […] While certain genes increase a person’s risk for Sjögren’s syndrome, other factors, such as viral or bacterial infections, appear necessary for sending the immune system into overdrive. […] Those at risk also include people who already have an autoimmune disease, such as rheumatoid arthritis or lupus, which might share some disease mechanisms with Sjögren’s.
  • #13 Sjögrens
    https://www.arthritis.org/diseases/sjogrens-syndrome
    Evidence also suggests that a person has a higher risk for Sjgrens if a relative has it. In fact, about 12% of people with it have one or more relatives with the disease. […] Although there isnt yet a concrete answer as to why Sjgrens affects women more often than men, researchers believe the hormone estrogen might play a role. A major risk factor for developing the disease is being post-menopausal, when estrogen levels in the body drop.
  • #14 Sjögren’s syndrome
    https://www.nhs.uk/conditions/sjogrens-syndrome/
    Sjgren’s syndrome happens when your immune system attacks the glands that produce fluid. […] It’s not clear why this happens, but it may be: linked to certain genes, related to certain hormones, triggered by a viral or bacterial infection. […] More women get Sjgren’s syndrome than men.
  • #15 Sjogren’s Syndrome | Dry Mouth | Dry Eyes | MedlinePlus
    https://medlineplus.gov/sjogrenssyndrome.html
    Sjogren’s syndrome, also called Sjogren’s disease, is a chronic (long-lasting) autoimmune disease. […] Researchers don’t know for sure what causes the immune system to do this. But they think that it is caused by a combination of genetic and environmental factors. […] Some researchers also think that the disease may be triggered by something in the environment. For example, they think that one possible trigger could be a previous infection with a virus or bacteria.
  • #16 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Viruses are viable candidates as environmental triggers, although proof of causation has remained elusive, and certainly no single virus has been implicated. Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role. […] Damage and/or cell death due to viral infection or other causes may provide triggering antigens to Toll-like receptors in or on dendritic or epithelial cells, which, by recognizing pathogen-associated patterns, are activated and begin producing cytokines, chemokines, and adhesion molecules. […] A population-based cohort study of Taiwanese residents found that exposure to carbon monoxide (CO), nitric oxide (NO), and methane (CH4) was associated with a higher risk of developing primary Sjgren syndrome.
  • #17 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Sjogrens syndrome is a chronic autoimmune condition caused by lymphocytic infiltration into the exocrine glands and other organs. […] There are several factors thought to cause Sjgrens syndrome including genetic, environmental, and hormonal factors. The human leukocyte antigen (HLA)-DR and HLA-DQ alleles in the major histocompatibility complex (MHC) class II gene region, have been linked to Sjgrens syndrome, and are typically dependent on the ethnicity of the individual. Viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus could also cause Sjgrens syndrome. […] Infectious agents, particularly viruses, may play a role in Sjogrens syndrome etiology. Viruses are hypothesized to increase autoantibody formation by molecular mimicry, which results in tissue damage.
  • #18 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Certain viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus are proposed as possible inducers of Sjgrens syndrome given that they may cause persistent infection of the salivary glands, leading to organ damage. […] Alleles in the major histocompatibility complex (MHC) class II gene region, particularly the human leukocyte antigen (HLA)-DR and HLA-DQ alleles, are linked to Sjgrens syndrome. These gene correlations differ depending on a patients ethnic origin. For example, the haplotype HLA-DRB1*0301-DRB3*0101-DQA1*0501-DQB1*0201 was found to be associated with the development of primary Sjgrens syndrome in White individuals from California. Similarly, women from Japan and China had a higher prevalence of the haplotype HLA-DRB1*08032/DQA1*0103/DQB1*0601, DRB1*08032, and HLA-DRB1*0405-DRB4*0101-DQA1*0301-DQB1*0401 alleles associated with Sjgrens syndrome.
  • #19 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
    https://www.mdpi.com/2077-0383/9/7/2299
    According to the current physiopathogenic model of SS, environmental factors including viral infection lead to SGEC and Toll Like Receptors (TLRs) activation. […] Primary viruses involved in SS induction include Epstein–Barr (EBV) viruses, Human T-lymphotropic virus type I (HTLVI), hepatitis virus C (HCV) and coxsackievirus. […] Genetic predisposition to SS plays a role in the trigger phase of the disease. […] A strong association between human leucocyte antigen (HLA)-DR and HLA-DQ alleles belonging to the group of major histocompatibility genes (MHC) class II genes and SS was observed throughout different populations including Caucasian, Japanese and Chinese populations. […] While gene polymorphism plays an indisputable role in the triggering phase of SS, the individual contribution of each genetic factor remains to be assessed.
  • #20 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Sjogrens syndrome is a chronic autoimmune condition caused by lymphocytic infiltration into the exocrine glands and other organs. […] There are several factors thought to cause Sjgrens syndrome including genetic, environmental, and hormonal factors. The human leukocyte antigen (HLA)-DR and HLA-DQ alleles in the major histocompatibility complex (MHC) class II gene region, have been linked to Sjgrens syndrome, and are typically dependent on the ethnicity of the individual. Viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus could also cause Sjgrens syndrome. […] Infectious agents, particularly viruses, may play a role in Sjogrens syndrome etiology. Viruses are hypothesized to increase autoantibody formation by molecular mimicry, which results in tissue damage.
  • #21 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    Viral proteins, engulfed molecules, or degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of Sjgren’s disease development. […] The pathogenetic mechanisms of Sjgren’s disease have not been fully elucidated, resulting in the lack of pathophysiology knowledge of the management of this autoimmune exocrinopathy. […] In the presence of a susceptible genetic background, both environmental and hormonal factors are thought capable of triggering the infiltration of lymphocytes, specifically CD4+ T cells, B cells, and plasma cells, causing glandular dysfunction in the salivary and lacrimal glands.
  • #22 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Certain viruses such as HCV, HIV, Epstein-Barr virus, cytomegalovirus, human T-lymphotropic virus type 1, human herpesvirus 6, and coxsackievirus are proposed as possible inducers of Sjgrens syndrome given that they may cause persistent infection of the salivary glands, leading to organ damage. […] Alleles in the major histocompatibility complex (MHC) class II gene region, particularly the human leukocyte antigen (HLA)-DR and HLA-DQ alleles, are linked to Sjgrens syndrome. These gene correlations differ depending on a patients ethnic origin. For example, the haplotype HLA-DRB1*0301-DRB3*0101-DQA1*0501-DQB1*0201 was found to be associated with the development of primary Sjgrens syndrome in White individuals from California. Similarly, women from Japan and China had a higher prevalence of the haplotype HLA-DRB1*08032/DQA1*0103/DQB1*0601, DRB1*08032, and HLA-DRB1*0405-DRB4*0101-DQA1*0301-DQB1*0401 alleles associated with Sjgrens syndrome.
  • #23 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Viruses are viable candidates as environmental triggers, although proof of causation has remained elusive, and certainly no single virus has been implicated. Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role. […] Damage and/or cell death due to viral infection or other causes may provide triggering antigens to Toll-like receptors in or on dendritic or epithelial cells, which, by recognizing pathogen-associated patterns, are activated and begin producing cytokines, chemokines, and adhesion molecules. […] A population-based cohort study of Taiwanese residents found that exposure to carbon monoxide (CO), nitric oxide (NO), and methane (CH4) was associated with a higher risk of developing primary Sjgren syndrome.
  • #24 Sjogren’s Syndrome: Causes, Symptoms, and Treatment Options
    https://www.webmd.com/a-to-z-guides/sjogrens-syndrome
    Doctors don’t know the exact cause. You may have genes that put you at risk. An infection with a bacteria or virus may be a trigger that sets the disease in motion. […] For example, let’s say you have a defective gene that’s linked to Sjogren’s and then you get an infection. Your immune system swings into action. […] White blood cells normally lead the attack against the germs. But because of your faulty gene, your white blood cells target healthy cells in the glands that make saliva and tears. There’s no letup in the fight, so your symptoms will keep going unless you get treatment. […] Some other things can make you more likely to have Sjogren’s, including: Age. Sjogren’s usually affects people over 40, but younger adults and children can get it, too. […] Sex. Women are 10 times more likely to have Sjogren’s than men. […] Other autoimmune issues. Nearly half of all people who have Sjogren’s also have another autoimmune condition, like lupus or rheumatoid arthritis.
  • #25 Sjögren disease | Causes, symptoms, treatments
    https://versusarthritis.org/about-arthritis/conditions/sjoegren-disease/
    In Sjgren disease, the bodys immune system produces inflammation that damages the tissues and nerve signals to certain glands in the body. […] The inflammation mainly attacks glands that produce fluids, such as the lacrimal glands behind the eyelids, and the salivary glands in the mouth. This reduces the amount of saliva, tears and other fluids the glands produce. […] We dont know what causes the immune system to start attacking the body, but its thought it could be partly due to the genes we inherit from our parents. However, this doesnt mean that children of people with Sjgren disease will automatically get the condition. […] Its also thought that the female hormone oestrogen could be linked to Sjgren disease, as more women get the condition than men.
  • #26 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacterium. […] The cause of Sjgren’s disease is unknown, but it may be the influence of a combination of genetic, environmental, and other factors, as is the case with many other autoimmune disorders. […] The observation of high rates of autoimmune disorders in families with a history of Sjgren’s disease is linked with a genetic predisposition to the disease. […] Since Sjgren’s disease is associated with a high prevalence in women, sex hormones, especially estrogen, are believed to affect humoral and cell-mediated immune responses affecting susceptibility to the disease. […] Microchimerism of fetal cells (offspring lymphoid cells in maternal circulation) may generate autoimmunity in women who have previously been pregnant.
  • #27 Sjogren’s syndrome: Symptoms, causes, and treatment
    https://www.medicalnewstoday.com/articles/233747
    Sjogrens syndrome happens when white blood cells infiltrate saliva glands, tear glands, and other exocrine tissues, decreasing tear and saliva production. […] The causes of Sjogrens remain largely unknown. […] However, researchers believe a viral or bacterial infection may trigger the condition, but that the underlying cause is primarily genetic and environmental. […] As the majority of people who develop Sjogrens are female, one theory is that estrogen, a female hormone, plays an important role. […] Some studies theorize that estrogen protects against Sjogrens syndrome, and falling levels of the hormone may alter immune function and trigger the condition. This means a major risk factor for Sjogrens syndrome is menopause.
  • #28 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Reduced estrogen levels have been linked to Sjogrens syndrome-like symptoms in animal studies. In one animal study showed that mice that were unable to generate estrogen due to inactivated aromatase genes experienced autoimmune symptoms similar to those of Sjogrens syndrome. Human research, on the other hand, has yielded conflicting outcomes regarding the role of hormone levels in Sjogrens syndrome.
  • #29 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
    https://www.mdpi.com/2077-0383/9/7/2299
    The epigenetic processes more closely linked to the disease are DNA methylation, miRNA, circular mRNA and long non-coding RNA function. […] The expression analysis of lncRNAs in SS patients has shown lncRNAs LINC00657, LINC00511 and CTD-2020K17.1 potentially associated with the disease. […] The strong predisposition of women to develop SS clearly demonstrates the role of sex hormones as a risk factor of the disease. […] In a recent case-control study, pSS in women was associated with lower oestrogen exposure and lower cumulative menstrual cycling time compared to sicca controls. […] The role of HCV, a single stranded RNA small virus belonging to Flaviviridae family, has been examined in the initial triggering phase of SS. […] Despite possible involvement of viral infection in SS, the most common antiviral drugs do not seem to show real benefit in the treatment of SS.
  • #30 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacterium. […] The cause of Sjgren’s disease is unknown, but it may be the influence of a combination of genetic, environmental, and other factors, as is the case with many other autoimmune disorders. […] The observation of high rates of autoimmune disorders in families with a history of Sjgren’s disease is linked with a genetic predisposition to the disease. […] Since Sjgren’s disease is associated with a high prevalence in women, sex hormones, especially estrogen, are believed to affect humoral and cell-mediated immune responses affecting susceptibility to the disease. […] Microchimerism of fetal cells (offspring lymphoid cells in maternal circulation) may generate autoimmunity in women who have previously been pregnant.
  • #31 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Viruses are viable candidates as environmental triggers, although proof of causation has remained elusive, and certainly no single virus has been implicated. Epstein-Barr virus (EBV), HTLV-1, human herpesvirus 6 (HHV-6), HIV, hepatitis C virus (HCV), and cytomegalovirus (CMV) may have a role. […] Damage and/or cell death due to viral infection or other causes may provide triggering antigens to Toll-like receptors in or on dendritic or epithelial cells, which, by recognizing pathogen-associated patterns, are activated and begin producing cytokines, chemokines, and adhesion molecules. […] A population-based cohort study of Taiwanese residents found that exposure to carbon monoxide (CO), nitric oxide (NO), and methane (CH4) was associated with a higher risk of developing primary Sjgren syndrome.
  • #32 Sjögren’s Disease: Causes, Symptoms and Treatment | Banner
    https://www.bannerhealth.com/services/rheumatology/conditions/sjogrens-disease
    Sjgrens disease is an autoimmune disorder that affects nearly four million Americans each year. […] Sjgrens disease occurs when the immune system attacks the glands that produce moisture. […] Sjgrens disease occurs when the immune system doesn’t work as it should. Typically, your immune system is there to protect you from infections. But in this case, it mistakenly targets healthy parts of your body. […] Experts dont know exactly why this happens, but some factors may increase your risk, including: […] A family history of autoimmune disorders may increase your risk of developing Sjgrens disease. […] The disease is more common in women or people assigned female at birth (AFAB). […] While Sjgrens disease can occur at any age, symptoms tend to appear after age 40. […] People with other autoimmune diseases, like rheumatoid arthritis, lupus, type 1 diabetes or even recent viral infections may have a higher risk of developing Sjgrens disease. […] Exposure to certain environmental factors, like chronic stress or pollution, may trigger the disease in people who are more likely to develop it due to genetics.
  • #33 What Causes Sjögren’s Syndrome | Pompa Program
    https://pompaprogram.com/what-causes-sjogrens-syndrome/
    What Causes Sjögren’s Syndrome – And How To Manage It […] Exactly what causes Sjögren’s syndrome is not known, although autoimmune conditions result from a combination of genetics, toxin exposure, and microbiome dysfunction. […] Sjögren’s syndrome is caused by chronic inflammation that goes unchecked, causing the immune system to become overactive and attack healthy cells, gradually destroying them. […] Genetics is one of the main causes of Sjogren’s syndrome. Research has shown that certain genes may increase a person’s risk of developing this autoimmune disorder. […] Stressors are one of the many causes of Sjogren’s Syndrome. Stress can cause an increase in inflammation, which may trigger the symptoms associated with Sjogren’s Syndrome. […] Mitochondria dysfunction is thought to be a possible cause of Sjögren’s Syndrome. […] Oxidative stress is a major factor contributing to Sjögren’s Syndrome. […] Environmental factors play a role in the development of Sjogren’s syndrome, including exposure to certain environmental toxins. […]
  • #34 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
    https://www.mdpi.com/2077-0383/9/7/2299
    The epigenetic processes more closely linked to the disease are DNA methylation, miRNA, circular mRNA and long non-coding RNA function. […] The expression analysis of lncRNAs in SS patients has shown lncRNAs LINC00657, LINC00511 and CTD-2020K17.1 potentially associated with the disease. […] The strong predisposition of women to develop SS clearly demonstrates the role of sex hormones as a risk factor of the disease. […] In a recent case-control study, pSS in women was associated with lower oestrogen exposure and lower cumulative menstrual cycling time compared to sicca controls. […] The role of HCV, a single stranded RNA small virus belonging to Flaviviridae family, has been examined in the initial triggering phase of SS. […] Despite possible involvement of viral infection in SS, the most common antiviral drugs do not seem to show real benefit in the treatment of SS.
  • #35 Sjögren syndrome Information | Mount Sinai – New York
    https://www.mountsinai.org/health-library/diseases-conditions/sj-gren-syndrome
    Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The condition may affect other parts of the body, including the kidneys and lungs. […] The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most often in women ages 40 to 50. It is rare in children. […] Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder. […] Secondary Sjögren syndrome occurs along with another autoimmune disorder, such as: Rheumatoid arthritis (RA), Systemic lupus erythematosus, Scleroderma, Polymyositis, Hepatitis C can affect the salivary glands and looks like Sjögren syndrome, IgG4 disease can look like Sjögren syndrome and should be considered.
  • #36 Diagnosis and classification of Sjögren’s disease – UpToDate
    https://www.uptodate.com/contents/diagnosis-and-classification-of-sjogrens-disease
    Sjögren’s disease (SjD) is a chronic autoimmune inflammatory disorder characterized by impaired lacrimal and salivary gland function with resultant dryness of the eyes and mouth. […] SjD may occur alone or in association with other systemic autoimmune rheumatic diseases. […] The most common of these are rheumatoid arthritis and systemic lupus erythematosus. […] SjD is most prevalent in female adults in their 50s and 60s but can affect adolescents and young adults, as well as males.
  • #37 Sjögren syndrome Information | Mount Sinai – New York
    https://www.mountsinai.org/health-library/diseases-conditions/sj-gren-syndrome
    Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The condition may affect other parts of the body, including the kidneys and lungs. […] The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most often in women ages 40 to 50. It is rare in children. […] Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder. […] Secondary Sjögren syndrome occurs along with another autoimmune disorder, such as: Rheumatoid arthritis (RA), Systemic lupus erythematosus, Scleroderma, Polymyositis, Hepatitis C can affect the salivary glands and looks like Sjögren syndrome, IgG4 disease can look like Sjögren syndrome and should be considered.
  • #38 Sjogren Syndrome – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK431049/
    As is the case with the majority of autoimmune disorders, the precise etiology of Sjogren syndrome is unknown. A genetic predisposition involving the major histocompatibility (MHC) locus is likely with individuals sharing haplotypes in the HLA-DQA_DQB_ region having an increased relative risk, although the diseases are described worldwide. These major histocompatibility haplotypes are hypothesized to result in an aberrant immune response in conjunction with certain environmental triggers. Laboratory studies, as well as some indirect epidemiologic evidence, implicates viruses, including Epstein-Barr virus (EBV) as participants in disease pathogenesis.[4][5] […] Sjogren syndrome frequently occurs in conjunction with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). In this setting, authors will refer to it as secondary Sjogren or Sjogren-overlap syndrome.
  • #39 Sjögren’s Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/4929-sjogrens-syndrome
    Sjgrens syndrome is an autoimmune disease that happens when your immune system damages the glands that produce and control moisture in your body. […] Sjgrens syndrome is an autoimmune disease that makes your glands produce less moisture than they should. […] Sjgrens syndrome is an autoimmune disease. Autoimmune diseases happen when your immune system mistakenly damages your body instead of protecting it. Experts arent sure what makes your immune system attack your glands and cause Sjgrens syndrome. […] Primary Sjgrens syndrome happens with no known trigger or cause. […] Other health conditions trigger secondary Sjgrens syndrome, especially other autoimmune diseases and some viral infections. […] Any autoimmune disease can trigger secondary Sjgrens syndrome. Some autoimmune diseases that are related to Sjgrens syndrome include: Rheumatoid arthritis, Psoriatic arthritis, Lupus. […] Even though studies have linked Sjgrens syndrome to other conditions, theres no guarantee youll develop it if you have these conditions.
  • #40 Sjögren’s Syndrome Causes, Symptoms, and Treatments
    https://www.upmc.com/services/rheumatology/conditions/sjogrens-disease
    Sjgrens disease is an autoimmune condition where your immune system, which normally fights infections, mistakenly attacks your own body. […] The cause of Sjgrens disease is unknown but likely involves a combination of genetic and environmental risk factors, such as viral or bacterial infections. […] Sjgren’s can occur on its own without another autoimmune disorder or with another autoimmune condition. Half of cases occur with another autoimmune condition, such as lupus, rheumatoid arthritis, or scleroderma.
  • #41 Sjögren’s Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/4929-sjogrens-syndrome
    Sjgrens syndrome is an autoimmune disease that happens when your immune system damages the glands that produce and control moisture in your body. […] Sjgrens syndrome is an autoimmune disease that makes your glands produce less moisture than they should. […] Sjgrens syndrome is an autoimmune disease. Autoimmune diseases happen when your immune system mistakenly damages your body instead of protecting it. Experts arent sure what makes your immune system attack your glands and cause Sjgrens syndrome. […] Primary Sjgrens syndrome happens with no known trigger or cause. […] Other health conditions trigger secondary Sjgrens syndrome, especially other autoimmune diseases and some viral infections. […] Any autoimmune disease can trigger secondary Sjgrens syndrome. Some autoimmune diseases that are related to Sjgrens syndrome include: Rheumatoid arthritis, Psoriatic arthritis, Lupus. […] Even though studies have linked Sjgrens syndrome to other conditions, theres no guarantee youll develop it if you have these conditions.
  • #42 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    Viral proteins, engulfed molecules, or degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of Sjgren’s disease development. […] The pathogenetic mechanisms of Sjgren’s disease have not been fully elucidated, resulting in the lack of pathophysiology knowledge of the management of this autoimmune exocrinopathy. […] In the presence of a susceptible genetic background, both environmental and hormonal factors are thought capable of triggering the infiltration of lymphocytes, specifically CD4+ T cells, B cells, and plasma cells, causing glandular dysfunction in the salivary and lacrimal glands.
  • #43 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    The etiology of Sjgren syndrome is not well understood. The presence of activated salivary gland epithelial cells expressing major histocompatibility complex (MHC) class II molecules and the identification of inherited susceptibility markers suggest that environmental or endogenous antigens trigger a self-perpetuating inflammatory response in susceptible individuals. In addition, the continuing presence of active interferon pathways in Sjgren syndrome suggests ongoing activation of the innate immune system. […] Together, these findings suggest an ongoing interaction between the innate and acquired immune systems in Sjgren syndrome. […] The frequency of HLA-DR52 in patients with primary Sjgren syndrome is estimated to be 87%, but it is also significantly increased in secondary Sjgren syndrome that occurs with rheumatoid arthritis or systemic lupus erythematosus.
  • #44 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    Viral proteins, engulfed molecules, or degraded self-structures may initiate autoimmunity by molecular mimicry and increase the chances of Sjgren’s disease development. […] The pathogenetic mechanisms of Sjgren’s disease have not been fully elucidated, resulting in the lack of pathophysiology knowledge of the management of this autoimmune exocrinopathy. […] In the presence of a susceptible genetic background, both environmental and hormonal factors are thought capable of triggering the infiltration of lymphocytes, specifically CD4+ T cells, B cells, and plasma cells, causing glandular dysfunction in the salivary and lacrimal glands.
  • #45
    https://link.springer.com/article/10.1007/s10238-021-00728-6
    It is widely accepted that epigenetic factors play an important role in autoimmune disorders. […] Nowadays, epithelial cells are considered major players in the pathogenesis of SS, so that the term autoimmune „epithelitis” is increasingly used to describe this condition. […] An over-expression of IFN-inducible genes, also known as IFN-signature, was demonstrated among patients with SS, both in peripheral blood and salivary glands. […] In recent years, also Natural Killer (NK) cells have been implicated in SS pathogenesis. […] B lymphocytes are considered major players in SS pathogenesis and its main complication (namely lymphoma). […] Besides B lymphocytes, also T cells significantly contribute to SS pathogenesis.
  • #46 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
    https://www.mdpi.com/2077-0383/9/7/2299
    According to the current physiopathogenic model of SS, environmental factors including viral infection lead to SGEC and Toll Like Receptors (TLRs) activation. […] Primary viruses involved in SS induction include Epstein–Barr (EBV) viruses, Human T-lymphotropic virus type I (HTLVI), hepatitis virus C (HCV) and coxsackievirus. […] Genetic predisposition to SS plays a role in the trigger phase of the disease. […] A strong association between human leucocyte antigen (HLA)-DR and HLA-DQ alleles belonging to the group of major histocompatibility genes (MHC) class II genes and SS was observed throughout different populations including Caucasian, Japanese and Chinese populations. […] While gene polymorphism plays an indisputable role in the triggering phase of SS, the individual contribution of each genetic factor remains to be assessed.
  • #47 Diagnosis and Management of Sjögren Syndrome | AAFP
    https://www.aafp.org/pubs/afp/issues/2009/0315/p465.html
    Sjgren syndrome is a systemic autoimmune disease characterized by dry eyes and dry mouth. […] The pathogenesis of Sjgren syndrome is obscure. It is probably the result of an environmental stimulus that promotes an auto-immune reaction in genetically susceptible persons. Infectious agents most commonly sialotropic viruses have been postulated to trigger the syndrome; however, associations with most of the potential viral candidates, including cytomegalovirus and Epstein-Barr virus, are weak. […] Serologic studies show an association between primary Sjgren syndrome and HLA-DR haplotypes. […] Sjgren syndrome represents a complex, multifaceted activation of the immune system. B-lymphocyte dysregulation and hyper-activity play a major role in the disease.
  • #48 Sjogren’s Syndrome – Arthritis and Rheumatology Clinics of Kansas
    https://arck.org/patient-education/sjogrens-syndrome/
    Antibodies seen in SS include the anti-nuclear antibody (ANA) also seen in SLE, the rheumatoid factor (RF) also observed in RA, and some more specific antibodies known as Ro/SSA and La/SSB. […] While these latter two markers can also be seen in some SLE patients, their presence in a patient who has dry mouth and/or eyes in the absence of other features of SLE strongly supports a diagnosis of SS.
  • #49 Sjögren’s Syndrome (Causes, Symptoms, and Treatment)
    https://patient.info/doctor/sjogrens-syndrome-pro
    Primary Sjgren’s syndrome is an immune-mediated condition of unknown aetiology characterised by focal lymphocytic infiltration of exocrine glands. […] The disease is called primary if it develops in isolation and secondary if it occurs with other autoimmune diseases, usually rheumatoid arthritis, systemic lupus erythematosus or scleroderma. […] The best-described autoantibodies in primary Sjgren’s syndrome are the anti-Ro and anti-La antibodies. About two thirds of patients with primary Sjgren’s syndrome have anti-Ro antibodies and/or anti-La antibodies. […] Sjogren’s syndrome is a chronic, debilitating condition that warrants effective management. […] Disease-modifying drugs have been used to treat some of the systemic manifestations of the disease but the level of evidence is low and larger-scale trials of the most promising treatments are needed. […] There is an increased risk of developing non-Hodgkin’s lymphoma.
  • #50 Sjögren’s Syndrome | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/s/sjogrens-syndrome.html
    Sjgren’s syndrome is a disease in which the body’s defense system (immune system) attacks healthy tissues causing dryness of the mouth, eyes and other membranes of the body that secrete mucous. […] While its cause is not known, there may be a genetic factor. […] A person who has Sjgren’s syndrome has a 44 times higher risk of lymphoma compared to other people. Additionally, such individuals are at higher risk of Waldenstrm’s macroglobulinemia.
  • #51 Sjogren’s Syndrome – Arthritis and Rheumatology Clinics of Kansas
    https://arck.org/patient-education/sjogrens-syndrome/
    SS is a common yet under-diagnosed condition causing dryness of the mouth and eyes as well as inflammation in other parts of the body. […] This condition seems to result from the body’s immune system attacking the glands that make saliva and tears, causing these glands to swell and/or malfunction. […] These different parts of the body can become inflamed due to an attack from the immune system much like what we see in patients with SLE. […] Another problem worth mentioning in SS is the increased risk for developing lymphoma, a cancer of the lymph nodes and blood cells. This occurs in 5% of SS patients, making them 44 times more likely than the general population to develop lymphoma. […] It is also important to exclude other causes of these symptoms include medications (anti-histamines, certain kinds of anti-depressants), radiation to the head and neck, viral infections, diabetes, and a number of other chronic illnesses.
  • #52 What Is Sjögren’s Syndrome? Symptoms, Causes, Diagnosis, Treatment, and Prevention
    https://www.everydayhealth.com/sjogrens-syndrome/guide/
    Researchers arent sure exactly what causes Sjgrens syndrome, but they believe that a combination of genetic and environmental factors may predispose someone to developing this condition. […] Several genes appear to be involved in the development of Sjgrens, and a previous bout of a viral or bacterial infection may play a role in triggering the disease. […] In people who are genetically predisposed, an infection may act as a trigger to activate their immune system and cause it to attack healthy cells and tissue. […] There is also some evidence that having a relative who has Sjgrens syndrome may increase your risk: Approximately 12 percent of people with Sjgrens have one or more relatives who also have the disease. […] And since Sjgrens affects women at a far greater rate than men 9 out of 10 people with the disease are women its believed that estrogen may play a role in the development of the disease. […] Being a post-menopausal woman is a risk factor for Sjorgens syndrome.
  • #53 Sjogren’s Syndrome: Causes, Symptoms, and Treatment Options
    https://www.webmd.com/a-to-z-guides/sjogrens-syndrome
    Doctors don’t know the exact cause. You may have genes that put you at risk. An infection with a bacteria or virus may be a trigger that sets the disease in motion. […] For example, let’s say you have a defective gene that’s linked to Sjogren’s and then you get an infection. Your immune system swings into action. […] White blood cells normally lead the attack against the germs. But because of your faulty gene, your white blood cells target healthy cells in the glands that make saliva and tears. There’s no letup in the fight, so your symptoms will keep going unless you get treatment. […] Some other things can make you more likely to have Sjogren’s, including: Age. Sjogren’s usually affects people over 40, but younger adults and children can get it, too. […] Sex. Women are 10 times more likely to have Sjogren’s than men. […] Other autoimmune issues. Nearly half of all people who have Sjogren’s also have another autoimmune condition, like lupus or rheumatoid arthritis.
  • #54 Sjögren’s Syndrome | EdRheum
    https://www.edrheum.org/sjogren-syndrome/
    Sjgrens syndrome most commonly affects people between aged between 40-60 years. […] The exact cause of Sjgrens syndrome is unknown, but there is evidence of genetic and environmental factors. […] Although there is no common inheritance of Sjgrens syndrome in families, other autoimmune conditions associated with secondary Sjgrens syndrome can be seen more commonly.
  • #55 What Is Sjögren’s Syndrome? Symptoms, Causes, Diagnosis, Treatment, and Prevention
    https://www.everydayhealth.com/sjogrens-syndrome/guide/
    Researchers arent sure exactly what causes Sjgrens syndrome, but they believe that a combination of genetic and environmental factors may predispose someone to developing this condition. […] Several genes appear to be involved in the development of Sjgrens, and a previous bout of a viral or bacterial infection may play a role in triggering the disease. […] In people who are genetically predisposed, an infection may act as a trigger to activate their immune system and cause it to attack healthy cells and tissue. […] There is also some evidence that having a relative who has Sjgrens syndrome may increase your risk: Approximately 12 percent of people with Sjgrens have one or more relatives who also have the disease. […] And since Sjgrens affects women at a far greater rate than men 9 out of 10 people with the disease are women its believed that estrogen may play a role in the development of the disease. […] Being a post-menopausal woman is a risk factor for Sjorgens syndrome.
  • #56 Sjogren’s syndrome: Symptoms, causes, and treatment
    https://www.medicalnewstoday.com/articles/233747
    Sjogrens syndrome happens when white blood cells infiltrate saliva glands, tear glands, and other exocrine tissues, decreasing tear and saliva production. […] The causes of Sjogrens remain largely unknown. […] However, researchers believe a viral or bacterial infection may trigger the condition, but that the underlying cause is primarily genetic and environmental. […] As the majority of people who develop Sjogrens are female, one theory is that estrogen, a female hormone, plays an important role. […] Some studies theorize that estrogen protects against Sjogrens syndrome, and falling levels of the hormone may alter immune function and trigger the condition. This means a major risk factor for Sjogrens syndrome is menopause.
  • #57 Diagnosing Sjogren’s Syndrome | NYU Langone Health
    https://nyulangone.org/conditions/sjogrens-syndrome/diagnosis
    Experts dont know for sure what causes Sjogrens syndrome, but some believe that its a combination of genetics and a bacterial or viral infection. […] The majority of people diagnosed with Sjogrens syndrome are between the ages of 40 and 69. Those at risk include people who already have an autoimmune disease, such as rheumatoid arthritis or systemic lupus erythematosus, commonly known as lupus, and those who have family members with an autoimmune disease. […] There are two types of Sjogrens syndrome. Primary Sjogrens syndrome occurs on its own and is not associated with another illness. Secondary Sjogrens syndrome develops in a person who has another autoimmune disease, usually rheumatoid arthritis or lupus.
  • #58 Sjögren Disease: Symptoms, Treatment, and More
    https://www.healthline.com/health/sjogren-syndrome
    Sjgren disease, previously known as Sjgrens syndrome, is an autoimmune disorder that affects the glands responsible for producing saliva and tears. […] In this autoimmune disorder, the bodys immune system mistakenly targets and damages these glands. […] People likely develop Sjgren disease due to a combination of genetic and environmental factors, such as previous infection with viruses or bacteria. […] About half of the people with Sjgren disease have rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or polymyositis.
  • #59 Sjögren’s Syndrome | NeuropathyCommons
    https://neuropathycommons.org/neuropathy/causes-neuropathy/sj%C3%B6grens-syndrome
    Risk factors such as age, sex, and family history should also be considered. Sjgren’s syndrome patients tend to be female, 40 to 60 years of age, and about half have another autoimmune disorder. A family history of autoimmune issues is a risk factor as well. […] Precautions should be taken when considering lip biopsy testing. False-negative results can be obtained if patients are taking or have recently taken immunosuppressive medication including corticosteroids, in patients who have had Sjgren’s syndrome for long enough for salivary glands to entirely degenerate, and in cigarette smokers. False positives can occur in older people, after trauma, or in people with hepatitis C, lymphoma, or sarcoidosis. […] Salivary gland biopsies are an important tool for the diagnosis of Sjgren’s in people with negative blood tests and no dry mouth or eye, but with other Sjgren’s-like symptoms.
  • #60 Causes of Sjögren’s Syndrome | Sjogren’s Syndrome NewsEnvelope icon
    https://sjogrenssyndromenews.com/causes-of-sjogrens-syndrome/
    Sjögren’s syndrome is an autoimmune disorder primarily characterized by inflammation of the tear and salivary glands, leading to the condition’s signature symptoms of dry eyes and mouth. […] Experts don’t know for sure what causes Sjögren’s syndrome, but some believe that a combination of genetics and an infection causes the immune system to start mistakenly attacking the body’s own cells. […] It is thought that Sjögren’s arises from a complex mix of genetic and environmental factors. […] The disease is 12 times more common among relatives of Sjögren’s patients than in the general population, pointing to a genetic component. […] While certain genes increase a person’s risk for Sjögren’s syndrome, other factors, such as viral or bacterial infections, appear necessary for sending the immune system into overdrive. […] Those at risk also include people who already have an autoimmune disease, such as rheumatoid arthritis or lupus, which might share some disease mechanisms with Sjögren’s.
  • #61 Sjögren’s Disease: Causes, Symptoms and Treatment | Banner
    https://www.bannerhealth.com/services/rheumatology/conditions/sjogrens-disease
    Sjgrens disease is an autoimmune disorder that affects nearly four million Americans each year. […] Sjgrens disease occurs when the immune system attacks the glands that produce moisture. […] Sjgrens disease occurs when the immune system doesn’t work as it should. Typically, your immune system is there to protect you from infections. But in this case, it mistakenly targets healthy parts of your body. […] Experts dont know exactly why this happens, but some factors may increase your risk, including: […] A family history of autoimmune disorders may increase your risk of developing Sjgrens disease. […] The disease is more common in women or people assigned female at birth (AFAB). […] While Sjgrens disease can occur at any age, symptoms tend to appear after age 40. […] People with other autoimmune diseases, like rheumatoid arthritis, lupus, type 1 diabetes or even recent viral infections may have a higher risk of developing Sjgrens disease. […] Exposure to certain environmental factors, like chronic stress or pollution, may trigger the disease in people who are more likely to develop it due to genetics.
  • #62 What Causes Sjogrens Syndrome? – Carolina Arthritis
    https://www.carolinaarthritis.com/what-causes-sjogrens-syndrome/
    Sjogrens Syndrome is a chronic autoimmune disorder that causes inflammation in various areas of the body, but most often affects the exocrine glands that produce tears and salvia. […] One of the big unknowns behind Sjogrens Syndrome is what causes it; as of now, scientists arent exactly sure why some people develop Sjogrens Syndrome. According to the Mayo Clinic, certain genes put people at higher risk of the disorder, but it appears that a triggering mechanism such as infection with a particular virus or strain of bacteria is also necessary. […] There is a lot of speculation behind the causes of Sjogrens; some theories include environmental factors that change the immune system such as a hepatitis C or Epstein-Barr infection. And because so many people affected by Sjogrens are female, some doctors believe that estrogen could also be a factor.
  • #63 Sjögren’s Syndrome | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/s/sjogrens-syndrome.html
    Sjgren’s syndrome is a disease in which the body’s defense system (immune system) attacks healthy tissues causing dryness of the mouth, eyes and other membranes of the body that secrete mucous. […] While its cause is not known, there may be a genetic factor. […] A person who has Sjgren’s syndrome has a 44 times higher risk of lymphoma compared to other people. Additionally, such individuals are at higher risk of Waldenstrm’s macroglobulinemia.
  • #64 Sjogren’s Syndrome – Arthritis and Rheumatology Clinics of Kansas
    https://arck.org/patient-education/sjogrens-syndrome/
    SS is a common yet under-diagnosed condition causing dryness of the mouth and eyes as well as inflammation in other parts of the body. […] This condition seems to result from the body’s immune system attacking the glands that make saliva and tears, causing these glands to swell and/or malfunction. […] These different parts of the body can become inflamed due to an attack from the immune system much like what we see in patients with SLE. […] Another problem worth mentioning in SS is the increased risk for developing lymphoma, a cancer of the lymph nodes and blood cells. This occurs in 5% of SS patients, making them 44 times more likely than the general population to develop lymphoma. […] It is also important to exclude other causes of these symptoms include medications (anti-histamines, certain kinds of anti-depressants), radiation to the head and neck, viral infections, diabetes, and a number of other chronic illnesses.
  • #65 Facilitating timely diagnosis of Sjögren’s syndrome | BDJ Team
    https://www.nature.com/articles/bdjteam201826
    Sjgren’s syndrome is an autoimmune disease in which intense lymphocytic infiltration of the exocrine glands and hyperactivity of B lymphocytes cause inflammation, which damages glandular tissue and impairs function. […] Primary Sjgren’s syndrome is diagnosed in the absence of any other connective tissue disease, while secondary Sjgren’s syndrome is accompanied by other autoimmune diseases, such as rheumatoid arthritis, lupus erythematosus, Graft Versus Host Disease, or in rare cases, Behet’s disease. Secondary Sjgren’s syndrome comprises approximately 60% of cases. […] The systemic autoimmunity associated with Sjgren’s syndrome can also result in dry skin, fatigue, low grade fever, constipation, myalgia, and joint pain. Other conditions which might occur include small vessel vasculitis, Raynaud’s phenomenon, pulmonary symptoms, nephritis, neuropathy, hypothyroidism, and lymphoma.
  • #66 Facilitating timely diagnosis of Sjögren’s syndrome | BDJ Team
    https://www.nature.com/articles/bdjteam201826
    Sjgren’s syndrome is an autoimmune disease in which intense lymphocytic infiltration of the exocrine glands and hyperactivity of B lymphocytes cause inflammation, which damages glandular tissue and impairs function. […] Primary Sjgren’s syndrome is diagnosed in the absence of any other connective tissue disease, while secondary Sjgren’s syndrome is accompanied by other autoimmune diseases, such as rheumatoid arthritis, lupus erythematosus, Graft Versus Host Disease, or in rare cases, Behet’s disease. Secondary Sjgren’s syndrome comprises approximately 60% of cases. […] The systemic autoimmunity associated with Sjgren’s syndrome can also result in dry skin, fatigue, low grade fever, constipation, myalgia, and joint pain. Other conditions which might occur include small vessel vasculitis, Raynaud’s phenomenon, pulmonary symptoms, nephritis, neuropathy, hypothyroidism, and lymphoma.
  • #67 Sjögren Syndrome – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/systemic-rheumatic-diseases/sj%C3%B6gren-syndrome
    Sjgren syndrome is a chronic, systemic, autoimmune, inflammatory disorder of unknown cause. […] It is classified as primary when there is no other associated disease. It is secondary in approximately 30% of patients with autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, Hashimoto thyroiditis, primary biliary cirrhosis, or chronic autoimmune hepatitis. […] Genetic associations have been found (eg, HLA-DR3 antigens in White people with primary Sjgren syndrome) but are not necessary for diagnosis or clinical management. […] The most common causes of dry eyes and dry mouth (sicca symptoms) are aging and medications, but when parotid enlargement occurs in addition to sicca symptoms, diseases such as hepatitis C, HIV, bulimia, and sarcoidosis should be differentiated from Sjgren syndrome. […] When submandibular glands are enlarged, particularly in patients with a history of pancreatitis, IgG4-related disease (characterized by lymphoplasmacytic infiltration and fibrosis of various organs) should be considered.
  • #68 Facilitating timely diagnosis of Sjögren’s syndrome | BDJ Team
    https://www.nature.com/articles/bdjteam201826
    Sjgren’s syndrome is an autoimmune disease in which intense lymphocytic infiltration of the exocrine glands and hyperactivity of B lymphocytes cause inflammation, which damages glandular tissue and impairs function. […] Primary Sjgren’s syndrome is diagnosed in the absence of any other connective tissue disease, while secondary Sjgren’s syndrome is accompanied by other autoimmune diseases, such as rheumatoid arthritis, lupus erythematosus, Graft Versus Host Disease, or in rare cases, Behet’s disease. Secondary Sjgren’s syndrome comprises approximately 60% of cases. […] The systemic autoimmunity associated with Sjgren’s syndrome can also result in dry skin, fatigue, low grade fever, constipation, myalgia, and joint pain. Other conditions which might occur include small vessel vasculitis, Raynaud’s phenomenon, pulmonary symptoms, nephritis, neuropathy, hypothyroidism, and lymphoma.
  • #69 Facilitating timely diagnosis of Sjögren’s syndrome | BDJ Team
    https://www.nature.com/articles/bdjteam201826
    The diagnostic criteria for Sjgren’s syndrome is evolving, and has been revised numerous times since 1965. […] In 2016, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) developed and validated an international consensus of data-driven classification criteria for primary Sjgren’s syndrome (SS), which were established for use in individuals with signs and/or symptoms suggestive of SS.
  • #70 Sjögren Syndrome – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/systemic-rheumatic-diseases/sj%C3%B6gren-syndrome
    Sjgren syndrome is a chronic, systemic, autoimmune, inflammatory disorder of unknown cause. […] It is classified as primary when there is no other associated disease. It is secondary in approximately 30% of patients with autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, Hashimoto thyroiditis, primary biliary cirrhosis, or chronic autoimmune hepatitis. […] Genetic associations have been found (eg, HLA-DR3 antigens in White people with primary Sjgren syndrome) but are not necessary for diagnosis or clinical management. […] The most common causes of dry eyes and dry mouth (sicca symptoms) are aging and medications, but when parotid enlargement occurs in addition to sicca symptoms, diseases such as hepatitis C, HIV, bulimia, and sarcoidosis should be differentiated from Sjgren syndrome. […] When submandibular glands are enlarged, particularly in patients with a history of pancreatitis, IgG4-related disease (characterized by lymphoplasmacytic infiltration and fibrosis of various organs) should be considered.
  • #71 Sjogren’s Syndrome – Arthritis and Rheumatology Clinics of Kansas
    https://arck.org/patient-education/sjogrens-syndrome/
    SS is a common yet under-diagnosed condition causing dryness of the mouth and eyes as well as inflammation in other parts of the body. […] This condition seems to result from the body’s immune system attacking the glands that make saliva and tears, causing these glands to swell and/or malfunction. […] These different parts of the body can become inflamed due to an attack from the immune system much like what we see in patients with SLE. […] Another problem worth mentioning in SS is the increased risk for developing lymphoma, a cancer of the lymph nodes and blood cells. This occurs in 5% of SS patients, making them 44 times more likely than the general population to develop lymphoma. […] It is also important to exclude other causes of these symptoms include medications (anti-histamines, certain kinds of anti-depressants), radiation to the head and neck, viral infections, diabetes, and a number of other chronic illnesses.
  • #72 Facilitating timely diagnosis of Sjögren’s syndrome | BDJ Team
    https://www.nature.com/articles/bdjteam201826
    The diagnostic criteria for Sjgren’s syndrome is evolving, and has been revised numerous times since 1965. […] In 2016, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) developed and validated an international consensus of data-driven classification criteria for primary Sjgren’s syndrome (SS), which were established for use in individuals with signs and/or symptoms suggestive of SS.
  • #73 Sjögren’s Syndrome | NeuropathyCommons
    https://neuropathycommons.org/neuropathy/causes-neuropathy/sj%C3%B6grens-syndrome
    Risk factors such as age, sex, and family history should also be considered. Sjgren’s syndrome patients tend to be female, 40 to 60 years of age, and about half have another autoimmune disorder. A family history of autoimmune issues is a risk factor as well. […] Precautions should be taken when considering lip biopsy testing. False-negative results can be obtained if patients are taking or have recently taken immunosuppressive medication including corticosteroids, in patients who have had Sjgren’s syndrome for long enough for salivary glands to entirely degenerate, and in cigarette smokers. False positives can occur in older people, after trauma, or in people with hepatitis C, lymphoma, or sarcoidosis. […] Salivary gland biopsies are an important tool for the diagnosis of Sjgren’s in people with negative blood tests and no dry mouth or eye, but with other Sjgren’s-like symptoms.
  • #74 Sjögren’s Syndrome | NeuropathyCommons
    https://neuropathycommons.org/neuropathy/causes-neuropathy/sj%C3%B6grens-syndrome
    Risk factors such as age, sex, and family history should also be considered. Sjgren’s syndrome patients tend to be female, 40 to 60 years of age, and about half have another autoimmune disorder. A family history of autoimmune issues is a risk factor as well. […] Precautions should be taken when considering lip biopsy testing. False-negative results can be obtained if patients are taking or have recently taken immunosuppressive medication including corticosteroids, in patients who have had Sjgren’s syndrome for long enough for salivary glands to entirely degenerate, and in cigarette smokers. False positives can occur in older people, after trauma, or in people with hepatitis C, lymphoma, or sarcoidosis. […] Salivary gland biopsies are an important tool for the diagnosis of Sjgren’s in people with negative blood tests and no dry mouth or eye, but with other Sjgren’s-like symptoms.
  • #75
    https://www.advocatehealth.com/health-services/rheumatology/sjogrens-syndrome
    Sjogren’s syndrome is a chronic autoimmune disease that primarily affects your moisture-producing glands and causes extensive dryness throughout the body. It often accompanies other autoimmune disorders but can develop on its own. […] Sjogren’s syndrome is a chronic inflammatory disorder where your immune system mistakenly attacks your moisture-producing glands, causing widespread inflammation and dryness. […] Scientists aren’t sure what causes autoimmune diseases, including Sjogren’s syndrome. However, a common link is an overactive immune system due to genetic and environmental factors. […] Your immune system, which normally protects your body against harmful invaders such as viruses and bacteria, starts attacking your own body’s cells. If you have Sjogren’s syndrome, it attacks your moisture-producing glands.
  • #76 Sjögren’s Syndrome: Causes, Symptoms, Treatment, Diet
    https://www.medicinenet.com/sjogrens_syndrome/article.htm
    Sjgren’s syndrome can cause white blood cells to attack the tear and salivary glands. This can dry out the eyes and mouth. […] While the exact cause of Sjgren’s syndrome is not known, there is growing scientific support for genetic (inherited) factors. The genetic background of Sjgren’s syndrome patients is an active area of research. […] The main risk factor for the development of Sjgren’s syndrome is being a member of a family that is already characterized as having autoimmune illnesses. […] Accordingly, certain genes that are inherited from ancestors can likely predispose one to the development of Sjgren’s syndrome. […] Sjgren syndrome is an area of active immunology research. Many new treatments will be available in the near future. Research has suggested that rituximab (Rituxan) may be beneficial for many features of Sjgren syndrome.
  • #77 Sjögren’s Syndrome: Causes, Symptoms, Treatment, Diet
    https://www.medicinenet.com/sjogrens_syndrome/article.htm
    Sjgren’s syndrome can cause white blood cells to attack the tear and salivary glands. This can dry out the eyes and mouth. […] While the exact cause of Sjgren’s syndrome is not known, there is growing scientific support for genetic (inherited) factors. The genetic background of Sjgren’s syndrome patients is an active area of research. […] The main risk factor for the development of Sjgren’s syndrome is being a member of a family that is already characterized as having autoimmune illnesses. […] Accordingly, certain genes that are inherited from ancestors can likely predispose one to the development of Sjgren’s syndrome. […] Sjgren syndrome is an area of active immunology research. Many new treatments will be available in the near future. Research has suggested that rituximab (Rituxan) may be beneficial for many features of Sjgren syndrome.
  • #78 Sjögren’s Syndrome (Causes, Symptoms, and Treatment)
    https://patient.info/doctor/sjogrens-syndrome-pro
    Primary Sjgren’s syndrome is an immune-mediated condition of unknown aetiology characterised by focal lymphocytic infiltration of exocrine glands. […] The disease is called primary if it develops in isolation and secondary if it occurs with other autoimmune diseases, usually rheumatoid arthritis, systemic lupus erythematosus or scleroderma. […] The best-described autoantibodies in primary Sjgren’s syndrome are the anti-Ro and anti-La antibodies. About two thirds of patients with primary Sjgren’s syndrome have anti-Ro antibodies and/or anti-La antibodies. […] Sjogren’s syndrome is a chronic, debilitating condition that warrants effective management. […] Disease-modifying drugs have been used to treat some of the systemic manifestations of the disease but the level of evidence is low and larger-scale trials of the most promising treatments are needed. […] There is an increased risk of developing non-Hodgkin’s lymphoma.
  • #79 Sjögren’s Syndrome | ColumbiaDoctors
    https://www.columbiadoctors.org/treatments-conditions/sjogrens-syndrome
    In autoimmune diseases like Sjogren’s syndrome, your immune system attacks your body’s own cells and tissues causing inflammation and pain. Researchers do not know what causes Sjogren’s syndrome, though there may be a genetic link to developing autoimmune disorders plus a trigger, like an exposure to an infection that activates the disease. […] Research is underway to better understand what causes autoimmune disorders so that cures can be developed and lives can be greatly improved.