Zespół sjögrena
Objawy

Zespół Sjögrena to przewlekła, autoimmunologiczna choroba charakteryzująca się limfocytarnym naciekiem gruczołów wydzielniczych, prowadzącym do ich dysfunkcji i zmniejszonej produkcji łez oraz śliny. Występuje głównie u kobiet (9:1) w wieku 40-60 lat, z średnim czasem diagnostycznym około 4,7 lat. Kluczowe objawy to suchość oczu (xerophthalnia) i jamy ustnej (xerostomia), które znacząco obniżają jakość życia pacjentów. Objawy oczne obejmują pieczenie, swędzenie, fotofobię, niewyraźne widzenie i zapalenie powiek, natomiast objawy jamy ustnej to uczucie suchości, trudności w połykaniu, zaburzenia smaku, próchnica i owrzodzenia. Zmęczenie dotyka 70-80% pacjentów, a bóle stawów i mięśni są powszechne, często z symetrycznym zajęciem drobnych stawów i zapaleniem stawów u około 10% chorych.

Charakterystyka zespołu Sjögrena

Zespół Sjögrena jest przewlekłą, autoimmunologiczną chorobą, charakteryzującą się zapaleniem i dysfunkcją gruczołów wydzielających łzy i ślinę. Głównym mechanizmem patogenetycznym jest naciek limfocytarny gruczołów wydzielniczych, prowadzący do ich uszkodzenia i zaburzenia funkcji, co skutkuje zmniejszoną produkcją łez i śliny12. Choroba może mieć charakter pierwotny (występujący samodzielnie) lub wtórny (towarzyszący innym chorobom autoimmunologicznym, takim jak reumatoidalne zapalenie stawów czy toczeń rumieniowaty układowy)3.

Zespół Sjögrena występuje znacznie częściej u kobiet niż u mężczyzn (w stosunku 9:1), a najczęściej diagnozowany jest u osób w wieku 40-60 lat, chociaż może dotknąć również młodszych pacjentów45. Średni czas od wystąpienia pierwszych objawów do postawienia diagnozy wynosi około 4,7 lat, co związane jest z niespecyficznymi objawami, które mogą przypominać inne choroby6.

Główne objawy zespołu Sjögrena

Objawy oczne

Suchość oczu (xerophthalnia) jest jednym z głównych objawów zespołu Sjögrena. Pacjenci często opisują uczucie piasku lub ciała obcego w oku78. Inne objawy oczne obejmują:

  • Pieczenie lub swędzenie oczu
  • Zaczerwienienie oczu
  • Uczucie ziarnistości lub tarcia w oczach
  • Nadwrażliwość na światło (fotofobia)
  • Niewyraźne widzenie
  • Zmęczenie oczu
  • Zapalenie powiek (blepharitis)91011

Suchość oczu w zespole Sjögrena jest postępująca i ma znaczący negatywny wpływ na jakość życia pacjentów. Chorzy doświadczają codziennie znacznych wahań ostrości widzenia podczas mrugania, niewyraźnego widzenia, zmęczenia oczu i trudności w czytaniu, co z kolei może negatywnie wpływać na pracę zawodową i wydajność w miejscu pracy12.

Objawy w jamie ustnej

Suchość jamy ustnej (xerostomia) to drugi charakterystyczny objaw zespołu Sjögrena. Pacjenci często skarżą się na713:

  • Uczucie suchości, jakby jama ustna była wypełniona watą lub miała kredową konsystencję
  • Trudności w połykaniu, mówieniu lub jedzeniu suchych pokarmów
  • Zaburzenia smaku lub jego utratę
  • Potrzebę częstego picia płynów
  • Gęstą lub lepką ślinę
  • Owrzodzenia jamy ustnej i ból
  • Próchnicę zębów i zapalenie dziąseł
  • Chrypkę814

Niedobór śliny może prowadzić do trudności w połykaniu i mówieniu. Pacjenci często opisują, że jedzenie przyczepia się do podniebienia, język przykleja się do podniebienia, a mówienie przez dłuższy czas staje się trudne lub powoduje chrypkę8.

Zmęczenie i ból

Zmęczenie jest jednym z najczęstszych ogólnych objawów zespołu Sjögrena, występującym u około 70-80% pacjentów1516. Może być ono bardzo nasilone, wpływając na codzienne funkcjonowanie. Pacjenci często opisują zmęczenie w zespole Sjögrena jako:

  • Przytłaczające uczucie wyczerpania
  • Zmęczenie zarówno fizyczne jak i psychiczne
  • Trudności w koncentracji
  • Uczucie zmęczenia nawet po niewielkim wysiłku1718

Bóle stawów i mięśni są również powszechne u pacjentów z zespołem Sjögrena. Mogą one obejmować713:

  • Ból i sztywność stawów (najczęściej drobnych stawów, symetrycznie po obu stronach ciała)
  • Zapalenie stawów (występuje u około 10% pacjentów)
  • Bóle mięśniowe lub osłabienie mięśni
  • Sztywność poranna1619

Objawy pozagruczołowe zespołu Sjögrena

Zespół Sjögrena jest chorobą układową, która może wpływać na wiele narządów i tkanek w organizmie. Objawy pozagruczołowe (ekstraglandularne) występują u około 71% pacjentów16. Poniżej przedstawiono najczęstsze manifestacje pozagruczołowe.

Objawy skórne

U pacjentów z zespołem Sjögrena często występują problemy skórne20:

  • Suchość skóry (xeroderma)
  • Wysypki skórne
  • Świąd
  • Plamica (purpura) wyczuwalna i niewyczuwalna
  • Pokrzywka
  • Nadwrażliwość na promieniowanie UV i ciepło2119

Objawy układu oddechowego

Zaburzenia ze strony układu oddechowego występują u 9-24% pacjentów, ale subkliniczne nieprawidłowości można zidentyfikować nawet u 75% chorych19:

  • Przewlekły suchy kaszel (występuje u 40-60% pacjentów z zespołem Sjögrena)
  • Zapalenie tchawicy i oskrzeli (xerotrachea)
  • Nawracające zapalenie oskrzeli
  • Zapalenie śródmiąższowe płuc
  • Duszność
  • Zapalenie opłucnej
  • Nadciśnienie płucne2221

Objawy neurologiczne

Objawy neurologiczne występują u około 10-20% pacjentów z zespołem Sjögrena23 i mogą obejmować:

  • Zaburzenia koncentracji i pamięci („mgła mózgowa”)
  • Neuropatia obwodowa powodująca drętwienie, mrowienie lub pieczenie w kończynach
  • Neuropatia autonomiczna wpływająca na koordynację akcji serca, oddychania i perystaltyki żołądka
  • Zapalenie rdzenia kręgowego (myelitis)
  • Zapalenie nerwu wzrokowego
  • Bóle głowy242526

Neurpatia czuciowo-ataktyczna i bolesna neuropatia drobnych włókien to dwie najbardziej typowe formy neuropatii związanych z zespołem Sjögrena27.

Objawy żołądkowo-jelitowe

Problemy z przewodem pokarmowym u pacjentów z zespołem Sjögrena mogą obejmować28:

  • Trudności z połykaniem (dysfagia)
  • Refluks żołądkowo-przełykowy
  • Przewlekłe zapalenie żołądka
  • Zaburzenia trawienia (dyspepsja)
  • Zaburzenia motoryki przewodu pokarmowego
  • Zespół jelita drażliwego
  • Autoimmunologiczne zapalenie trzustki2129

Objawy układu moczowo-płciowego

U pacjentów z zespołem Sjögrena mogą wystąpić21:

  • Zapalenie śródmiąższowe pęcherza (śródmiąższowe zapalenie pęcherza moczowego)
  • Śródmiąższowe zapalenie nerek
  • Kłębuszkowe zapalenie nerek
  • Suchość pochwy u kobiet
  • Bolesne współżycie (dyspareunia)
  • Przewlekłe zapalenie prostaty u mężczyzn30

Inne objawy pozagruczołowe

Do pozostałych objawów pozagruczołowych w zespole Sjögrena należą242:

Progresja choroby i długoterminowe następstwa

Zespół Sjögrena jest chorobą przewlekłą, która postępuje bardzo powoli w czasie. Z tego powodu może upłynąć nawet 10 lat między pojawieniem się pierwszych objawów a postawieniem diagnozy33.

Przebieg choroby

Progresja zespołu Sjögrena jest bardzo zróżnicowana u poszczególnych pacjentów2:

  • U niektórych pacjentów objawy pozostają stabilne
  • U innych mogą się nasilać z czasem
  • U niektórych mogą występować okresy remisji
  • Niektórzy doświadczają cykli łagodnych i potem ciężkich objawów3435

Pacjenci zwykle doświadczają stabilnych objawów lub co najwyżej łagodnej, ciągłej progresji choroby gruczołowej, która jest główną cechą zespołu Sjögrena, z dożywotnimi objawami15. Wielu pacjentów uczy się z czasem zarządzać swoimi objawami, które mogą stać się mniej intensywne36.

Znacząca część pacjentów (50-70%) doświadcza objawów systemowych lub pozagruczołowych w momencie wystąpienia objawów gruczołowych lub w ciągu 6 miesięcy15. Pacjenci, którzy doświadczają manifestacji pozagruczołowych, mają zróżnicowany przebieg choroby, zależny od konkretnego zajętego układu narządów.

Powikłania długoterminowe

Główne przewlekłe powikłania choroby wynikają z długotrwałych problemów z nawilżeniem głównych błon śluzowych33:

  • Powikłania oczne: infekcje oczu, owrzodzenia rogówki, uszkodzenie rogówki, problemy z widzeniem3738
  • Powikłania jamy ustnej: próchnica zębów, choroby przyzębia, infekcje grzybicze jamy ustnej (kandydoza), kamienie w gruczołach ślinowych3940
  • Powikłania narządowe: niewydolność nerek, zwłóknienie płuc, problemy z układem krążenia i nerwowym41

Jeśli pewne stany układowe nie zostaną wykryte i leczone wystarczająco szybko, pacjent może rozwinąć przewlekłą niewydolność narządów, na przykład niewydolność nerek (przewlekła niewydolność nerek) lub płuc (zwłóknienie płuc)33.

Ryzyko chłoniaka

Najpoważniejszym powikłaniem zespołu Sjögrena jest rozwój chłoniaka, szczególnie chłoniaka nieziarniczego (non-Hodgkin)33. Badania wykazały, że:

  • Ryzyko rozwoju chłoniaka nieziarniczego jest około 14-44 razy wyższe u pacjentów z zespołem Sjögrena w porównaniu do populacji ogólnej4243
  • Około 5-10% pacjentów z zespołem Sjögrena rozwija jakąś formę chłoniaka4445
  • Najczęstszym podtypem histologicznym chłoniaka w zespole Sjögrena jest chłoniak MALT (mucosa-associated lymphoid tissue), który może rozwinąć się w każdej tkance infiltrowanej przez tkankę limfoidalną – najczęściej w gruczołach ślinowych46

Pacjenci z ciężkim przebiegiem choroby są znacznie bardziej narażeni na rozwój chłoniaka niż pacjenci z łagodnym lub umiarkowanym przebiegiem43. Czynniki ryzyka rozwoju chłoniaka obejmują m.in. powiększenie ślinianek, limfadenopatię, powiększenie wątroby i śledziony, plamicę, leukopenię, niewydolność nerek oraz obecność przeciwciał przeciwko SSA/SSB47.

Wpływ na jakość życia

Zespół Sjögrena może znacząco wpływać na jakość życia pacjentów5:

  • Ciągłe zmęczenie może uniemożliwiać normalne aktywności
  • Suchość oczu może powodować problemy z czytaniem i pracą przy komputerze
  • Suchość jamy ustnej utrudnia jedzenie, połykanie i mówienie
  • Ból stawów i mięśni może ograniczać aktywność fizyczną
  • Zaburzenia poznawcze mogą wpływać na pracę i codzienne funkcjonowanie4318

Około 30-40% pacjentów w wieku produkcyjnym jest zmuszonych do zaprzestania pracy, głównie z powodu zmęczenia i „mgły mózgowej”48. Spadek jakości życia jest podobny do tego obserwowanego w innych przewlekłych chorobach, takich jak reumatoidalne zapalenie stawów, toczeń i fibromialgia43.

Rokowanie w zespole Sjögrena

Ogólnie rzecz biorąc, rokowanie w zespole Sjögrena jest dobre dla większości pacjentów49. Ogólny stan zdrowia i długość życia są głównie niezmienione u pacjentów, których jedynymi objawami są suche oczy i usta50.

Czynniki wpływające na rokowanie:

  • Obecność objawów pozagruczołowych – rokowanie jest mniej korzystne u pacjentów z zajęciem narządów wewnętrznych51
  • Rozwój chłoniaka – stanowi największe zagrożenie dla życia, ale gdy zostanie wcześnie wykryty, ma względnie korzystne rokowanie z 15-letnim przeżyciem wynoszącym prawie 80%52
  • Aktywność choroby – długotrwała, bardzo aktywna choroba zwiększa ryzyko powikłań53

Przy odpowiedniej uwadze poświęconej pielęgnacji oczu i jamy ustnej, perspektywy dla pacjentów z zespołem Sjögrena są ogólnie doskonałe54. Chociaż zespół Sjögrena nie jest uleczalny, większość pacjentów może prowadzić normalne życie z odpowiednim leczeniem objawowym55.

Wnioski końcowe

Zespół Sjögrena jest złożoną, przewlekłą chorobą autoimmunologiczną, która może mieć różnorodne manifestacje kliniczne – od suchości oczu i jamy ustnej po poważne powikłania narządowe. Objawy mogą się utrzymywać na stałym poziomie, nasilać się lub w rzadkich przypadkach ulegać remisji. Choroba postępuje powoli, a wczesne rozpoznanie i odpowiednie leczenie mogą znacznie poprawić jakość życia pacjentów i zapobiec poważnym powikłaniom56.

Pacjenci z zespołem Sjögrena wymagają regularnej kontroli medycznej w celu monitorowania progresji choroby, dostosowania leczenia i wczesnego wykrycia potencjalnych powikłań. Multidyscyplinarne podejście do leczenia, obejmujące reumatologów, okulistów, stomatologów i innych specjalistów, jest kluczowe dla optymalnego zarządzania tą złożoną chorobą57.

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Sjögren’s Disease/Syndrome – Selected Immune Disorders and Disability – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK584486/
    Sjgrens disease is a chronic inflammatory autoimmune disease also referred to as Sjgrens syndrome (SS) (Baer and Hammitt, 2021). The primary disease pathogenesis includes exocrine gland inflammation and eventual dysfunction resulting in decreased tear and saliva production. The hallmark clinical manifestations of SS are xerostomia (dry mouth) and keratoconjunctivitis (dry eyes), which are termed sicca symptoms. Sicca symptoms are present in almost all adults at the time of diagnosis. Musculoskeletal pain and fatigue are also present in the majority of patients (Romo et al., 2018). […] The classic presentation of SS involves a middle-aged woman with dry eyes, oral dryness, and joint pain (Brito-Zern et al., 2017; Jonsson et al., 2018). SS is a chronic, incurable disease with a wide range of presentations. It can damage vital organs and may remain stable, worsen, or go into remission. The primary features of SS are keratoconjunctivitis (dry eyes) and xerostomia (dry mouth), as the immune system targets the salivary glands, which produce saliva, and the lacrimal glands, which produce tears. Those symptoms (dry mouth and dry eyes) are collectively referred to as sicca. Patients also may experience organ-specific systemic symptoms, known as extra-glandular disease, as well as general symptoms including fatigue, depression, and chronic musculoskeletal pain.
  • #2 Understanding Sjögren’s | Sjögren’s Foundation
    https://sjogrens.org/understanding-sjogrens
    Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas. […] As a systemic disease, affecting the entire body, symptoms may remain steady or worsen overtime. There is no one single progression of the disease and this can make it challenging for patients and their physicians. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatments are important as they may prevent serious complications and greatly improve a patient’s quality of life. […] While many patients experience dry eyes, dry mouth, fatigue and joint pain, Sjgren’s can also cause dysfunction of organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients also have a higher chance of developing lymphoma.
  • #3 Arthritis and Sjögren’s Syndrome
    https://www.webmd.com/rheumatoid-arthritis/arthritis-sjogrens-syndrome
    Sjgren’s syndrome occurs in two basic forms: primary Sjgren’s syndrome — the disease by itself and not associated with any other illness; and secondary Sjgren’s syndrome — disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, lupus, or vasculitis. […] The disease can affect people of any race or age, although the average age of onset is the late 40s. […] The diagnosis of primary Sjgren’s syndrome is based on several factors, including: Presence of dry eyes and mouth. An eye doctor can detect dry eyes by measuring tear production or by carefully examining the status of the tear film of the eye. […] Secondary Sjgren’s syndrome is generally diagnosed when someone with an established autoimmune disease, such as rheumatoid arthritis or lupus, develops extreme dryness of the eyes and mouth.
  • #4 Sjogren syndrome: Symptoms, Causes, and Treatment – DermNet
    https://dermnetnz.org/topics/sjoegren-syndrome
    Sjgren syndrome (SS) is an autoimmune disease that mainly affects secretory glands of the eyes and mouth, reducing production of tears and saliva (sicca symptoms) and resulting in dry eyes (called keratoconjunctivitis sicca) and dry mouth (xerostomia). […] Many patients with Sjgren syndrome also have features of other autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus or rheumatoid arthritis. […] Primary Sjgren syndrome is 9 times more common in females than males. […] Up to 60% of people with Sjgren syndrome will develop at least one episode of arthralgia or arthritis at some stage. Arthritis does not usually result in erosion of joint structures or deformity. […] Most sufferers have a chronic condition but their symptoms can be alleviated. They have a lower than average incidence of cardiovascular disease. Prognosis can be worsened by the development of lymphoma, neurological disease, and vasculitis affecting the kidneys and lungs.
  • #5 Sjogren Syndrome Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/332125-clinical
    The clinical presentation of Sjgren syndrome may vary. Most patients are women, and onset is usually at age 40-60 years, but the syndrome also can affect men and children. The onset is insidious. The first symptoms in primary Sjgren syndrome can be easily overlooked or misinterpreted, and diagnosis can be delayed for as long as several years. […] Xerophthalmia (dry eyes) and xerostomia (dry mouth) are the main clinical presentations in adults. Bilateral parotid swelling is the most common sign of onset in children. […] Symptoms of Sjgren syndrome can decrease the patient’s quality of life in terms of its physical, psychological, and social aspects. […] Although dry eyes and dry mouth are the most common symptoms in patients with Sjgren syndrome, most patients who report these symptoms have other underlying causes. The incidence of sicca symptoms increases with age. Indeed, more than one third of elderly persons have sicca symptoms.
  • #6 Sjögren’s Syndrome | MouthHealthy – Oral Health Information from the ADA
    https://www.mouthhealthy.org/all-topics-a-z/sjogrens-syndrome
    Sjgrens syndrome is a chronic autoimmune disorder in which the bodys immune system mistakenly attacks its own moisture-producing glands, the tear-secreting and salivary glands as well as other organs. Symptoms include: […] Dry eyes and dry mouth […] Dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system […] Extreme fatigue and joint pain. […] Because the symptoms of Sjgrens are similar to other conditions and diseases, it can be very hard to diagnose. According to the Sjgrens Syndrome Foundation, on average it takes 4.7 years to receive a diagnosis of Sjgrens.
  • #7 Sjogren’s syndrome – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/symptoms-causes/syc-20353216
    Sjogren’s (SHOW-grins) syndrome is a disorder of your immune system identified by its two most common symptoms dry eyes and a dry mouth. […] The two main symptoms of Sjogren’s syndrome are: Dry eyes. Your eyes might burn, itch or feel gritty as if there’s sand in them. Dry mouth. Your mouth might feel like it’s full of cotton, making it difficult to swallow or speak. […] Some people with Sjogren’s syndrome also have one or more of the following: Joint pain, swelling and stiffness, Swollen salivary glands particularly the set located behind your jaw and in front of your ears, Skin rashes or dry skin, Vaginal dryness, Persistent dry cough, Prolonged fatigue.
  • #8 Sjogren Syndrome Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/332125-clinical
    Patients may describe the effects dry mouth in the following ways: Inability to eat dry food (eg, crackers) because it sticks to the roof the mouth, Tongue sticking to the roof of the mouth, Putting a glass of water on the bed stand to drink at night (and resulting nocturia), Difficulty speaking for long periods of time or the development of hoarseness, Higher incidence of dental caries and periodontal disease, Altered sense of taste, Difficulty wearing dentures, Development of oral candidiasis with angular cheilitis, which can cause mouth pain. […] Dry eyes may be described as red, itchy, and painful. However, the most common complaint is that of a gritty or sandy sensation in the eyes. Symptoms typically worsen throughout the day, probably due to evaporation of the already scanty aqueous layer.
  • #9 Sjogren’s Disease Symptoms – Dry Mouth & Eyes | NIAMS
    https://www.niams.nih.gov/health-topics/sjogrens-disease
    Sjgrens disease, also known as Sjgrens and Sjgrens syndrome, is a chronic (long-lasting) autoimmune disorder that happens when the immune system attacks the glands that make moisture in the eyes, mouth, and other parts of the body. The main symptoms are dry eyes and mouth, but other parts of the body may be affected as well, with many people reporting fatigue and joint and muscle pain. […] Common symptoms include: Dry eyes. Your eyes may burn or itch or feel like they have sand in them. Sometimes, the dryness causes blurry vision or sensitivity to bright light. You may get irritated, itchy eyelids due to inflammation. Dry mouth. Your mouth may feel chalky, and you may have trouble swallowing, speaking, and tasting. Because you lack the protective effects of saliva, you may develop more dental decay (cavities) and mouth infections, such as candidiasis (also called thrush).
  • #10 Sjogren’s Syndrome | Condition | UT Southwestern Medical Center
    https://utswmed.org/conditions-treatments/sjogrens-syndrome/
    Sjogrens syndrome is an autoimmune disorder in which the immune system mistakenly attacks the glands that produce moisture, such as the tear and salivary glands, resulting in dry eyes and a dry mouth. […] In people with Sjogrens syndrome, symptoms usually affect the eyes and mouth first. Common symptoms include: […] Eye symptoms: Dry, burning, or itchy eyes; Sensation of something in the eye. […] Mouth and throat symptoms: Dry mouth; Difficulty swallowing, speaking, or eating dry foods; Hoarseness or dry cough; Mouth sores or pain; Enlargement of the salivary glands; Tooth decay and gum inflammation; Thick or stringy saliva. […] Other symptoms can include: Fatigue; Joint and muscle pain; Skin or vaginal dryness. […] Less common symptoms include: Neuropathies; Inflammation of the lungs, kidneys, or liver; Skin rashes; Thyroid gland disorders; Lymphoma.
  • #11 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. […] Sjgren’s syndrome is an autoimmune disease characterized by dryness of the mouth and eyes. […] Symptoms of Sjgren’s syndrome can involve the glands, as above, but there are also possible effects of the illness involving other organs of the body (extraglandular manifestations). […] When the tear gland (lacrimal gland) is inflamed from Sjgren’s, the resulting eye dryness can progressively lead to symptoms such as eye irritation, decreased tear production, a „gritty” sensation, and eye infection, and serious abrasion of the dome of the eye (cornea). […] Dry eyes can lead to infections of the eyes and inflammation of the eyelids (blepharitis). The condition of having dry eyes is medically referred to as xerophthalmia.
  • #12 Sjögren’s Disease/Syndrome – Selected Immune Disorders and Disability – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK584486/
    Sicca is present in approximately 90 percent of patients at the time of SS diagnosis (Brito-Zern et al., 2017). There are varying degrees of dryness at the time of diagnosis, which may be related to duration of disease prior to diagnosis, though dryness can also be severe in patients with aggressive short disease duration. […] SS-related dry eye is progressive and has a major negative impact on the quality of life of affected patients. Such patients experience, on a daily basis, significant vision fluctuation with blinking, blurred vision, eye fatigue, and difficulty reading, which in turn can have a negative impact on employment and workplace productivity. Additionally, SS can cause corneal melt and perforation, uveitis, scleritis, retinal vasculitis, and optic neuritis (Akpek et al., 2019).
  • #13 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. The most common symptom is chronic, unusual dryness in your eyes, mouth or vagina. […] Chronic dryness throughout your body is the most common Sjgrens syndrome symptom. […] The most common symptom of Sjgrens syndrome is unusual dryness, including: Dry eyes or itchy eyes. Dry mouth (xerostomia), mouth sores or thickened spit (saliva). Vaginal dryness. Dry skin. Dry nose and frequent nosebleeds. Dry throat (including coughing frequently). […] In addition to dryness, Sjgrens syndrome can cause other symptoms, including: Joint pain. Muscle pain or weakness. Swollen lymph nodes. Fatigue (feeling tired all the time). Trouble swallowing (dysphagia) or talking. Brain fog (trouble thinking or focusing). Loss of taste. Tooth decay (cavities) or loss. Skin rashes. Neuropathy. Heartburn or other types of indigestion. Light sensitivity.
  • #14 Sjogren syndrome: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/000456.htm
    Dry eyes and dry mouth are the most common symptoms of this syndrome. […] Eye symptoms: Itching eyes, Burning sensation in eyes, Feeling that something is in the eye. […] Mouth and throat symptoms: Difficulty swallowing or eating dry foods, Loss of sense of taste, Problems speaking, Thick or stringy saliva, Mouth sores or pain, Teeth decay and gum inflammation, Hoarseness. […] Other symptoms may include: Fatigue, Fever, Change in the color of hands or feet with cold exposure (Raynaud phenomenon), Joint pain or joint swelling, Swollen glands, Skin rash, Numbness and pain due to neuropathy, Cough and shortness of breath due to lung disease, Irregular heartbeat, Nausea and heartburn, Vaginal dryness or painful urination. […] The disease is most often not life threatening. The outcome depends on what other diseases you have. […] There is a higher risk for lymphoma and early death when Sjögren syndrome has been very active for a long time, as well as in people with vasculitis, low complements, and cryoglobulins.
  • #15 Sjögren’s Disease/Syndrome – Selected Immune Disorders and Disability – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK584486/
    The most prevalent general symptom in SS is fatigue, which occurs in approximately 70-80 percent of SS patients. […] SS is slowly progressive. Patients accrue advancing gland damage leading to varying levels of decreased exocrine gland function, and they may also accumulate additional autoimmune diagnoses. […] There is no cure for Sjgrens disease, and treatment generally focuses on managing symptoms without the expectation of complete disease remission. Patients typically experience stable symptoms or, at most, a mild continued progression of glandular disease, the main feature of Sjgren, with lifelong symptoms. […] A significant proportion of SS patients (50-70 percent) experience systemic symptoms or extra-glandular manifestations at the time of glandular onset or within 6 months (Parisis et al., 2020). Patients who experience extra-glandular manifestations have a varied disease course which is dependent on the specific organ system involvement.
  • #16 Reviewing primary Sjögren’s syndrome: beyond the dryness – From pathophysiology to diagnosis and treatment
    https://www.medsci.org/v14p0191.htm
    The most prevalent general symptom is fatigue, occurring in up to 70-80% of pSS patients. Fatigue in pSS has been well studied using the multidimensional fatigue inventory (MFI) on which pSS patients scores were two-fold worse on all dimensions as compared to healthy controls. In addition, chronic pain is often seen in pSS due to accompanying fibromyalgia and/or polyarthralgia. Depression and anxiety are also more common in pSS patients compared to healthy controls. […] Approximately 71% of the patients with pSS present with extraglandular manifestations. Of those, lymphoma has the highest mortality. A large cohort study reported a nearly 5-fold higher relative risk in pSS patients with a life-time risk of approximately 10%. The most common subtype is the mucosa-associated lymphoid tissue (MALT) lymphoma often seen in the parotid glands, which is usually a low-grade indolent neoplasm. Clinical risk factors include persistent, unilateral salivary gland enlargement, lymphadenopathy, splenomegaly, skin vasculitis, cryoglobulinemia and the development of glomerulonephritis.
  • #17 Sjögren disease | Causes, symptoms, treatments
    https://versusarthritis.org/about-arthritis/conditions/sjoegrens-syndrome/
    The most common symptoms are: dry eyes, a dry mouth, feeling tired and achy. […] Many people who have the condition don’t have any other symptoms. However, symptoms can vary a lot from person to person. Some people find they can manage their condition well, while others find it has a big effect on their daily life. […] Your eyes may be dry and feel sore, irritable or as if they have grit or sand in them. Some people feel discomfort under strong lights, or find their eyes become sticky with mucus. […] Your mouth may be dry and you may get mouth ulcers. Your mouth or throat might also feel sticky. […] Fatigue is an overwhelming feeling of tiredness that can happen after doing very little and has no obvious cause. You may feel extremely tired, both physically and mentally, and you might find it difficult to concentrate.
  • #18 Sjogren’s Disease Symptoms – Dry Mouth & Eyes | NIAMS
    https://www.niams.nih.gov/health-topics/sjogrens-disease
    In some cases, Sjgrens disease affects other tissues and organs and has more widespread effects on the body. These other effects may cause: Fatigue. Joint pain. Dry skin. Dry nasal passages and throat, and a dry cough. Skin rashes. Muscle aches. Acid reflux. Vaginal dryness. Swelling of the glands around the face and neck. Trouble sleeping. Poor concentration and memory problems. Numbness, tingling, and weakness, especially in the extremities. Shortness of breath or trouble breathing. Muscle weakness. […] The symptoms can be severe, with some people reporting debilitating pain and fatigue.
  • #19
    https://link.springer.com/article/10.1007/s10238-021-00728-6
    The frequency of joint pain without inflammatory signs (arthralgia) is reported up to 75% of patients, whereas overt inflammatory joint disease (arthritis) is less frequent and can be observed in approximately 10% of SS patients. […] Besides xerosis (skin dryness), which is the commonest cutaneous manifestation of SS, other manifestations of skin involvement are relatively frequent. […] Respiratory tract involvement is often described among SS patients. […] The prevalence of pulmonary involvement in patients with SS is estimated between 9 and 24%, but subclinical abnormalities of pulmonary function tests (PFTs), bronchoalveolar lavage (BAL) and computed tomography (CT) can be identified in up to 75% of patients. […] Nervous system involvement can be observed in up to 20% of SS patients, sometimes anticipating the sicca symptoms.
  • #20 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Sjgren syndrome is a systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs. The disorder most often affects women, and the median age of onset is around 50 to 60 years. Most individuals with Sjgren syndrome present with sicca symptoms, such as xerophthalmia (dry eyes), xerostomia (dry mouth), and parotid gland enlargement, which is seen in the image below. […] In addition, numerous extraglandular features may develop, such as the following: Arthralgia, Arthritis, Raynaud phenomenon, Myalgia, Pulmonary disease, Gastrointestinal disease, Leukopenia, Anemia, Lymphadenopathy, Neuropathy, Vasculitis, Renal tubular acidosis, Lymphoma. […] About 50% of patients with Sjgren syndrome have cutaneous findings, such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria.
  • #21 Symptoms | Sjögren’s Foundation
    https://sjogrens.org/understanding-sjogrens/symptoms
    Difficulty swallowing, heartburn, reflux, esophagitis. […] Profound fatigue, vasculitis, lymphomas. […] Dry skin, skin sensitivity to UV light and heat, rashes. […] Recurrent bronchitis, interstitial lung disease, pneumonia. […] Arthritis, joint/muscle pain. […] Abnormal liver function tests, chronic active autoimmune hepatitis, primary biliary cholangitis. […] Peripheral neuropathy, Raynauds. […] Stomach upset, gastroparesis, autoimmune pancreatitis. […] Irritable bowel, autoimmune gastrointestinal dysmotility. […] Interstitial cystitis, interstitial nephritis including renal tubular acidosis, glomerulonephritis. […] Vaginal dryness, vulvodynia; chronic prostatitis.
  • #22 Sjogren Syndrome Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/332125-clinical
    Patients with Sjgren syndrome may have a history of recurrent parotitis, often bilateral. […] Patients with Sjgren syndrome can develop dryness of the tracheobronchial mucosa (xerotrachea), which can manifest as a dry cough. […] Dryness of the pharynx and esophagus frequently leads to difficulty with swallowing (deglutition), in which case patients usually describe food becoming stuck in the upper throat. […] Patients with gastritis should be tested for Helicobacter pylori infection, because of its association with gastric mucosa-associated lymphoid tissue lymphomas. […] Patients with Sjgren syndrome are at increased risk for delayed gastric emptying, which can cause early satiety, upper abdominal discomfort, nausea, and vomiting. […] Pericarditis and pulmonary hypertension, with their attendant symptomatology, can occur in Sjgren syndrome.
  • #23 Neurologic Complications : Johns Hopkins Sjögren’s Center
    https://www.hopkinssjogrens.org/disease-information/sjogrens-disease/neurologic-complications/
    Neurological complications may affect 10-20% of those with Sjgrens disease and range from cognitive difficulties to burning toes and feet. […] Sjgrens disease can cause inflammation and damage to both the CNS and PNS. […] Neuropathy, which means inflammation and/or damage to the peripheral nerves, can occur in 5-10% of people with Sjgrens disease. Neuropathy can cause various symptoms, from numbness, to coldness; in its most severe, neuropathy has been described as burning, lancinating, or feeling like my skin is on fire. Neuropathy can also cause weakness and clumsiness. […] Sjgrens disease can cause nerve damage which regulates the coordination of heartbeat, respiration, and gastric motility. This is called an autonomic neuropathy. Symptoms include lightheadedness when standing, decreased or increased sweating, and feeling full despite eating small meals.
  • #24 Symptoms | Sjögren’s Foundation
    https://sjogrens.org/understanding-sjogrens/symptoms
    Sjgren’s symptoms often differ from patient to patient and can present with varying degrees of severity. […] While many patients experience dry eyes, dry mouth, fatigue and joint pain, Sjgren’s patients can also experience organ involvement, gastrointestinal issues, interstitial lung disease, vasculitis, lymphomas, and nervous system dysfunction. […] Every patient experiences Sjgren’s differently. Below is a list of symptoms to learn the impact of this disease. […] Neurological problems, concentration/memory-loss, brain fog, dysautonomia, headaches. […] Dry eyes, corneal ulcerations and infections. […] Dry nose, recurrent sinusitis, nosebleeds. […] Dry mouth, mouth sores, dental decay, difficulty with chewing, speech, taste and dentures. […] Swollen, painful parotid/salivary glands.
  • #25 Neurological Manifestations of Sjogren Syndrome
    https://www.rheumatologyadvisor.com/features/expert-insight-a-closer-look-at-neurological-manifestations-of-sjogren-syndrome/
    Sjogren syndrome is an autoimmune disease characterized by dysfunction of exocrine glands that results in symptoms of dry mouth and dry eyes. […] Primary Sjogren syndrome is characterized by xerostomia and xerophthalmia, with a range of systemic manifestations that may be more or less severe. […] Neurologic manifestations of primary Sjogren syndrome include different forms of neuropathy such as sensory ataxic neuropathy, multiple mononeuropathy, painful sensory neuropathy without sensory ataxia, multiple cranial neuropathy, trigeminal neuropathy, radiculoneuropathy, and autonomic neuropathy. […] Common symptoms of neurologic involvement secondary to Sjogren syndrome include numbness and tingling of the extremities or problems with balance, representing peripheral neuropathy, facial pain secondary to trigeminal neuralgia, and patchy painful stinging and burning of the skin caused by small fiber neuropathy.
  • #26 Neurological Manifestations of Sjogren Syndrome
    https://www.rheumatologyadvisor.com/features/expert-insight-a-closer-look-at-neurological-manifestations-of-sjogren-syndrome/
    Occasionally, there is weakness of one of the upper or lower extremities caused by motor neuropathy. […] The interval between the onset of Sjogren syndrome and the development of extraglandular neurologic symptoms is variable and can range from weeks to years. […] Mild sensory neuropathic symptoms may only require symptomatic treatment with oral medication such as gabapentin. However, more severe neurologic syndromes associated with Sjogren syndrome, such as vasculitis-associated motor weakness and demyelinating disease, are serious extraglandular manifestations that may result in poor functional outcomes and high morbidity. […] Different studies report differing numbers for age and sex prevalence. In general, the incidence is greater in women and the ratio is 30:1 (women:men) with neurological involvement, and 12:1 (women:men) with no neurological involvement in primary Sjogren syndrome.
  • #27
    https://link.springer.com/article/10.1007/s10238-021-00728-6
    The latter is usually the most involved, and sensory ataxic neuronopathy and painful small fibers neuropathy are the two most typical forms of SS-associated neuropathies. […] The prevalence of the renal disease in SS has been reported to be approximately 5%, but it is probably underestimated. […] Chronic tubulointerstitial nephritis is the predominant form of SS-associated renal involvement, which clinically translates mostly into distal (type I) renal tubular acidosis (RTA). […] Studies suggest that up to 80 percent of SS patients may experience some degree of dysphagia, with negative consequences on their quality of life. […] Lymphoma is one of the most severe complications of SS, indeed approximately 5% of SS patients develop lymphoma.
  • #28 Sjögren’s Syndrome
    https://www.uspharmacist.com/article/sj%C3%B6grens-syndrome
    Sjgren’s syndrome can affect nearly any part of the body including the respiratory tract, ears, nose, kidneys, liver, gastrointestinal tract, nervous system, muscles and joints, heart, reproductive system, and immune system. There is no way to forecast which organs or areas of the body will be affected in a particular patient. […] Patients with lung involvement can develop atelectasis, an absence of gas from part or all of the lungs; due to build up of mucus in the bronchi and lung; tracheobronchitis, interstitial lung disease; hoarseness of the voice if the larynx is involved; pleurisy; or even pulmonary hypertension. […] Patients with affected kidneys can develop interstitial nephritis. This can result in renal tubular acidosis, glomerulonephritis, nephrogenic diabetes insipidus, and, if the urinary bladder is involved, interstitial cystitis.
  • #29 Sjögren’s syndrome | Comprehensive Rheumatology
    https://comprehensiverheumatology.com/index.html@p=300.html
    Sjgrens syndrome is a chronic autoimmune disease where the immune system mistakenly attacks the moisture-producing glands of the eyes and mouth causing dry eyes and dry mouth. […] In addition to causing dry eyes and dry mouth, Sjgrens syndrome can affect other organs like the skin, lung, heart, kidney, and nerves. […] Sjgrens syndrome is a systemic disease. In mild cases in can only cause dry eyes or dry mouth. In more severe cases it can affect other organs: […] Eyes: dry eyes, eye infections, corneal ulcers […] Mouth: dry mouth, mouth sores, cavities, difficulty with chewing, speech […] Nose: dry nose, nosebleeds, chronic sinusitis […] Esophagus: difficulty swallowing, heartburn, inflamed esophagus […] Lungs: bronchitis, pneumonia, interstitial lung disease […] Stomach: upset stomach, slowed digestion, autoimmune pancreatitis
  • #30 Sjogren Syndrome Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/332125-clinical
    The occurrence of central nervous system (CNS) and spinal cord involvement in Sjgren syndrome is estimated by various studies to be 8-40%, with manifestations including myelopathy, optic neuropathy, seizures, cognitive dysfunction, and encephalopathy. […] Sensory, motor, or sensorimotor peripheral neuropathy, often subclinical, can be detected in up to 55% of unselected patients with Sjgren syndrome. […] Renal calculi, renal tubular acidosis, and osteomalacia, nephrogenic diabetes insipidus, and hypokalemia can occur secondary to tubular damage caused by interstitial nephritis, the most common form of kidney involvement in Sjgren syndrome. […] Interstitial cystitis, with symptoms of dysuria, frequency, urgency, and nocturia, is strongly associated with Sjgren syndrome. […] Nasal dryness can result in discomfort and bleeding. Women may also have a dry vagina, which can lead to dyspareunia, vaginitis, and pruritus. […] Patients with Sjgren syndrome may report fatigue, joint pain, and, sometimes, joint swelling. […] Secondary Sjgren syndrome appears late in the course of the primary disease. However, in some patients, primary Sjgren syndrome may precede SLE by many years.
  • #31 Sjögren’s Disease: Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/sjogrens-syndrome-facts-190388
    In some people, the decreased output of saliva and mucus can start to damage tissues and organs over time, leading to complications like: Tooth decay and periodontal disease; Interstitial pneumonitis (inflammation of lung tissues); Bronchiectasis (irreversible widening of airways of the lungs); Interstitial nephritis (inflammation of kidney tissues); Hypothyroidism (low thyroid function); Irritable bowel syndrome (IBS); Peripheral neuropathy (burning, numbness, or tingling sensation caused by nerve damage); Vasculitis (inflammation of blood vessels); Pregnancy complications. […] Sjgren’s disease is an autoimmune disease that affects moisture-producing glands and tissues, causing dry eyes, skin, mouth, and nose as well as fatigue, joint pain, and blurred vision. Over the long term, the disease can cause damage to the kidneys, lungs, and nerves in some people.
  • #32 Sjögren disease | Causes, symptoms, treatments
    https://versusarthritis.org/about-arthritis/conditions/sjoegrens-syndrome/
    Your joints may be painful and swollen due to inflammation, or you might feel that various parts of your body, such as your muscles, are achy and tender. […] You may feel as though other parts of the body are drier than normal, such as your bowel, bladder, vagina, skin, and air passages. […] Though the symptoms can be uncomfortable and last quite a long time, you should be able to lead the life you want if the condition is treated properly. For most people, the condition only affects the tear and saliva glands, but some people may experience other symptoms, such as fatigue and joint inflammation. […] People with Sjgren disease might be more likely to develop hypothyroidism, Raynaud’s phenomenon, inflamed blood vessels, loss of feeling in the hands and feet, IBS, kidney problems, liver problems, purple spots on the lower legs, pleurisy, and coeliac disease.
  • #33 Progression of Sjögren Syndrome | Hospital Clínic Barcelona
    https://www.clinicbarcelona.org/en/assistance/diseases/sjogren-syndrome/evolution-of-the-disease
    Sjgren Syndrome is a chronic disease that progresses very slowly over time. Because of this, there may be a period of up to 10 years between the onset of symptoms and the diagnosis of the disease. […] The main chronic complications of the disease derive from prolonged periods of hydration problems affecting the main mucous membranes. This leads to many symptoms that become chronic, including itchy eyes, a dry mouth, dry cough, or tiredness and chronic pain. […] If certain systemic conditions are not detected and treated quickly enough, the patient may develop chronic organ failure, for example, of the kidneys (chronic renal failure) or lungs (pulmonary fibrosis). […] Lymphoma is the most serious complication of the disease, and leading specialists understand the factors that can help diagnose this cancer at an early stage.
  • #34 Sjögren’s Syndrome Causes, Symptoms, and Treatments
    https://www.upmc.com/services/rheumatology/conditions/sjogrens-disease
    Just as every person is different, every person’s Sjgrens manifestations also are different. Some people have constant symptoms, while others have symptoms that come and go. Still, others have cycles of mild and serious symptoms. […] Two symptoms that people most share are: Dry eyes. Your eyes may burn, itch, or feel gritty. Your vision may be blurry, or your eyes may be sensitive to bright lights. […] Dry mouth. You may have trouble talking, swallowing, and tasting. You may have a dry tongue and throat. […] Sjgrens disease also can cause symptoms in other body parts, such as: Brain fog. […] Dry, lingering cough. […] Dry skin. […] Fatigue. […] Joint pain. […] Muscle pain. […] Numbness in feet or hands. […] Rashes. […] Swelling of the glands. […] Vaginal dryness. […] If you’re experiencing symptoms of Sjgrens Disease that are causing discomfort or affecting your activities of daily living, its time to talk to your doctor.
  • #35 Sjögren’s syndrome
    https://www.nhs.uk/conditions/sjogrens-syndrome/
    There are lots of possible symptoms of Sjgren’s syndrome. The condition affects people differently. […] Common symptoms of Sjgren’s syndrome include: dry eyes, a dry mouth, aching muscles and joints, extreme tiredness. […] Other symptoms of Sjgren’s syndrome can include: dry skin that may be itchy, a cough that does not go away, a quiet or hoarse voice, an unpleasant taste in your mouth caused by stomach acid (acid reflux), swollen glands in your cheeks, neck and under your jaw (salivary glands), vaginal dryness. […] There may be times when your symptoms get worse (flare-ups) and times when they’re better (remission). […] Sjgren’s syndrome affects everyone who has it differently. […] For example, a dry mouth can make eating difficult, and dry eyes may cause vision problems. You may also have muscle and joint pain and feel tired all the time.
  • #36 Sjögren’s Syndrome: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/4929-sjogrens-syndrome
    You should expect to manage your symptoms for a long time (maybe for the rest of your life). However, your symptoms should get less severe as you find treatments that work for you. People with Sjgrens syndrome usually find that their symptoms become less intense over time because they learn how to manage them.
  • #37 Sjogren’s syndrome – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/diagnosis-treatment/drc-20353221
    Sjogren’s syndrome can be difficult to diagnose because the signs and symptoms vary from person to person and can be similar to those caused by other diseases. […] Many people manage the dry eye and dry mouth of Sjogren’s syndrome by using over-the-counter eyedrops and sipping water more frequently. But some people need prescription medications, or even surgical procedures. […] Many Sjogren’s syndrome symptoms respond well to self-care measures. […] Dry mouth increases your risk of dental cavities and tooth loss.
  • #38
    https://www.aao.org/eye-health/diseases/what-is-sjogren-syndrome
    Sjgren’s syndrome is a common autoimmune disease, and it may sometimes be detected during a comprehensive eye exam. […] Eye-related symptoms of Sjgren’s syndrome include: Very dry eyes, Blurry vision, Burning or stinging eyes, A gritty feeling in the eye, Being very sensitive to light, Discomfort wearing contact lenses, Blepharitis. […] Other common symptoms include dry mouth and pain in your joints. […] It is important to treat the ongoing dry eye of Sjgren’s syndrome. Otherwise, your eyes will remain irritated and very uncomfortable. In rare cases, they can become infected and scarred, affecting your vision.
  • #39 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. […] Much of the delay between onset and diagnosis is related to the diverse symptoms, which make diagnosis difficult, plus a lack of awareness of the condition on the part of various healthcare professionals encountered by many Sjgren’s patients, who frequently fail to connect the dots regarding the numerous signs and symptoms affecting multiple body systems. […] Sjgren’s syndrome is mainly characterised by dry eyes and a dry mouth, and may also affect other mucosal tissues such as the nose, larynx, gastro-intestinal tract and vagina. Dry eyes can lead to blurred vision. Oral-related complications include sialoliths (salivary stones), salivary gland swelling, high caries risk, particularly at the cusp tips and cervical sites, periodontal disease, and soft tissue infections such as candidiasis, and angular cheilitis. Dysphagia might also occur, which can lead to aspiration pneumonia.
  • #40 Sjögren’s Syndrome | ColumbiaDoctors
    https://www.columbiadoctors.org/treatments-conditions/sjogrens-syndrome
    Sjogrens syndrome is systemic in nature, which means it can involve your nervous system, lungs, reproductive organs, kidneys, and blood. Late onset symptoms can include: […] In the short term, there are consequences of dry eye and mouth for people with Sjgrens syndrome. Dental cavities can form because without the protective benefit of saliva, teeth are more prone to decay. Similarly, oral yeast infections may develop because of insufficient saliva. Dry eyes can lead to vision problems such as blurred vision, corneal ulcers, and light sensitivity. […] Over time, the immune system for people with Sjogren’s syndrome can damage other parts of your body, including joints, nerves, skin, and organs such as kidneys, liver, and lungs. These long-term consequences can be quite debilitating. Patients with Sjgrens are also more likely to develop lymph node cancer (lymphoma) which can be fatal.
  • #41 Sjogren’s syndrome | Better Health Channel
    https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/sjogrens-syndrome
    Complications can include: increased risk of dental decay, increased risk of developing thrush infections in the mouth, vision problems, inflammation of internal organs (such as the kidneys, lungs or liver), problems with the circulatory and nervous system. […] There is no cure for Sjogren’s syndrome, but it can be managed effectively.
  • #42 Sjogren’s Syndrome – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/ddi/sjogrens-syndrome/
    Risk factors for a poor prognosis in patients with primary Sjgrens syndrome include presence of extra-glandular manifestations, malignant lymphoma particularly B-cell lymphoma, hematological malignancies, primary biliary cirrhosis, autoimmune hepatitis, pulmonary arterial hypertension, and amyloidosis. […] The presence of Sjgrens syndrome is considered a high-risk category in pregnancy, because the disease can cause complications resulting in premature deliveries and spontaneous abortions. Sjgrens syndrome is likely to worsen during the postpartum period as women have an increased risk of experiencing pulmonary hypertension. […] Patients with primary Sjgrens syndrome are at a 14-fold increased risk of developing lymphomas, particularly B-cell lymphoma, compared with patients without Sjgrens syndrome. Chronic swelling of the salivary gland may require an open biopsy.
  • #43 Sjögren’s disease – Wikipedia
    https://en.wikipedia.org/wiki/Sj%C3%B6gren%27s_disease
    Results from a number of studies indicate that, compared to other autoimmune diseases, Sjgren’s disease is associated with a notably high incidence of non-Hodgkin lymphoma, a cancer of white blood cells. About 5% of patients with SS develop some form of lymphoid malignancy. Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases. […] The reduction in QoL is similar to that seen in other chronic conditions such as rheumatoid arthritis, lupus and fibromyalgia.
  • #44 Sjögren’s Syndrome
    https://www.uspharmacist.com/article/sj%C3%B6grens-syndrome
    The entire length of the gastrointestinal tract can be involved as well. Symptoms of dryness begin in the mouth, followed by effects in the stomach and pancreas. Patients may have difficulty swallowing food and can experience gastric reflux and chronic atrophic gastritis that can cause dyspepsia. […] Patients with Sjgren’s syndrome are at an increased risk for the development of lymphoma. It is estimated that between 2% and 10% of patients will develop lymphoma. One study found that patients with Sjgren’s syndrome have up to a 44-fold increased risk of developing lymphoma, compared with the general population.
  • #45
    https://link.springer.com/article/10.1007/s10238-021-00728-6
    Ocular and/or mouth dryness are the symptoms most frequently complained by SS patients. Virtually all patients report at least one of them (98%), while 89% present both symptoms. […] Symptoms of decreased salivary production include dysphagia, dysgeusia (altered taste sensation), pain and burning sensation. […] Patients usually complain of dry food swallowing impairment or the inability of speaking for long periods without drinking liquids. […] Glandular swelling usually involves parotid glands; however, submandibular or sublingual glands may be affected too. […] Fatigue is reported by about 70-80% of SS patients, and it often has a negative impact on their quality of life. […] Musculoskeletal involvement, comprehending myalgia, arthralgia and morning stiffness are present in the majority of SS patients.
  • #46 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Morbidity associated with Sjgren syndrome is mainly associated with the gradually decreased function of exocrine organs, which become infiltrated with lymphocytes. […] The increased mortality rate associated with the condition is primarily related to disorders commonly associated with Sjgren syndrome, such as SLE, RA, and primary biliary cirrhosis. […] Among patients with Sjgren syndrome, the incidence of non-Hodgkin lymphoma is 4.3% (18.9 times higher than in the general population), with a median age at diagnosis of 58 years. The mean time to the development of non-Hodgkin lymphoma after the onset of Sjgren syndrome is 7.5 years. […] The most common histologic subtype of non-Hodgkin lymphoma in Sjgren syndrome is mucosa-associated lymphoid tissue (MALT) lymphoma, which can develop in any nonlymphoid tissue infiltrated by periepithelial lymphoid tissue most commonly the salivary glands, but also the stomach, nasopharynx, skin, liver, kidneys, and lungs. The progression of these infiltrates to lymphoma occurs slowly and in a stepwise fashion.
  • #47 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Risk factors for lymphoma include the following: Salivary gland enlargement, Regional or generalized lymphadenopathy, Hepatosplenomegaly, Palpable purpura, Leukopenia, Renal insufficiency, Loss of a previously positive polyclonal gammopathy, Development of a monoclonal gammopathy or a monoclonal cryoglobulinemia, RF positivity, Anti-SSA/SSB positivity, Hypocomplementemia. […] Women with Sjgren syndrome are at higher risk for experiencing complications during pregnancy. Worsening of pulmonary hypertension and increased rates of spontaneous abortion and preterm deliveries have been reported. […] Children born to mothers with antibodies against SSA/Ro and SSB/La are at an increased risk of neonatal lupus and congenital heart block.
  • #48 Sjogren’s is Serious | sjogrensadvocate
    https://www.sjogrensadvocate.com/serious
    Permanent loss of structure or function may impact almost any part of the body. A few examples of irreversible damage from Sjogrens include loss of teeth, severe corneal damage, scarred lung tissue, or loss of sensation from nerve damage. […] Unpleasant or painful symptoms reduce the quality of life of most Sjogrens patients. Common reasons for discomfort include flu-like fatigue, muscle and joint pain, nausea, difficulty swallowing, chronic cough, and neuropathy pain. […] Many Sjogrens patients lose their ability to work or keep up with social activities or the demands of home life and parenting. An estimated 30-40 % of working-age patients are forced to stop working, mostly because of fatigue and brain fog. […] Sjogrens patients usually experience ongoing disease activity. This sometimes results in permanent loss of function and irreversible disease damage. Sjogrens tends to progress slowly, although sudden changes in disease status may occur.
  • #49 Sjogren Syndrome: Practice Essentials, Etiology, Epidemiology
    https://emedicine.medscape.com/article/332125-overview
    Importantly, classic clinical features of Sjgren syndrome may also be seen in infections with certain viruses. These include hepatitis C virus, human immunodeficiency virus (HIV), and human T-cell lymphotrophic virus (HTLV). […] Most patients with primary Sjogren syndrome have two specific antibodies: against Ro (SS-A) and La (SSB) antigens. […] Treatment for Sjgren syndrome is largely based on symptoms (eg, lotion for dry skin, artificial tears for dry eyes). […] Sjgren syndrome carries a generally good prognosis. In patients who develop a disorder associated with Sjgren syndrome, the prognosis is more closely related to the associated disorder (eg, SLE, lymphoma). […] Although salivary and lacrimal function generally stabilize, the presence of SSA and/or hypocomplementemia may predict a decline in function.
  • #50 Sjögren Syndrome – Bone, Joint, and Muscle Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/bone-joint-and-muscle-disorders/systemic-rheumatic-diseases/sj%C3%B6gren-syndrome
    In other people, many organs are affected. Sjgren syndrome can dry out the skin and the mucous membranes lining the nose, throat, digestive tract, voice box, windpipe, airways of the lungs, vulva, and vagina. Dryness of the vulva and vagina can make sexual intercourse painful. Dryness of the trachea can cause cough and problems with how the vocal cords function. Nerve, lung, and other tissues may be affected by the inflammation. […] Joint inflammation (arthritis) occurs in approximately 20% of people, affecting the same joints that rheumatoid arthritis affects, but the joint inflammation of Sjgren syndrome tends to be milder and is usually not destructive. […] Fatigue is common in people with Sjgren syndrome. […] The prognosis is generally good. Overall health and life expectancy are mainly unaffected in people whose only symptoms are dry eyes and mouth. However, death occasionally occurs if the lungs are very damaged and, rarely, if kidney failure or lymphoma develops.
  • #51 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 often affects the eyes or mouth initially and sometimes exclusively (sicca syndrome). Dry eyes can cause a sandy, gritty sensation without pruritus. In advanced cases, the cornea is severely damaged, epithelial strands hang from the corneal surface (keratitis filiformis), and vision can be impaired. Diminished saliva (xerostomia) results in difficulty chewing and swallowing, secondary Candida infection, tooth decay, and calculi in the salivary ducts. Taste and smell may be diminished. […] Dryness may also develop in the skin and in mucous membranes of the nose, throat, larynx, bronchi, vulva, and vagina. Dryness of the respiratory tract may cause cough and dysphonia. […] Fatigue is often present but is not a specific feature of the disease. […] Sjgren syndrome is chronic. Overall health and life expectancy are mainly unaffected in patients whose only symptoms are dry eyes and mouth. However, prognosis is less favorable in those with systemic organ involvement. In those with severe disease, death may occasionally result from pulmonary involvement and, rarely, from renal failure or lymphoma. Associated systemic autoimmune disorders may dictate prognosis.
  • #52 Reviewing primary Sjögren’s syndrome: beyond the dryness – From pathophysiology to diagnosis and treatment
    https://www.medsci.org/v14p0191.htm
    By performing the ESSDAI in pSS patients on a regular basis, all the above discussed systemic manifestations can be recognized. pSS is also associated with hepatitis C (12%), autoimmune thyroid disease (10%), autoimmune chronic active hepatitis (2%) and primary biliary cirrhosis (5%), but the ESSDAI does not include these diseases. […] Patients with pSS should be closely monitored to evaluate the development of systemic manifestations and the effects of treatment. Compared to the general population, pSS patients have an increased mortality risk. The standardized mortality ratio (ratio of observed deaths in the study group to expected deaths in the general population, SMR) of pSS patients is on average 2.86, showing that pSS has an impact on patients’ survival. The leading cause of mortality in pSS is lymphoma with a lymphoma-specific SMR of 7.89, associating lymphoproliferative disorders directly with death in pSS. However, once lymphoma is diagnosed, the prognosis is relatively favourable with a 15-year survival of almost 80%. Other causes of death in pSS include vasculitis, renal failure due to glomerulonephritis and infections after the administration of immunosuppressive medication. Morbidity in pSS is mainly due to extreme fatigue and the presence of systemic manifestations and should be evaluated for each patient individually. Patients with systemic complications and lymphoma development have an increased mortality risk. Therefore, risk factors (clinical and biological) for lymphoma and other organ involvement (e.g. pulmonary function, renal function, neurological evaluation) should be assessed frequently.
  • #53 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. […] Dry eyes and dry mouth are the most common symptoms of this syndrome. Eye symptoms: Itching eyes, Burning sensation in eyes, Feeling that something is in the eye. Mouth and throat symptoms: Difficulty swallowing or eating dry foods, Loss of sense of taste, Problems speaking, Thick or stringy saliva, Mouth sores or pain, Teeth decay and gum inflammation, Hoarseness. Other symptoms may include: Fatigue, Fever, Change in the color of hands or feet with cold exposure (Raynaud phenomenon), Joint pain or joint swelling, Swollen glands, Skin rash, Numbness and pain due to neuropathy, Cough and shortness of breath due to lung disease, Irregular heartbeat, Nausea and heartburn, Vaginal dryness or painful urination. […] The disease is most often not life threatening. The outcome depends on what other diseases you have. There is a higher risk for lymphoma and early death when Sjögren syndrome has been very active for a long time, as well as in people with vasculitis, low complements, and cryoglobulins.
  • #54 Sjögren’s Syndrome: Causes, Symptoms, Treatment, Diet
    https://www.medicinenet.com/sjogrens_syndrome/article.htm
    Extraglandular (outside of the glands) problems in Sjgren’s syndrome include fatigue, joint pain or inflammation (arthritis), Raynaud’s phenomenon, lung inflammation, lymph node enlargement, and kidney, nerve, joint pain, and muscle disease with muscle pain and weakness. […] A rare serious complication of Sjgren’s syndrome is inflammation of the blood vessels (vasculitis), which can damage the tissues of the body that are supplied by these vessels. […] With proper attention to eye and oral care, the outlook for patients with Sjgren’s syndrome is generally excellent. […] Eye dryness can lead to serious injury to the eye, particularly the cornea, and this should be avoided by consultation with an eye specialist. […] Mouth dryness can lead to dental decay and parotid gland infection and parotid stones. Optimal oral hygiene is essential. […] The serious complications of primary biliary cirrhosis and lymphoma can dramatically affect prognosis and are monitored for in routine regular office visits.
  • #55 Sjögren’s Disease
    https://rheumatology.org/patients/sjogrens-disease
    Dryness in the eyes and mouth, due to inflammation affecting the tear ducts and salivary glands, are the most common symptoms of Sjgrens disease. […] Other common symptoms include joint and muscle pain, severe fatigue, skin dryness, vaginal dryness and rashes. […] Dry eyes and dry mouth are common symptoms of Sjgrens disease. […] The eyes may turn red, burn or feel gritty like sand sensation. […] It may be hard to eat dry foods or swallow because of lack of saliva. […] Other symptoms include dryness of skin, vagina, nasal passages and throat; dry cough, changes in voice, heartburn; joint and muscle pains; fatigue; and rashes. […] Because of chronic dryness, patients may be at risk for eye infections or cornea damage, tooth decay or gum disease, and vaginal yeast infections. […] Symptoms can range, but patients can often lead a normal life.
  • #56 Sjögren’s – Autoimmune Association
    https://autoimmune.org/disease-information/sjogrens/
    Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies, and lymphomas. […] As a systemic disease, affecting the entire body, symptoms may remain steady or worsen overtime. There is no one single progression of the disease and this can make it challenging for patients and their physicians. […] While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatments are important as they may prevent serious complications and greatly improve a patient’s quality of life.
  • #57 Overview of the management and prognosis of Sjögren’s disease – UpToDate
    https://www.uptodate.com/contents/overview-of-the-management-and-prognosis-of-sjogrens-disease
    Sjögren’s disease (SjD) is a chronic, multisystem autoimmune disorder characterized by lacrimal and salivary gland inflammation, with resultant dryness of the eyes and mouth and occasional glandular enlargement. In addition, a variety of systemic (so-called „extraglandular”) manifestations may occur, including fatigue, musculoskeletal symptoms, rashes, and internal organ (eg, pulmonary, kidney, hepatic, and neurologic) disease. There is also increased risk of non-Hodgkin B-cell lymphoma. […] The goals of therapy in patients with Sjögren’s disease (SjD) are to ameliorate symptoms of dry eye and mouth, prevent complications of mucosal dryness (such as dental decay, corneal ulceration, or oral candidal infection), and detect and manage systemic manifestations and glandular and lymphoproliferative disease.