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Sjögren’s syndrome

Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva

Prevalence

7-10/100,000

23,170 - 35,945

Us Estimated

33,100 - 51,350

Europe estimated

Age of Onset

ICD-10

M35. 0

Inheritance

This condition does not appear to have a clear pattern of inheritance.

Rare View

Sjögren’s syndrome is a chronic 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. In addition, the disease can damage the lungs, kidneys, and nervous system. Sjögren’s syndrome predominantly affects women.

5 Facts you should know

FACT

1

Autoimmune disease that affects the body's moisture-producing glands

FACT

2

The hallmark symptom of Sjögren syndrome is dry mouth and keratoconjunctivitis sicca

FACT

3

Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems

FACT

4

Sjögren's syndrome may be associated with other autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or systemic sclerosis

FACT

5

Those affected are also at an increased risk (15%) of lymphoma

Sjögren’s syndrome is also known as...

Sjögren’s syndrome is also known as:

SjS, SS 

What’s your Rare IQ?

Which famous female tennis player was diagnosed with Sjögren’s syndrome at age 31?

Common signs & symptoms

The most common symptoms are:

  • 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. You may develop more cavities and mouth infections.

In some cases, Sjögren’s syndrome affects other tissues and organs and has more widespread effects on the body. These other effects may cause:

Fatigue (feeling very tired)

Joint pain and muscle aches and weakness

Dry skin, nasal passages, and throat, and a dry cough

Skin rashes

Swelling of the glands around the face and neck

Trouble sleeping

Poor concentration and memory problems

Numbness, tingling, and weakness, especially in your arms and legs

Shortness of breath or trouble breathing

Source: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Current treatments

There is no cure for Sjögren’s syndrome, so treatment focuses on relieving symptoms and preventing complications. Treatments are different for each person and will depend on which parts of the body are affected.

Eye treatments:

Eye drops (artificial tears)

Some people need prescription eye drops that contain medications to suppress the immune system and reduce inflammation in the eye.

Eye ointments

Punctal occlusion

Small plugs placed in the tear duct in the corners of the eyes block drainage and keep tears in the eyes longer.

Mouth treatments:

Artificial saliva

Saliva production stimulators

Cevimeline (Evoxac)

Pilocarpine (Salagen)

Anti-fungal medications

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsLocationsMore Information
A Study to Evaluate the Efficacy and Safety of VIB4920 in Participants With Sjögren's SyndromeThe purpose of the study is to evaluate the efficacy, safety, and tolerability of VIB4920 (formerly MEDI4920) in adult participants with Sjögren's Syndrome (SS). The study will enroll 2 SS populations: Population 1 will include participants with moderate to high systemic disease activity defined by European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) >= 5; Population 2 will include participants with moderate to severe subjective symptoms defined by EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) score >= 5 and residual stimulated salivary flow but with mild systemic disease activity defined by ESSDAI score < 5. Phase 2RecruitingDrug: VIB4920|Drug: PlaceboResearch Site, Fullerton, California, United States|Research site, Upland, California, United States|Research Site, Lawrenceville, Georgia, United States|Research Site, Kansas City, Kansas, United States|Research Site, Baltimore, Maryland, United States| More sites available. Please see the more information link. More Info
A Study of Nipocalimab in Adults With Primary Sjogren's Syndrome (pSS)Phase 2RecruitingOther: Placebo|Drug: Nipocalimab|Drug: Standard of Care TreatmentArizona Arthritis and Rheumatology Research, PLLC, Glendale, Arizona, United States|Arizona Arthritis & Rheumatology Research, PLLC, Mesa, Arizona, United States|St. Jude Heritage Medical Group, Fullerton, California, United States|Inland Rheumatology Clinical Trials, Inc., Upland, California, United States|More sites available. Please see the more information link. More Info
Study of Safety and Efficacy of Multiple Doses of CFZ533 in Two Distinct Populations of Patients With Sjögren's SyndromePhase 2RecruitingDrug: CFZ533|Other: PlaceboNovartis Investigative Site, Duluth, Georgia, United States|Novartis Investigative Site, Indianapolis, Indiana, United States|Novartis Investigative Site, Baton Rouge, Louisiana, United States|More sites available. Please see the more information link. More Info
A Phase 2 Study to Evaluate the Safety and Efficacy of LOU064 in Patients With Moderate to Severe Sjögren's SyndromePhase 2RecruitingDrug: LOU064|Drug: PlaceboNovartis Investigative Site, Boston, Massachusetts, United States|Novartis Investigative Site, Oklahoma City, Oklahoma, United States|Novartis Investigative Site, San Antonio, Texas, United States|More sites available. Please see the more information link. More Info
Tivanisiran for Dry Eye in Subjects With Sjögren's SyndromePhase 3RecruitingDrug: Tivanisiran sodium ophthalmic solution|Drug: Vehicle ophthalmic solutionSylentis Investigative Site, Scottsdale, Arizona, United States|Sylentis Investigative Site, Glendale, California, United States|Sylentis Investigative Site, Los Angeles, California, United States|Sylentis Investigative Site, Newport Beach, California, United States|Sylentis Investigative Site, Rancho Cordova, California, United States|More sites available. Please see the more information link. More Info
Safety, Tolerability, Pharmacokinetics, and Therapeutic Efficacy of SAR441344 in Primary Sjögren's Syndrome (pSjS)Phase 2RecruitingDrug: SAR441344|Drug: PlaceboInvestigational Site Number :8400005, DeBary, Florida, United States|Investigational Site Number :8400001, Duncansville, Pennsylvania, United States|Investigational Site Number :0320002, Caba, Buenos Aires, Argentina|More sites available. Please see the more information link. More Info
Efficacy and Safety of S95011 in Primary Sjögren's Syndrome PatientsPhase 2RecruitingDrug: S95011 concentrate for solution for infusion|Drug: Placebo concentrate for solution for infusionAttune Health, Beverly Hills, California, United States|RASF, Boca Raton, Florida, United States|Tufts School of Dental Medicine, Boston, Massachusetts, United States|Monash Health, Clayton, Australia|More sites available. Please see the more information link. More Info
Study to Assess Safety and Effectiveness of Branebrutinib Treatment in Participants With Active Systemic Lupus Erythematosus or Primary Sjögren's Syndrome, or Branebrutinib Treatment Followed by Open-label Abatacept Treatment in Study Participants With Active Rheumatoid ArthritisPhase 2RecruitingDrug: branebrutinib|Drug: abatacept|Drug: branebrutinib placeboArizona Arthritis and Rheumatology Research, Phoenix, Arizona, United States|Medvin Clinical Research - Covina Office, Covina, California, United States|TriWest Research Associates, El Cajon, California, United States|Encino Research Center, Encino, California, United States||More sites available. Please see the more information link. More Info

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyCompany ContactClinical StudiesMore Information
Dazodalibep (HZN - 4920)A fusion protein binding CD40L on T cells, blocking their interaction with CD40-expressing B cells.Phase 2Horizon TherapeuticsStudy Director 1-866-479-6742 [email protected]More InfoMore Info

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