Overview
Primary Sjögren disease (also called primary Sjögren's syndrome or pSS) is a chronic autoimmune condition in which the body's immune system mistakenly attacks its own moisture-producing glands, especially the tear glands and salivary glands. This leads to the hallmark symptoms of persistent dry eyes and dry mouth. However, Sjögren disease is much more than just dryness — it is a systemic condition that can affect many parts of the body, including the joints, skin, lungs, kidneys, nervous system, and blood vessels. The disease is called "primary" when it occurs on its own, rather than alongside another autoimmune condition like rheumatoid arthritis or lupus. It predominantly affects women, with about 9 out of 10 patients being female, and it most commonly develops in middle adulthood, typically between ages 40 and 60. Fatigue is one of the most common and debilitating symptoms, often significantly affecting quality of life. There is currently no cure for primary Sjögren disease. Treatment focuses on managing symptoms and preventing complications. This includes artificial tears and saliva substitutes for dryness, medications to stimulate saliva production (such as pilocarpine and cevimeline), and immunosuppressive drugs for more serious organ involvement. Patients with Sjögren disease also have a slightly increased risk of developing a type of cancer called non-Hodgkin lymphoma, so regular monitoring is important. With proper management, most people with Sjögren disease can lead full and active lives, though the chronic nature of the condition requires ongoing care.
Also known as:
Key symptoms:
Persistent dry eyes that feel gritty or burningDry mouth making it hard to swallow or speakSevere fatigue that does not improve with restJoint pain and stiffnessSwollen salivary glands (near the jaw and ears)Dry skinVaginal drynessPersistent dry coughDifficulty concentrating or brain fogNumbness or tingling in hands and feetSkin rashes, especially on the legsFrequent dental cavities and tooth decayRecurrent oral yeast infections (thrush)Muscle pain
Clinical phenotype terms (50)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsCentral Hospital, Nancy, France
Shanghai Escugen Biotechnology Co., Ltd — PHASE2
Christian von Buchwald — PHASE2
Lubris Bio Pty Ltd — PHASE2
Guangdong ProCapZoom Biosciences Co., Ltd. — EARLY_PHASE1
Peking Union Medical College Hospital — NA
Koite Health Oy — NA
Pamukkale University
argenx — PHASE3
Resolve Therapeutics — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Primary Sjögren disease.
12 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Primary Sjögren disease.
Community
No community posts yet. Be the first to share your experience with Primary Sjögren disease.
Start the conversation →Latest news about Primary Sjögren disease
Disease timeline:
New recruiting trial: Exploring the Novel Value of PSMA PET in Sjögren's Syndrome: Integrated Analysis With Established FAPI PET Imaging
A new clinical trial is recruiting patients for Primary Sjögren disease
New recruiting trial: Construction of Clinical Model of Primary Sjogren's Syndrome Combined Traditional Chinese and Western Medicine
A new clinical trial is recruiting patients for Primary Sjögren disease
New recruiting trial: Medico-economic and Quality of Life Impact of Sjogren-associated Small Fiber Neuropathy
A new clinical trial is recruiting patients for Primary Sjögren disease
New recruiting trial: A Study of RSLV-132 in Females With Sjögren's Disease
A new clinical trial is recruiting patients for Primary Sjögren disease
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What type of Sjögren disease do I have, and how severe is my case?,Which of my organs are affected, and how will you monitor for complications?,What is my risk of developing lymphoma, and how will you screen for it?,What treatments do you recommend for my specific symptoms, and what are the side effects?,Should I see other specialists, such as an eye doctor or dentist, and how often?,Are there any clinical trials or newer treatments I should consider?,What lifestyle changes can I make to help manage my symptoms and protect my teeth and eyes?
Common questions about Primary Sjögren disease
What is Primary Sjögren disease?
Primary Sjögren disease (also called primary Sjögren's syndrome or pSS) is a chronic autoimmune condition in which the body's immune system mistakenly attacks its own moisture-producing glands, especially the tear glands and salivary glands. This leads to the hallmark symptoms of persistent dry eyes and dry mouth. However, Sjögren disease is much more than just dryness — it is a systemic condition that can affect many parts of the body, including the joints, skin, lungs, kidneys, nervous system, and blood vessels. The disease is called "primary" when it occurs on its own, rather than alongsid
How is Primary Sjögren disease inherited?
Primary Sjögren disease follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Primary Sjögren disease typically begin?
Typical onset of Primary Sjögren disease is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Primary Sjögren disease?
Yes — 12 recruiting clinical trials are currently listed for Primary Sjögren disease on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Primary Sjögren disease?
21 specialists and care centers treating Primary Sjögren disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.