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ORPHA:289390

Primary Sjögren disease

high confidenceDisorder

Also known as: Primary SjD · Primary Sjögren syndrome

Publications

21,044

97.7th percentile

Trials

184

Interventional, condition-specific

Researchers

1,080

Distinct authors in sample

Gene link

—

Readiness

5/6

Stages with a signal

Clinical definition (Orphanet)

A rare systemic autoimmune disease characterized by exocrine gland dysfunction, resulting predominately in keratoconjunctivitis sicca and xerostomia, but also affecting exocrine glands of the skin, as well as respiratory, urogenital, and digestive tract. Extraglandular manifestations include arthritis, interstitial lung disease, renal disease, and peripheral . The disease is accompanied by a substantially increased risk to develop B-cell non-Hodgkin lymphoma, especially MALT (mucosa-associated lymphoid tissue) lymphoma.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (7)

Sjogren syndrome · Sjögren syndrome · Sjögren-Gougerot syndrome · primary Sjogren-Gougerot syndrome · primary Sjögren-Gougerot syndrome · sicca syndrome · syndrome, Sjogren's

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

5/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    21,044 matched papers (12,424 in last 10 years) Source

  3. Phenotype characterisedPresent

    100 HPO annotations (e.g. Anxiety; Usual interstitial pneumonia; Anti-salivary protein antibody positivity) Source

  4. Animal modelPresent

    40 genotype models (Mus musculus) Source

  5. Orphan designationPresent

    2 FDA designations (1 FDA orphan-indication approval) — e.g. Bromhexine Source

  6. Interventional trialPresent

    184 matched on ClinicalTrials.gov (43 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

100

Associated phenotypes · MONDO:0010030

  • Anxiety
  • Usual interstitial pneumonia
  • Anti-salivary protein antibody positivity
  • Anti-carbonic anhydrase VI antibody positivity
  • Glomerulonephritis

Showing 5 of 100 — open Monarch for the full list.

Animal models (Monarch / Alliance)

40

Model associations linked to this Mondo ID

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

2

Designations · 1 with FDA orphan-indication approval

  • FDA BromhexineKeratoconjunctivitis Sicca Sjogren Syndrome · 1989-05-15 · Not FDA Approved for Orphan Indication
  • FDA Pilocarpine HCl (Salagen)Keratoconjunctivitis Sicca Xerostomia Sjogren Syndrome · 1992-02-28

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

82

Drugs / clinical candidates · MONDO_0010030

CTD chemicals (MyDisease.info)

18 associated chemicals · 115 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Anethole Trithione · therapeutic
  • Bromhexine · therapeutic
  • bufotalin · therapeutic
  • CHOP protocol · therapeutic
  • Hydroxychloroquine · therapeutic
  • Methotrexate · therapeutic
  • Nandrolone Decanoate · therapeutic
  • Nizatidine · therapeutic
  • Pilocarpine · therapeutic
  • Prednisolone · therapeutic
  • Sodium Bicarbonate · therapeutic
  • Sodium Citrate · therapeutic

Pathways: Glycerolipid metabolism; Glycerophospholipid metabolism; Metabolic pathways; Basal transcription factors; cGMP-PKG signaling pathway; Cytokine-cytokine receptor interaction; Chemokine signaling pathway; NF-kappa B signaling pathway

MyDisease.info · MONDO:0010030

Literature

Is anyone studying this?

21,044

21,044 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

21,044 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

12,424 in the last 10 years · high confidence · 97.7th percentile (publications denominator)

Phrase hits: 20,683 · MeSH hits: 516

Open Europe PMC search

Who's working on it?

1,080

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Wang Y11 papers · 2026

    Department of Rheumatology and Immunology, Huangshan City People's Hospital, Huangshan, Anhui, China.

    Papers in Europe PMC
  2. 02
    Liu Y9 papers · 2026

    Department of Rheumatology and Clinical Immunology, the First Affiliated Hospital of Xiamen University, Xiamen 361000, Fujian, China.

    Papers in Europe PMC
  3. 03
    Zhang Y8 papers · 2026

    Department of Immunology, School of Basic Medical Sciences, NHC Key Laboratory of Medical Immunology, Medicine Innovation Center for Fundamental Research on Major Immunology-related Diseases, Peking University, Beijing 100191, China.

    Papers in Europe PMC
  4. 04
    Li Y6 papers · 2026

    Department of Rheumatology, Shanghai Guanghua Hospital of Integrative Medicine, Shanghai University of Traditional Chinese Medicine, Shanghai, China.

    Papers in Europe PMC
  5. 05
    Liu C6 papers · 2026

    Department of Clinical Laboratory, Peking University People's Hospital, #11 Xizhimen South Street, Beijing, 100044, China. liuchen-best@pku.edu.cn.

    Papers in Europe PMC
  6. 06
    Wang J6 papers · 2026

    Department of Emergency, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China.

    Papers in Europe PMC
  7. 07
    Wang S6 papers · 2026

    Department of Emergency, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China.

    Papers in Europe PMC
  8. 08
    Wang X6 papers · 2026

    Department of Rheumatology and Immunology, School of Medicine, Tongji Hospital, Tongji University, No. 389 Xincun Road, Shanghai, 200065, China. xuan2018@tongji.edu.cn.

    Papers in Europe PMC
  9. 09
    Chen Y5 papers · 2026

    Institute of Clinical Pharmacology, School of Pharmacy, Anhui Medical University, Key Laboratory of Anti-inflammatory and Immune Medicine (Anhui Medical University), Ministry of Education, Hefei, 230032, China.

    Papers in Europe PMC
  10. 10
    Jin Y5 papers · 2026

    Department of Rheumatology, Shanghai Guanghua Hospital of Integrative Medicine, Shanghai University of Traditional Chinese Medicine, Shanghai, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

184

interventional trials for this specific condition

184 interventional trials matched this specific condition name; 43 currently recruiting in our sample.

Data as of 11 September 2026

184 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.2th percentile).

high confidence · 99.2th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

184 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

92 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

General rare disease registries you may be eligible for

These studies enroll across many rare conditions. They are not counted as evidence that anyone is studying this specific disease.

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 32 · after dedupe 32 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 32 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (32)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Primary Sjögren disease — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Primary Sjögren disease" OR "Primary SjD" OR "Primary Sjögren syndrome" OR "Sjogren syndrome" OR "Sjögren syndrome" OR "Sjögren-Gougerot syndrome" OR "primary Sjogren-Gougerot syndrome" OR "primary Sjögren-Gougerot syndrome" OR "sicca syndrome" OR "syndrome, Sjogren's"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Sjogren's Syndrome

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Primary Sjögren disease" OR "Primary SjD" OR "Primary Sjögren syndrome" OR "Sjogren syndrome" OR "Sjögren syndrome" OR "Sjögren-Gougerot syndrome" OR "primary Sjogren-Gougerot syndrome" OR "primary Sjögren-Gougerot syndrome" OR "sicca syndrome" OR "syndrome, Sjogren's" OR "Sjogren's Syndrome"

Interventional trials matched via: both, phrase, mesh (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 184 interventional · 92 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T12:13:18.581Z