RARE DISEASERESEARCH ATLAS

ORPHA:79078

IgG4-related dacryoadenitis and sialadenitis

high confidenceSubtype of disorder

Also known as: Chronic dacryoadenitis and sialadenitis · Mikulicz disease

Publications

801

81.5th percentile

Trials

0

Interventional, condition-specific

Researchers

1,040

Distinct authors in sample

Gene link

Readiness

1/6

Stages with a signal

Clinical definition (Orphanet)

IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease characterized by persistent, usually painless, bilateral enlargement of the lacrimal, parotid, and submandibular glands associated with elevated levels of serum immunoglobulin (Ig) G4 and with lymphocyte and IgG4-positive plasmacyte infiltration. It predominantly causes mouth and eye dryness but can also affect other organs such as the lungs, liver, and kidneys, and be accompanied by complications such as autoimmune pancreatitis (AIP), retroperitoneal fibrosis, and tubulointerstitial nephritis.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (1)

chronic dacryoadenitis and sialadenitis

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.”

1/6 stages with a signal

Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    801 matched papers (242 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialNot found

    No matched interventional trial under our ClinicalTrials.gov rules

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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

801

801 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

801 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

242 in the last 10 years · high confidence · 81.5th percentile (publications denominator)

Phrase hits: 801 · MeSH hits: 9

Open Europe PMC search

Who's working on it?

1,040

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

Who's working on it?

No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.

  1. 01
    Takahashi H15 papers · 2025

    Department of Clinical Immunology and Rheumatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

    Papers in Europe PMC
  2. 02
    Yamamoto M15 papers · 2025

    First Department of Internal Medicine, School of Medicine, Sapporo Medical University, Hokkaido 0608543, Japan.

    Papers in Europe PMC
  3. 03
    Kawano M9 papers · 2025

    Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa-city, Ishikawa, 920-8640, Japan.

    Papers in Europe PMC
  4. 04
    Suzuki C8 papers · 2019

    Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, Sapporo, Division of Advanced Medicine Promotion, Advanced Clinical Research Center, Institute of Medical Science, University of Tokyo, Tokyo, Department of Otolaryngology, Sapporo Medical University School of Medicine, Sapporo and Center for Antibody and Vaccine Therapy, Institute of Medical Science, University of Tokyo, Tokyo, Japan.

    Papers in Europe PMC
  5. 05
    Himi T7 papers · 2025

    Department of Otolaryngology, Sapporo Medical University School of Medicine, Chuo-ku, Sapporo 060-8543, Japan.

    Papers in Europe PMC
  6. 06
    Kamekura R7 papers · 2025

    Department of Human Immunology, Research Institute for Frontier Medicine, Sapporo Medical University School of Medicine, Sapporo, Japan.

    Papers in Europe PMC
  7. 07
    Li J7 papers · 2026

    Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

    Papers in Europe PMC
  8. 08
    Shimizu Y6 papers · 2025

    Division of Nephrology, Department of Internal Medicine, Juntendo Shizuoka Hospital, Izunokuni, Japan.

    Papers in Europe PMC
  9. 09
    Takano K6 papers · 2026

    Department of Otolaryngology, Sapporo Medical University School of Medicine, Chuo-ku, Sapporo 060-8543, Japan.

    Papers in Europe PMC
  10. 10
    Zhang X6 papers · 2025

    College of Life Science and Bio‑engineering, Beijing University of Technology, Beijing 100124, P.R. China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

0

interventional trials for this specific condition

No interventional trial testing a treatment matched this specific condition name on ClinicalTrials.gov (observational studies and pan-disease registries are listed separately when present).

Data as of 27 July 2026

No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.

high confidence · 36.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.

See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

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.

"IgG4-related dacryoadenitis and sialadenitis" OR "Chronic dacryoadenitis and sialadenitis" OR "Mikulicz disease"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Mikulicz' Disease

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

"IgG4-related dacryoadenitis and sialadenitis" OR "Chronic dacryoadenitis and sialadenitis" OR "Mikulicz disease" OR "Mikulicz' Disease"

Study-type breakdown: 0 interventional · 0 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 2026-07-27T01:54:28.326Z