ORPHA:79078
IgG4-related dacryoadenitis and sialadenitis
Also known as: Chronic dacryoadenitis and sialadenitis · Mikulicz disease
Publications
801
71.4th percentile
Trials
0
Interventional, condition-specific
Researchers
1,040
Distinct authors in sample
Gene link
—
Readiness
2/6
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.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0019191
- MeSH:D008882
- UMLS:C0026103
- NCIT:C34819
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.”
2/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
801 matched papers (242 in last 10 years) Source
- Phenotype characterisedPresent
33 HPO annotations (e.g. Periorbital fullness; Antinuclear antibody positivity; Cytoplasmic antineutrophil antibody positivity) Source
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- 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)
33
Associated phenotypes · MONDO:0019191
- Periorbital fullness
- Antinuclear antibody positivity
- Cytoplasmic antineutrophil antibody positivity
- Myositis
- Lymphadenopathy
Showing 5 of 33 — open Monarch for the full list.
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
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)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
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=3967) is 59.
801 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).
242 in the last 10 years · high confidence · 71.4th percentile (publications denominator)
Phrase hits: 801 · MeSH hits: 9
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.
- 01Takahashi H15 papers · 2025
Department of Clinical Immunology and Rheumatology, Sapporo Medical University School of Medicine, Sapporo, Japan.
Papers in Europe PMC - 02Yamamoto M15 papers · 2025
First Department of Internal Medicine, School of Medicine, Sapporo Medical University, Hokkaido 0608543, Japan.
Papers in Europe PMC - 03Kawano 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 - 04Suzuki 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 - 05Himi T7 papers · 2025
Department of Otolaryngology, Sapporo Medical University School of Medicine, Chuo-ku, Sapporo 060-8543, Japan.
Papers in Europe PMC - 06Kamekura R7 papers · 2025
Department of Human Immunology, Research Institute for Frontier Medicine, Sapporo Medical University School of Medicine, Sapporo, Japan.
Papers in Europe PMC - 07Li J7 papers · 2026
Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.
Papers in Europe PMC - 08Shimizu Y6 papers · 2025
Division of Nephrology, Department of Internal Medicine, Juntendo Shizuoka Hospital, Izunokuni, Japan.
Papers in Europe PMC - 09Takano K6 papers · 2026
Department of Otolaryngology, Sapporo Medical University School of Medicine, Chuo-ku, Sapporo 060-8543, Japan.
Papers in Europe PMC - 10Zhang 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 11 September 2026 · last trial check 11 September 2026
No matched interventional trials. This is true for 77.2% of diseases in the trials denominator (5501 of 7126). Here are the researchers publishing on it.
high confidence · 38.6th 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.
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 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · 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).
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 IgG4-related dacryoadenitis and sialadenitis — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This 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"
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