ORPHA:280302
Autoimmune pancreatitis type 1
Also known as: AIP type 1 · IgG4-related pancreatitis · Lymphoplasmacytic sclerosing pancreatitis
Publications
862
90.6th percentile
Trials
0
Interventional, condition-specific
Researchers
1,070
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease.
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:0017227
- UMLS:C4302243
Additional Mondo synonyms (2)
autoimmune pancreatitis type 1 · lymphoplasmacytic sclerosing pancreatitis
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
No specific-condition interventional trial, but broader-category trials exist — discuss eligibility with a clinician.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
862 matched papers (534 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPartial
None under the specific name; 8 for broader category autoimmune pancreatitis
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?
862
862 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.
862 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).
534 in the last 10 years · high confidence · 90.6th percentile (publications denominator)
Phrase hits: 862 · MeSH hits: 0
Who's working on it?
1,070
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.
- 01Löhr JM12 papers · 2025
Department of Medicine, Huddinge, Karolinska Institutet, 14186 Stockholm, Sweden.
Papers in Europe PMC - 02Vujasinovic M12 papers · 2025
Department of Upper Abdominal Diseases, Karolinska University Hospital, 14186 Stockholm, Sweden.
Papers in Europe PMC - 03Okazaki K7 papers · 2021
The Third Department of Internal Medicine Division of Gastroenterology and Hepatology, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan. okazaki@hirakata.kmu.ac.jp
Papers in Europe PMC - 04Nikolic S6 papers · 2025
Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden.
Papers in Europe PMC - 05Uchida K6 papers · 2021
Department of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
Papers in Europe PMC - 06Jin Z5 papers · 2026
Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China.
Papers in Europe PMC - 07Li Y5 papers · 2026
Department of PET/CT, The First Affiliated Hospital of Xi'an Jiaotong University, 277 Yanta West Road, Xi'an, 710061, Shaanxi, China.
Papers in Europe PMC - 08Huang H4 papers · 2026
Department of Gastroenterology, National Clinical Research Center for Digestive Diseases, Changhai Hospital; and National Key Laboratory of Immunity and Inflammation, Naval Medical University, Shanghai, China.
Papers in Europe PMC - 09Panic N4 papers · 2023
Faculty of Medicine, University of Belgrade, Digestive Endoscopy Unit, University Clinic "Dr Dragisa Misovic", Belgrade, Serbia.
Papers in Europe PMC - 10Valente R4 papers · 2020
Department for Digestive Diseases, Karolinska University Hospital, SE-141 86 Stockholm, Sweden. roberto.valente@sll.se.
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. 1 observational study did — shown below because natural-history and cohort work can be an important step toward a trial. 8 trials are registered for autoimmune pancreatitis, the broader category — shown separately because they may or may not enrol this specific subtype.
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.
8 interventional trials matched autoimmune pancreatitis, the broader category — listed below. Those studies are not counted in the condition-specific total.
Broader category: autoimmune pancreatitis
8
Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.
Worth raising with a clinician. How we count trials.
Recruiting under the broader category
- NCT06753318·NOT YET RECRUITING·Validation of Joint-AI in Diagnosing Pancreatic Solid Lesions
Conditions: Pancreatic Cancer · Pancreatitis · Pancreatic Neuroendocine Neoplasms (pNETs) · Autoimmune Pancreatitis·Matched via name phrase
Observational and natural-history studies
1 observational study matches 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.
None of the matched observational studies is currently listed as recruiting.
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 2026-07-26This 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.
"Autoimmune pancreatitis type 1" OR "AIP type 1" OR "IgG4-related pancreatitis" OR "Lymphoplasmacytic sclerosing pancreatitis"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Autoimmune pancreatitis type 1" OR "AIP type 1" OR "IgG4-related pancreatitis" OR "Lymphoplasmacytic sclerosing pancreatitis"
Study-type breakdown: 0 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"autoimmune pancreatitis"
Query health: ok — strategies attempted: phrase; with hits: phrase
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 2026-07-27T11:52:40.371Z