ORPHA:63999
IgG4-related mediastinitis
Also known as: Fibrosing mediastinitis · Mediastinal fibrosis · Sclerosing mediastinitis
Publications
2,237
Trials
1
Interventional, condition-specific
Researchers
974
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare systemic autoimmune disease characterized by an aggressive fibroinflammatory process with infiltration of IgG4-positive plasma cells in the mediastinum, potentially resulting in compression and functional impairment of vital mediastinal structures, and associated with elevated serum IgG4. Clinical symptoms are unspecific and include pain or symptoms due to mass effect. The condition may occur together with IgG4-related disease in other parts of the body.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0018978
- MeSH:C536136
- UMLS:C0264573
Additional Mondo synonyms (4)
Fibrosing Mediastinitis · fibrosing mediastinitis · mediastinal fibrosis · sclerosing mediastinitis
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
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
2,237 matched papers (1,121 in last 10 years) Source
- Phenotype characterisedNot found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- 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 trialPresent
1 matched on ClinicalTrials.gov (1 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)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
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?
2,237
2,237 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.
2,237 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).
1,121 in the last 10 years · low confidence
Phrase hits: 2,237 · MeSH hits: 24
Who's working on it?
974
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.
- 01Cao Y20 papers · 2026
Heart, Lung and Vessels Center, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, 32 West Second Section First Ring Road, Chengdu, Sichuan 610072, China.
Papers in Europe PMC - 02Su H16 papers · 2026
Department of Cardiology Pulmonary Vascular Disease Center, Gansu Provincial Hospital Lanzhou China.
Papers in Europe PMC - 03Jiang K10 papers · 2026
Department of Cardiology Pulmonary Vascular Disease Center, Gansu Provincial Hospital Lanzhou China.
Papers in Europe PMC - 04Li B9 papers · 2026
Department of Cardiology Pulmonary Vascular Disease Center, Gansu Provincial Hospital Lanzhou China.
Papers in Europe PMC - 05Wang A9 papers · 2026
Department of Cardiology Pulmonary Vascular Disease Center, Gansu Provincial Hospital Lanzhou China.
Papers in Europe PMC - 06Li Y8 papers · 2026
Department of Radiology, The Seventh Affiliated Hospital of Sun Yat-sen University, Shenzhen, China.
Papers in Europe PMC - 07Zhang M8 papers · 2026
Department of Respiratory and Critical Care Medicine, First Hospital of Shanxi Medical University, Shanxi Medical University, Taiyuan 030001, China.
Papers in Europe PMC - 08Zhang Y8 papers · 2026
Tianjin Medical University Eye Hospital, Translational Medicine Laboratory, Tianjin Key Laboratory of Retinal Functions and Diseases, Tianjin 300384, China.
Papers in Europe PMC - 09Li J7 papers · 2026
Department of Pulmonary and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Papers in Europe PMC - 10Wang Y6 papers · 2026
School of Clinical Medicine, Ningxia Medical University, No. 1160, Shengli Street, Yinchuan 750004, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
1
interventional trials for this specific condition
1 interventional trial matched this specific condition name; 1 currently recruiting in our sample.
Data as of 11 September 2026
1 interventional trial — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 80.1th percentile).
low confidence · 80.1th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
1 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT06980584·RECRUITING·Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension
Not reviewed·Conditions: Fibrosing Mediastinitis · Pulmonary Hypertension·Matched via name phrase
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 5 · after dedupe 5 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 5 · 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 (5)
- isrctn·ISRCTN16358704·No longer recruiting·A study of the safety, pharmacokinetics, and therapeutic activity of cibisatamab in combination with atezolizumab in participants with locally advanced and/or metastatic carcinoembryonic antigen (CEA)-positive solid tumors
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN15878562·No longer recruiting·Determining the extent of liver scarring in patients with psoriasis using a non-invasive scan and assessing the relationship between liver scarring and other potential risk factors for liver damage including methotrexate
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN11499979·No longer recruiting·Does the planning tool “Multi-Criteria Optimisation (MCO)” reduce the radiation dose received to the chest wall when planning a lung cancer radiotherapy treatment, in comparison to traditional planning methods?
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN11965217·No longer recruiting·A trial to assess whether the addition of atezolizumab to current standard treatment for patients with relapsed or refractory Diffuse Large B-Cell Lymphoma, who are not able to have high dose therapy, improves survival outcomes
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN99811594·No longer recruiting·The role of FluoroDeoxyGlucose-Positron Emission Tomography imaging in clinical stages Ia/IIa Hodgkin's disease
skipped — LLM skipped (--skip-llm)
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 mediastinitis — 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 mediastinitis" OR "Fibrosing mediastinitis" OR "Mediastinal fibrosis" OR "Sclerosing mediastinitis"
MeSH descriptor terms unioned into the query: Mediastinal Fibrosis
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"IgG4-related mediastinitis" OR "Fibrosing mediastinitis" OR "Mediastinal fibrosis" OR "Sclerosing mediastinitis"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 1 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
- Publication count (2237) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T01:08:14.850Z