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ORPHA:79126

Acute interstitial pneumonia

medium confidenceDisorder

Also known as: Acute interstitial pneumonitis · Hamman-Rich syndrome

Publications

2,518

88.7th percentile

Trials

1

Interventional, condition-specific

Researchers

1,230

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare, rapidly , and histologically distinct, form of interstitial pneumonia.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (3)

AIP · Hamman-rich syndrome · acute interstitial pneumonitis

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.”

3/6 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

    2,518 matched papers (1,281 in last 10 years) Source

  3. Phenotype characterisedPresent

    33 HPO annotations (e.g. Bronchiectasis; Pulmonary infiltrates; Peribronchovascular interstitial thickening) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    1 matched on ClinicalTrials.gov

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:0019203

  • Bronchiectasis
  • Pulmonary infiltrates
  • Peribronchovascular interstitial thickening
  • Parenchymal consolidation
  • Cyanosis

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?

2,518

2,518 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.

2,518 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).

1,281 in the last 10 years · medium confidence · 88.7th percentile (publications denominator)

Phrase hits: 2,518 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,230

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
    White BJ9 papers · 2026

    Department of Clinical Sciences, College of Veterinary Medicine, Kansas State University, Manhattan, KS 66502, USA.

    Papers in Europe PMC
  2. 02
    Larson RL7 papers · 2026

    Department of Clinical Sciences, College of Veterinary Medicine, Kansas State University, Manhattan, KS 66502, USA.

    Papers in Europe PMC
  3. 03
    Bortoluzzi EM5 papers · 2025

    Beef Cattle Institute, Kansas State University, Manhattan, KS 66506, USA.

    Papers in Europe PMC
  4. 04
    Jensen M5 papers · 2025

    Beef Cattle Institute, Kansas State University, Manhattan, KS 66506, USA.

    Papers in Europe PMC
  5. 05
    Lancaster PA5 papers · 2025

    Beef Cattle Institute, Kansas State University, Manhattan, KS 66506, USA.

    Papers in Europe PMC
  6. 06
    Schmidt PH5 papers · 2026

    Beef Cattle Institute, Kansas State University, Manhattan, KS 66506, USA.

    Papers in Europe PMC
  7. 07
    Yang Y4 papers · 2026

    Department of Respiratory Medicine, Shanxi Province Cancer Hospital/Shanxi Hospital Affiliated to Cancer Hospital, Chinese Academy of Medical Sciences/Cancer Hospital Affiliated with Shanxi Medical University, Taiyuan, China.

    Papers in Europe PMC
  8. 08
    Zhang J4 papers · 2026

    Medicine Imaging Department, Shanxi Province Cancer Hospital/Shanxi Hospital Affiliated to Cancer Hospital, Chinese Academy of Medical Sciences/Cancer Hospital Affiliated with Shanxi Medical University, Taiyuan, China.

    Papers in Europe PMC
  9. 09
    Brown RE3 papers · 2023

    Beef Cattle Institute, Kansas State University, Manhattan, KS 66506, USA.

    Papers in Europe PMC
  10. 10
    Campillo JA3 papers · 2025

    Immunology Service, Clinic University Hospital Virgen de la Arrixaca, Biomedical Research Institute of Murcia Pascual Parrilla (IMIB), 30120 Murcia, Spain.

    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; none in our sample are currently recruiting. 1,762 trials are registered for pneumonia, the broader category — shown separately because they may or may not enrol this specific subtype.

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).

medium 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.

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: pneumonia

1,762

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

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 38 · after dedupe 38 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 38 · 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 (38)

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 Acute interstitial pneumonia — 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.

"Acute interstitial pneumonia" OR "Acute interstitial pneumonitis" OR "Hamman-Rich syndrome"

Run this search on Europe PMC

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

"Acute interstitial pneumonia" OR "Acute interstitial pneumonitis" OR "Hamman-Rich syndrome"

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.

Parent-category trials query:

"pneumonia"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: AIP

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T01:59:49.003Z