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ORPHA:79128

Lymphoid interstitial pneumonia

low confidenceDisorder

Also known as: Lymphocytic interstitial pneumonia

Publications

2,523

Trials

1

Interventional, condition-specific

Researchers

1,187

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare interstitial pneumonia characterized by a diffuse, dense, polyclonal lymphoid cell infiltration of the pulmonary interstitium and air spaces, with high prevalence in patients with immune dysregulation. Presenting symptoms are non-specific and include dyspnea and cough. The clinical course is highly variable, ranging from spontaneous resolution to , fatal respiratory failure.

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 (5)

diffuse hyperplasia of bronchus-associated lymphoid tissue · lymphocytic interst. pneumonitis · lymphocytic interstitial pneumonia · lymphocytic interstitial pneumonitis · lymphoid interstitial pneumonia

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

    2,523 matched papers (1,348 in last 10 years) Source

  3. Phenotype characterisedPresent

    38 HPO annotations (e.g. Restrictive ventilatory defect; Multiple pulmonary cysts; Fatigue) 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)

38

Associated phenotypes · MONDO:0009537

  • Restrictive ventilatory defect
  • Multiple pulmonary cysts
  • Fatigue
  • Wheezing
  • Crackles

Showing 5 of 38 — 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?

2,523

2,523 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.

2,523 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).

1,348 in the last 10 years · low confidence

Phrase hits: 2,523 · MeSH hits: 28

Open Europe PMC search

Who's working on it?

1,187

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
    Zhang Y6 papers · 2026

    Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Nanjing University Medical School, Nanjing, 210008, Jiangsu, China.

    Papers in Europe PMC
  2. 02
    Yang Y5 papers · 2026

    Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

    Papers in Europe PMC
  3. 03
    Li X4 papers · 2026

    Department of Rheumatology and Immunology, Shaanxi Provincial People's Hospital, Xi'an, Shaanxi, China.

    Papers in Europe PMC
  4. 04
    Sambataro G4 papers · 2026

    Department of Medicine and Surgery, University of Enna "Kore", 94100 Enna, Italy.

    Papers in Europe PMC
  5. 05
    Zhang J4 papers · 2026

    Department of CT, The Second Affiliated Hospital of Harbin Medical University, Harbin, 15000, China. jinlingzi@163.com.

    Papers in Europe PMC
  6. 06
    Zhang X4 papers · 2026

    Department of Nuclear Medicine, Hebei General Hospital, Shijiazhuang, China.

    Papers in Europe PMC
  7. 07
    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
  8. 08
    Gimeno L3 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
  9. 09
    González-López R3 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
  10. 10
    Li Y3 papers · 2025

    Laboratory of Pharmacology, Institute of Materia Medica, Chinese Academy of Medical Sciences & Peking Union Medical College, 2 Nanwei Rd , Beijing, 100050, 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; 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).

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.

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

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.

Open the complete matched search on ClinicalTrials.gov

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 Lymphoid 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.

"Lymphoid interstitial pneumonia" OR "Lymphocytic interstitial pneumonia" OR "diffuse hyperplasia of bronchus-associated lymphoid tissue" OR "diffuse hyperplasia of the bronchus-associated lymphoid tissue" OR "lymphocytic interst. pneumonitis" OR "lymphocytic interstitial pneumonitis"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Lymphoid Interstitial Pneumonia

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

"Lymphoid interstitial pneumonia" OR "Lymphocytic interstitial pneumonia" OR "diffuse hyperplasia of bronchus-associated lymphoid tissue" OR "diffuse hyperplasia of the bronchus-associated lymphoid tissue" OR "lymphocytic interst. pneumonitis" OR "lymphocytic interstitial pneumonitis"

Interventional trials matched via: both (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 · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"pneumonia"

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 (2523) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T02:00:23.460Z