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ORPHA:420259

Secondary pulmonary alveolar proteinosis

medium confidenceDisorder

Also known as: Secondary PAP

Publications

490

76.1th percentile

Trials

0

Interventional, condition-specific

Researchers

1,205

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare, acquired, interstitial lung disease, characterized by alveolar surfactant accumulation, cough, dyspnea and respiratory insufficiency. The disease may be secondary to hematological disorder, toxic inhalation, and infection or may occur within the setting of immunosuppression after transplantation.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (2)

SPAP · secondary PAP

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    490 matched papers (304 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  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 trialPartial

    None under the specific name; 22 for broader category pulmonary alveolar proteinosis

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?

490

490 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

490 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).

304 in the last 10 years · medium confidence · 76.1th percentile (publications denominator)

Phrase hits: 490 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,205

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.

  1. 01
    Nakata K12 papers · 2026

    Bioscience Medical Research Center, Niigata University, 1-754 Asahimachi Dori, Chuoku, Niigata 951-8520, Japan. Electronic address: radical@med.niigata-u.ac.jp.

    Papers in Europe PMC
  2. 02
    Ishii H8 papers · 2020

    Department of Respiratory Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, 181-8611, Japan. Electronic address: h141@ks.kyorin-u.ac.jp.

    Papers in Europe PMC
  3. 03
    McCarthy C6 papers · 2026

    Department of Respiratory Medicine, St. Vincent's University Hospital, Dublin, Ireland.

    Papers in Europe PMC
  4. 04
    Bonella F5 papers · 2026

    Center for Interstitial and Rare Lung Diseases, Pneumology Department, Ruhrlandklinik University Hospital, University of Duisburg-Essen, Essen, Germany.

    Papers in Europe PMC
  5. 05
    Chen J5 papers · 2025

    Department of Thoracic Surgery, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China. Electronic address: chenjy@wuxiph.com.

    Papers in Europe PMC
  6. 06
    Inoue Y5 papers · 2024

    Department of Diffuse Lung Diseases and Respiratory Failure, Clinical Research Center, NHO Kinki-Chuo Chest Medical Center, Osaka, Japan.

    Papers in Europe PMC
  7. 07
    Takada T5 papers · 2020

    Division of Respiratory Medicine, Niigata University Graduate School of Medical and Dental Sciences, 1-754 Asahimachi-dori, Chuo-ku, Niigata 951-8520, Japan. Electronic address: ttakada@med.niigata-u.ac.jp.

    Papers in Europe PMC
  8. 08
    Zhang J5 papers · 2024

    Guanganmen Hospital, China Academy of Chinese Medical Sciences, Beijing, China.

    Papers in Europe PMC
  9. 09
    Chen Y4 papers · 2026

    Department of Thoracic Surgery, The Affiliated Wuxi People's Hospital of Nanjing Medical University, Wuxi, Jiangsu, China.

    Papers in Europe PMC
  10. 10
    Ichiwata T4 papers · 2014

    Department of Respiratory Medicine, Dokkyo University Hospital, Saitama, Japan.

    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). 22 trials are registered for pulmonary alveolar proteinosis, the broader category — shown separately because they may or may not enrol this specific subtype.

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.

medium 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.

22 interventional trials matched pulmonary alveolar proteinosis, the broader category — listed below. Those studies are not counted in the condition-specific total.

Broader category: pulmonary alveolar proteinosis

22

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 27 · after dedupe 27 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 27 · 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 (27)

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 Secondary pulmonary alveolar proteinosis — 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.

"Secondary pulmonary alveolar proteinosis" OR "Secondary PAP"

Run this search on Europe PMC

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

"Secondary pulmonary alveolar proteinosis" OR "Secondary PAP"

Study-type breakdown: 0 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"pulmonary alveolar proteinosis"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: SPAP

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 2026-07-27T15:46:32.154Z