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idiopathic interstitial pneumonia (IIP) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

idiopathic interstitial pneumonia

MONDO:0002429

Also known as:IIPdiffuse idiopathic pulmonary fibrosisidiopathic fibrosing alveolitis

Find Clinical TrialsTrials

Overview

A class of diffuse lung diseases that typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, Cryptogenic organizing pneumonitis).

OnsetINFANTILE

TreatmentsNo approved treatments · 12 in pipeline

Clinical Trials8 registered · 2 recruiting

Diagnosis

Biomarker and diagnostic research for idiopathic interstitial pneumonia has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for idiopathic interstitial pneumonia. An additional 12 compounds hold orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for idiopathic interstitial pneumonia, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for idiopathic interstitial pneumonia. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

8 clinical trials registered, 2 recruiting. Interventions under study include other interventions, drug therapy, and procedural interventions. Pipeline includes 1 PHASE2, 1 PHASE1, 1 EARLY_PHASE1. Research is primarily sponsored by academic and government institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05365802](https://clinicaltrials.gov/study/NCT05365802)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 3:31 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:98300

GARD

Genetic and Rare Diseases Info Center

GARD:13337

Questions for Your Care Team

→Are any of the 8 active clinical trials a good fit for my situation?
→With 12 compounds in the orphan drug pipeline, which are furthest along in development and which clinical trials should I be aware of?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Disease patterns and progression	37	35%
Research summaries	21	20%
Testing and diagnosis research	11	10%
Other research	10	10%
Laboratory research	10	10%
Clinical study results	9	9%
Patient case studies	7	7%

Kisho

idiopathic interstitial pneumonia

Overview

Diagnosis

Treatment

Orphan Drug Designations

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

[Interstitial pneumonia with primary pulmonary atypical carcinoid tumor and multiple brain metastases: a case report].

[Clinical characteristics of 20 lung adenocarcinoma patients misdiagnosed as interstitial pneumonia].

Hepatopulmonary Syndrome Associated With Metabolic Dysfunction-Associated Steatotic Liver Disease in a Patient With Interstitial Pneumonia and COPD.

idiopathic interstitial pneumonia

Overview

Diagnosis

Treatment

Orphan Drug Designations

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

[Interstitial pneumonia with primary pulmonary atypical carcinoid tumor and multiple brain metastases: a case report].

[Clinical characteristics of 20 lung adenocarcinoma patients misdiagnosed as interstitial pneumonia].

Hepatopulmonary Syndrome Associated With Metabolic Dysfunction-Associated Steatotic Liver Disease in a Patient With Interstitial Pneumonia and COPD.