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pulmonary fibrosis - Clinical Profile, Treatments & Trials | Kisho | Kisho

Rare Disease

pulmonary fibrosis

MONDO:0002771

Also known as:fibrosis of lungpulmonary interstitial fibrosis

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Overview

Chronic progressive interstitial lung disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure, or right heart failure. Causes include chronic inflammatory processes, exposure to environmental irritants, radiation therapy, autoimmune disorders, certain drugs, or it may be idiopathic (no identifiable cause).

Treatments1 FDA approved

Clinical Trials209 registered · 71 recruiting

Patient Orgs1 organizations · Coalition for Pulmonary Fibrosis

Diagnosis

Biomarker and diagnostic research for pulmonary fibrosis 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.

1 FDA-approved treatment is available for pulmonary fibrosis, including TREPROSTINIL (TYVASO DPI, approved 2022). An additional 2 compounds hold orphan drug designation.

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved	Market Status

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.

209 clinical trials registered, 71 recruiting. Interventions under study include other interventions, drug therapy, medical devices, and biologic therapy. Pipeline includes 1 PHASE4, 22 PHASE3, 47 PHASE2. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT06853145](https://clinicaltrials.gov/study/NCT06853145)

Data assembled from 4 of 12 sources · Last updated Nov 3, 2026, 2:30 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Patient Support

Financial & Access Support (External Programs)

View all programs →

Pulmonary Fibrosis Fund

Premium/CopayClosedOperated by HealthWell Foundation

Visit official site

Program availability and eligibility requirements are set by each foundation. Contact them directly to learn more about your options.

Patient Advocacy Groups

View all PAGs →

Coalition for Pulmonary FibrosisPrimary Focus

USWebsite ↗

Patient Registry

Enroll Now

Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.

Are you a patient organization not listed here? Claim this page to be listed and embed this content on your site — free.

Medical References

Questions for Your Care Team

→What are the benefits and potential side effects of TYVASO DPI for managing pulmonary fibrosis?
→Are any of the 209 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→How can I connect with Coalition for Pulmonary Fibrosis and other patient organizations for support?

← Back to All Diseases

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
N-(1¿,2-dihydroxy-[1,2¿-binapthalen]-4¿-yl)-4-methoxybenzenesulfonamide	N-(1¿,2-dihydroxy-[1,2¿-binapthalen]-4¿-yl)-4-methoxybenzenesulfonamide	Tvardi Therapeutics, Inc.	2022	—	Designated
Interferon beta-1a (recombinant human)	Interferon beta-1a (recombinant human)	Biogen Idec, Inc.	1999	—	Withdrawn

Research Type	Count	% of Total
Laboratory research	257	51%
Research summaries	112	22%
New treatment approaches	45	9%
Disease patterns and progression	39	8%
Testing and diagnosis research	24	5%
Clinical study results	10	2%
Patient case studies	8	2%
Other research	5	1%

Kisho

pulmonary fibrosis

Overview

Diagnosis

Treatment

Approved Therapies

Research Landscape

Patient Support

Financial & Access Support (External Programs)

Patient Advocacy Groups

Medical References

Questions for Your Care Team

Questions for Your Care Team

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Obesity and the risk of idiopathic pulmonary fibrosis: a nationwide population-based study.

Bristol Myers trial revisions shake confidence in new class of fibrosis drugs

Riddle syndrome with RNF168 mutation in first Asian family cluster exhibiting pulmonary fibrosis and achalasia.

Exploring the Autoimmune Spectrum of Combined Pulmonary Fibrosis and Emphysema: Inflammatory Myopathy Cases and a Systematic Review.

New medicine for two types of pulmonary fibrosis

pulmonary fibrosis

Overview

Diagnosis

Treatment

Approved Therapies

Research Landscape

Patient Support

Financial & Access Support (External Programs)

Patient Advocacy Groups

Medical References

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Obesity and the risk of idiopathic pulmonary fibrosis: a nationwide population-based study.

Bristol Myers trial revisions shake confidence in new class of fibrosis drugs

Riddle syndrome with RNF168 mutation in first Asian family cluster exhibiting pulmonary fibrosis and achalasia.

Exploring the Autoimmune Spectrum of Combined Pulmonary Fibrosis and Emphysema: Inflammatory Myopathy Cases and a Systematic Review.

New medicine for two types of pulmonary fibrosis