Rare Pulmonology News

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Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue

Prevalence

1-5 / 10 000

33,100 - 165,500

US Estimated

51,350 - 256,750

Europe Estimated

Age of Onset

Adult

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ICD-10

J84.1

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

Idiopathic pulmonary fibrosis is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue. It is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. Common symptoms include shortness of breath and a dry, hacking cough.

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Case courtesy of Dr Bruno Di Muzio, Radiopaedia.org. From the case rID: 28155

5 Facts you should know

FACT

1

IPF is characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.

FACT

2

Risk factors include cigarette smoking, acid reflux disease (GERD), certain viral infections.

FACT

3

Those in their 60s and 70s are most commonly affected.

FACT

4

Males are affected more often than females.

FACT

5

The average life expectancy following diagnosis is about four years.

Idiopathic Pulmonary Fibrosis (IPF)

 

Idiopathic pulmonary fibrosis (IPF) is also known as:

  • Idiopathic Diffuse Fibrosing Alveolitis (IDF)
  • Fibrosing alveolitis, cryptogenic
  • Familial idiopathic pulmonary fibrosis
  • Fibrocystic pulmonary dysplasia
  • Fibrosing alveolitis

What’s your rare IQ?

Which seemingly unrelated symptom is seen in idiopathic pulmonary fibrosis patients?

Common Signs & Symptoms.

Bronchiectasis

Permanent enlargement of the airways of the lungs

Clubbing of hands and fingers

Cough

Crackles

Exertional dyspnea

Gastroesophageal reflux

Acid reflux, Acid reflux disease, Heartburn

Ground-glass opacification on pulmonary HRCT

Pulmonary fibrosis

Reticular pattern on pulmonary HRCT

Frequent ear infections

Current treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Pirfenidone

Manufactured by InterMune, Inc.
FDA-approved indication: Treatment of idiopathic pulmonary fibrosis.
National Library of Medicine Drug Information Portal

Nintedanib

Manufactured by Boehringer Ingelheim Pharmaceuticals, Inc.
FDA-approved indication: October 2014, nintedanib (Ofev) was approved for the treatment of idiopathic pulmonary fibrosis.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

Top Clinical Trials

TitleDescriptionPhasesStatusConditionsInterventionsSponsor/CollaboratorsMore Information
Evaluation of Efficacy and Safety of Pamrevlumab in Patients With Idiopathic Pulmonary Fibrosis.This is a Phase 3 trial to evaluate the efficacy and safety of 30 mg/kg intravenous (IV) infusions of pamrevlumab administered every 3 weeks as compared to placebo in subjects with Idiopathic Pulmonary FibrosisPhase 3RecruitingIdiopathic Pulmonary FibrosisDrug: Pamrevlumab|Drug: PlaceboFibroGenMore Information
A Study to Evaluate Long Term Safety and Efficacy of Recombinant Human Pentraxin-2 (rhPTX-2; PRM-151) in Participants With Idiopathic Pulmonary Fibrosis This study will evaulate the long-term safety, efficacy and pharmacokinetics (PK) of recombinant human pentraxin-2 (rhPTX-2; PRM-151) administered by intravenous (IV) infusion to participants with idiopathic pulmonary fibrosis (IPF).Phase 3Not yet recruitingIdiopathic Pulmonary FibrosisDrug: PRM-151Hoffmann-La RocheMore Information
A Study to Evaluate the Efficacy and Safety of Recombinant Human Pentraxin-2 (rhPTX-2; PRM-151) in Participants With Idiopathic Pulmonary Fibrosis This phase III study will evaluate the efficacy, safety and pharmacokinetics (PK) of recombinant human pentraxin-2 (rhPTX-2; PRM-151) compared with placebo in participants with idiopathic pulmonary fibrosis (IPF).
Phase 3RecruitingIdiopathic Pulmonary FibrosisDrug: PRM-151|Drug: PlaceboHoffmann-La RocheMore Information
Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis Study RIN-PF-301 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with idiopathic pulmonary fibrosis (IPF) over a 52-week period.
Phase 3RecruitingIdiopathic Pulmonary Fibrosis|Interstitial Lung DiseaseDrug: Placebo|Drug: Inhaled Treprostinil|Device: Treprostinil Ultrasonic NebulizerUnited TherapeuticsMore Information
Prospective Treatment Efficacy in IPF Using Genotype for Nac Selection (PRECISIONS) Trial The purpose of this study is to compare the effect of n-acetylcysteine (NAC) plus standard care with matched placebo plus standard of care in patients diagnosed with idiopathic pulmonary fibrosis (IPF) who have the TOLLIP rs3750920 TT genotype. The study will compare the time to a composite endpoint of relative decline in lung function [10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplantation, or all-cause mortality]

The secondary objectives will be to examine the effect of NAC on the components of the primary composite endpoint, the rates of clinical events, change in physiology, change in health status, and change in respiratory symptoms.
Phase 3RecruitingIdiopathic Pulmonary FibrosisDrug: N-acetyl cysteine|Drug: PlaceboWeill Medical College of Cornell University|University of Virginia|University of Michigan|Pulmonary Fibrosis Foundation|University of Washington|National Heart, Lung, and Blood Institute (NHLBI)|Three Lakes FoundationMore Information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyCompany ContactClinical StudiesMore Information
PamrevlumabPamrevlumab is a fully-human monoclonal antibody that inhibits the activity of connective tissue growth factor (CTGF). CTGF is a common factor in chronic fibrotic and proliferative disorders, characterized by persistent and excessive fibrous tissue which can lead to organ dysfunction and failure, and in cancer, characterized by promotion of tumor growth.Phase 3FibrogenGustavo Lorente 650-273-2264 Zephyrus@Fibrogen.com
More InfoMore Information
rhPTX-2 (PRM-151)A recombinant human serum amyloid P/pentraxin 2 protein. Pentraxin-2 is an endogenous human protein that plays an important role in regulating the response to fibrosis. It directs the immune system to naturally turn off and reverse the process of fibrosis, which occurs as a result of excess collagen secretion and cellular growth and differentiation. Unlike other formulations that work by stopping a single target on the downstream side of fibrosis, this protein works by reversing and possibly healing the fibrotic tissue.Phase 3Hoffmann-La Roche
n/aMore Infon/a
Tyvaso® Treprostinil inhalation solutionTYVASO (treprostinil) is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to improve exercise ability. Studies establishing effectiveness included predominately patients with NYHA Functional Class III symptoms and etiologies of idiopathic or heritable PAH (56%) or PAH associated with connective tissue diseases (33%). The effects diminish over the minimum recommended dosing interval of 4 hours; treatment timing can be adjusted for planned activities.
While there are long-term data on use of treprostinil by other routes of administration, nearly all controlled clinical experience with inhaled treprostinil has been on a background of bosentan (an endothelin receptor antagonist) or sildenafil (a phosphodiesterase type 5 inhibitor). The controlled clinical experience was limited to 12 weeks in duration.
Phase 3United Therapeutics CorporationUnited Therapeutics Global Medical Information 919-485-8350 clinicaltrials@unither.comMore InfoMore Information
N-acetyl cysteineN-acetyl cysteine (NAC) is a medication used to loosen thick mucus. NAC was initially licensed for use in 1968. It is on the World Health Organization's List of Essential Medicines, the most effective and safe medicines needed in a health system, and it is available as a generic medication and is not very expensive. Inhaled NAC has been used as a mucus dissolving therapy in respiratory conditions with excessive and/or thick mucus production.Phase 3NIH | US Federal Agencyn/aMore InfoMore Information

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