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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
Age of Onset
Adult
ICD-10
J84.1
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
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.†
5 Facts you should know
FACT
IPF is characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.
FACT
Risk factors include cigarette smoking, acid reflux disease (GERD), certain viral infections.
FACT
Those in their 60s and 70s are most commonly affected.
FACT
Males are affected more often than females.
FACT
The average life expectancy following diagnosis is about four years.
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Common Signs & Symptoms.
Bronchiectasis
Permanent enlargement of the airways of the lungs
Clubbing of hands and fingers
Cough
Crackles
Exertional dyspnea
Gastroesophageal reflux
Ground-glass opacification on pulmonary HRCT
Pulmonary fibrosis
Reticular pattern on pulmonary HRCT
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
Title | Description | Phases | Status | Conditions | Interventions | Sponsor/Collaborators | More 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 Fibrosis | Phase 3 | Recruiting | Idiopathic Pulmonary Fibrosis | Drug: Pamrevlumab|Drug: Placebo | FibroGen | More Info |
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 3 | Not yet recruiting | Idiopathic Pulmonary Fibrosis | Drug: PRM-151 | Hoffmann-La Roche | More Info |
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 3 | Recruiting | Idiopathic Pulmonary Fibrosis | Drug: PRM-151|Drug: Placebo | Hoffmann-La Roche | More Info |
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 3 | Recruiting | Idiopathic Pulmonary Fibrosis|Interstitial Lung Disease | Drug: Placebo|Drug: Inhaled Treprostinil|Device: Treprostinil Ultrasonic Nebulizer | United Therapeutics | More Info |
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 3 | Recruiting | Idiopathic Pulmonary Fibrosis | Drug: N-acetyl cysteine|Drug: Placebo | Weill 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 Foundation | More Info |
Top Treatments in Research
Agent | Class/Mechanism of Action | Development Status | Company | Company Contact | Clinical Studies | More Information |
---|---|---|---|---|---|---|
Pamrevlumab | Pamrevlumab 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 3 | Fibrogen | Gustavo Lorente 650-273-2264 [email protected] | More Info | More 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 3 | Hoffmann-La Roche | n/a | More Info | n/a |
Tyvaso® Treprostinil inhalation solution | TYVASO (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 3 | United Therapeutics Corporation | United Therapeutics Global Medical Information 919-485-8350 [email protected] | More Info | More Information |
N-acetyl cysteine | N-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 3 | NIH | US Federal Agency | n/a | More Info | More Information |
† Genetic and Rare Diseases Information Center (GARD) - PO Box 8126, Gaithersburg, MD 20898-8126 https://rarediseases.info.nih.gov