IW001 is an investigational drug under clinical development by ImmuneWorks. It is an oral solution to target the autoimmune component of idiopathic pulmonary hypertension (IPF), believed to be the anti-Col (V) antibody. It is a one-of-a-kind approach to treat the condition. No FDA-approved treatment currently exists.

How IW001 Works

The Type V collagen (Col (V)) is a naturally occurring protein in the body, which is normally hidden. It is hypothesized that the immune system might recognize this hidden protein as a foreign ‘antigen’ and produce antibodies in response to it, in spite of the fact that Col (V) is a native protein. The miscommunication leads to a cascade of immune responses directed against the lungs, leading to inflammation, subsequent scarring, and increased arterial blood pressure.

When IW001 is administered orally, it is introduced into the mucosal immune system at the Peyer’s Patches in the distal small intestine, where antigen-presenting cells introduce the antigen to regulatory T-cells (Tregs). The antigen-specific Tregs enter the blood stream and migrate to areas where the specific antigen has generated an autoimmune response. IW001 might help in the production of a selective immune suppression response against Col (V).

IW001 Testing for IPF

Preclinical studies have shown that administration of Type V collagen in animal models with lung disease prevented progression of lung fibrosis by modifying the underlying autoimmunity, which is similar to that found in IPF patients. As a result, IW001 was put to test in human models for safety and tolerance. IIW001 was granted orphan drug status by the FDA in 2009.

As a part of the clinical development program, ImmuneWorks conducted an open label, multicenter, Phase I clinical trial in IPF patients who tested positive for anti-col(V) antibodies. This study was aimed at assessing the safety, tolerability, and biological effects of three doses (0.1mg, 0.5mg, 1.0mg) of IW001, when administered once daily orally for 24 weeks to 30 patients from 11 U.S. states.

Results showed that the formulation improved lung function (measured by forced vital capacity or FVC) and stabilized it by the end of 24 weeks. The drug was shown to be safe and well tolerated among patients with no adverse effects, pulmonary exacerbations or IPH-related hospitalizations. With the positive results, the company plans to proceed toward Phase 2 trials.

Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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