A Comparison of a Medication Adherence Platform (FORTISKAP™) vs. Usual Care in Subjects on Oral Medications for the Treatment of Interstitial Lung Disease, Sarcoid and Pulmonary Hypertension

Interstitial Lung Disease (ILD) is a group of conditions that cause progressive inflammation and often irreversible scarring of lung tissue. This fibrotic process disrupts oxygen and carbon dioxide exchange, leading to symptoms including dyspnea, cough, wheezing, chest discomfort, and hemoptysis. Patients with ILD experience substantial functional impairment; hospitalizations for respiratory failure carry a one-year mortality exceeding 50%. Long-term management frequently involves glucocorticoids, immunosuppressants (e.g., azathioprine), and antifibrotic agents - all of which carry significant side effects and substantial costs.

Pulmonary hypertension(PH) associated with lung disease is a serious and increasingly recognized complication of chronic parenchymal lung diseases, most notably interstitial lung disease (ILD) and chronic obstructive pulmonary disease. A recent systematic review and meta-analysis found a pooled prevalence of PH of 36% in ILD populations. Clinically, patients present with progressive exertional dyspnea and fatigue that is disproportionate to the severity of their underlying ILD, markedly reduced exercise capacity, worsening hypoxemia, and, in advanced disease, right heart failure. Management of remains challenging; most drugs approved for pulmonary arterial hypertension have demonstrated no benefit or potentially harmful effects in this population. A notable exception is inhaled treprostinil, which has demonstrated improvements in exercise capacity in patients with ILD-associated PH, representing a meaningful but limited advance in an area of substantial unmet therapeutic need.

Medication non-adherence is associated with greater morbidity and mortality across chronic diseases and has been estimated to increase U.S. healthcare costs by over $170 billion annually. For patients with ILD and PH - who carry high treatment burden, significant medication side effects, and complex regimens - adherence represents a particularly critical and modifiable variable.

The FORTISKAP™ platform (Cosmos Rx, Inc.) is a bottle-top electro-optical device that records time-stamped prescription bottle openings and estimates pill counts using proprietary image processing algorithms, providing an objective measure of adherence. A companion smartphone application delivers dose-deviation reminders to subjects, and a physician dashboard alerts clinicians to adherence trends. This study will generate the first prospective data on FORTISKAP™ performance in an ILD/PH population. Medication adherence is challenging for patients across the spectrum of medical disorders that are treated with chronic use of oral medications. Poor medication adherence can result in increased risk of complications,disease progression, increased healthcare utilization and poor therapeutic outcomes. While these therapies can be effective for certain ILD subtypes, they carry significant risks including cytopenias, nephrotoxicity, weight gain, adrenal suppression,etc and they can be expensive - creating a burden for the patient and the healthcare system.

4.3 Study Design/Statistical Analysis Plan