Systemic Sclerosis Associated Interstitial Lung Disease and Nintedanib: A Rare Disease and a Promising Drug

Abstract

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is a rare disease with a progressive nature, eventually leading to lung fibrosis. Nintedanib, a tyrosine kinase inhibitor, is a widely accepted drug for treating idiopathic pulmonary fibrosis (IPF), a disease that shares some similarities with SSc-ILD regarding pathological disease processes. In this review, we aim to discuss the pathogenesis of SSc-ILD and the overall role of nintedanib in the management of SSc-ILD. SSc-ILD involves multiple pathological mediators contributing to various pathways that ultimately cause lung fibrosis. The pathogenesis of SSc-ILD is a complex phenomenon and still needs further study. Nintedanib has demonstrated its efficacy in the treatment of SSc-ILD by reducing the progression of the pathological process. It has also proven its clinical significance in the management of SSc-ILD. However, the currently available literature does not have any evidence to compare the effectiveness of nintedanib with the already available treatment modalities such as cyclophosphamide (CYC), mycophenolate mofetil (MMF), and azathioprine (AZT). The current literature also lacks information about nintedanib's long-term consequences on patients with SSc-ILD. Therefore, to create better evidence-based treatment guidelines, we recommend that researchers conduct randomized clinical trials comparing nintedanib to MMF, CYC, AZT, etc., and continue surveillance to explore the longterm consequences of nintedanib.

The Role of SOX9 in IGF-II-Mediated Pulmonary Fibrosis

“…However, Nintedanib and tocilizumab are currently the only FDA-approved drugs for the treatment of SSc-PF [6][7][8]. These treatments merely slow the progression of fibrosis and fail to halt or reverse disease [9,10]. Lung transplantation is the only curative option, but it is not feasible at the scale that is needed [4].…”

Systemic sclerosis related interstitial lung disease and nintedanib

“…Systemic sclerosis (SSc) is a rarely seen connective tissue disease causing fibrosis of various internal organs, including kidneys, heart, lungs, musculoskeletal system and gastrointestinal tract and skin (1,2). Interstitial lung disease (ILD), develops in approximately 50% of patients within five years of being diagnosed with SSc (3).…”