YW Cheah¹, YH Lim¹, JY Chan¹, NL Lai¹, Haly Rozie. A², SK Teoh¹
¹Respiratory Unit, Ampang Hospital, Selangor, Malaysia
²Department of Respiratory Medicine, Serdang Hospital, Selangor, Malaysia
Introduction
Rheumatoid arthritis–associated interstitial lung disease (RA-ILD) is a known extra-articular manifestation of RA, often under-recognised in its early stages. Although RA-ILD may remain stable for some, but a subset of patients can progress to progressive pulmonary fibrosis (PPF), characterised by worsening symptoms, physiological decline, and radiological progression despite appropriate immunosuppressive treatment.
Case report
We present a case of a 20-year-old female with seropositive RA, initially treated with methotrexate but later developed ILD confirmed via HRCT-Thorax. Despite immunosuppressive therapy including corticosteroids, cyclophosphamide and rituximab, she showed evidence of disease progression with worsening lung function and exercise-induced desaturation. Repeat imaging and spirometry supported the diagnosis of PPF.
In view of this, antifibrotic therapy with Nintedanib was initiated, in accordance with findings from the INBUILD trial which demonstrated its efficacy in slowing the decline in forced vital capacity (FVC) among patients with non-IPF progressive fibrosing ILDs. She was subsequently referred to lung transplant team in Serdang Hospital as it remains the definitive treatment in improving quality of life and survival rate. While access to lung transplantation in Malaysia has historically been limited, significant progress is underway. A dedicated transplant team is now available in Malaysia mainly stationed in Serdang Hospital and Seremban Hospital, and the country is moving towards the establishment of its national lung transplantation program.
Conclusion
This case is reported with the aim of highlighting the importance of early identification of PPF in RA-ILD, timely initiation of antifibrotic therapy and the need for structured pathways towards lung transplantation in Malaysia.