Demystifying interstitial lung disease

Irish Society for Rheumatology Autumn Meeting, Radisson Blu Hotel, Little Island, Cork, 18-19 September 2025

Dr Michael Henry, Consultant Respiratory Physician and Director of the Regional Interstitial Lung Disease (ILD) Service, Cork University Hospital (CUH), delivered a talk at the Irish Society for Rheumatology Autumn Meeting titled ‘Interstitial lung disease demystified’.

Dr Henry began by examining the diagnostic challenges associated with progressive pulmonary fibrosis (PFF). He outlined how up to 40 per cent of patients with other types of ILD also develop a progressive fibrosing phenotype that shows similarities in underlying pathogenic mechanisms and clinical behaviour to idiopathic pulmonary fibrosis (IPF).

Rheumatoid arthritis (RA), systemic sclerosis, and polymyositis/dermatomyositis are classed as connective tissue disease-related ILDs, he added.

Disease progression in IPF is usually monitored by a decrease in forced vital capacity (FVC). Dr Henry highlighted criteria from the INBUILD trial, which used specific criteria to identify progressive fibrosing ILDs that were not IPF, defining disease progression within the 24 months before screening based on a number of factors.

Data from CUH, he said, showed a 30-month survival rate of 55-60 per cent among patients with IPF who were commenced on full-dose pirfenidone and nintedanib. He added that the data revealed no statistical difference in survival rates between patients on pirfenidone and nintedanib.

He also highlighted data from the Fibroneer-ILD trial of nerandomilast in patients with PPF, which was published earlier this year in the New England Journal of Medicine. The results suggest that patients with PPF who were treated with nerandomilast had a small decline in FVC over a 52-week period compared to placebo.

The treatment of scleroderma-ILD (SSc-ILD), both early/inflammatory and fibrotic/inflammatory, was highlighted. Dr Henry noted that, to date, pirfenidone has not been well studied in SSc-ILD.

He said, “if all else fails, it’s lung transplantation,” as the evidence for lung transplant in scleroderma “is pretty good”.

“IPF [is] now the largest group of patients that get lung transplants in Ireland,” he said. “But the transplant programme has not progressed particularly well in Ireland over the last couple of years. The numbers are not going up.”

Turning to RA-ILD, he said that smoking is a big risk factor and seems to predispose patients to the disease. Patients with RA-ILD have a survival rate of five to seven years “if lucky”, he added.

Methotrexate is the number one go-to treatment for RA-ILD. Abatacept is “not bad” while data on the effectiveness of rituximab is poor, despite it being commonly used, he said.

Lastly, Dr Henry looked at drug-induced ILD and noted that cases have increased over the years, adding that in such cases, the drug should be stopped at an early stage.