Interstitial Lung Disease

Pulmonary arterial hypertension in combination with ILD associated with worse overall prognosis

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The prevalence of pulmonary arterial hypertension (PAH) in combination with interstitial lung disease (ILD) is 7%, and individuals with this combination of conditions experienced poorer survival compared to those with ILD or scleroderma (SSc) alone, according to a study. The presence of PAH was associated with a worse overall prognosis than even extensive ILD.

Out of 1561 participants in the Australian Scleroderma Cohort Study (ASCS), 7% fulfilled the criteria for PAH-only, 24% had ILD-only, 7% had both PAH and ILD (PAH-ILD), and 62% had SSc-only.

Individuals with PAH-ILD were more frequently male, had diffuse skin involvement, higher inflammatory markers, older age of SSc onset, and a higher frequency of extensive ILD compared to the overall cohort (P < 0.001). People of Asian race were more prone to developing PAH-ILD (P < 0.001).

Individuals with PAH-ILD or PAH-only exhibited worse World Health Organization functional class and 6-minute walk distance compared to those with ILD-only. HRQoL scores were particularly impaired in individuals with PAH-ILD.

Survival rates were significantly reduced in the PAH-only and PAH-ILD groups (P < 0.01).

Using multivariable hazard modeling, the researchers determined that extensive ILD combined with PAH led to the worst prognosis, followed by PAH-only and PAH with limited ILD.

Fairley JL, Hansen D, Ross L, et al; Australian Scleroderma Interest Group. Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis. Arthritis Res Ther. 2023;25(1):77. doi: 10.1186/s13075-023-03059-x. PMID: 37173780; PMCID: PMC10176744.