‘Dismal Prognosis’ Follows Acute Exacerbation of ILD

Acute exacerbation of systemic autoimmune disease-interstitial lung disease (SAD-ILD) is not uncommon and has a dismal prognosis, according to a systematic review and meta-analysis published online in BMC Pulmonary Medicine.

“Acute exacerbation is a devastating phenomenon and reported to be complicated with systemic autoimmune disease-associated interstitial lung disease,” researchers explained. “The aim of this study was to investigate the incidence and prognosis of acute exacerbation of systemic autoimmune disease-interstitial lung disease and clarify relevant clinical information predictive of these outcomes.”

The 24 studies in the review and analysis included 420 patients who developed acute exacerbation of SAD-ILD, 45.7% of whom were men, researchers reported. In 34.2% of cases, the underlying systemic autoimmune disease was rheumatoid arthritis. Polymyositis/dermatomyositis was the underlying systemic autoimmune disease in 31.9% of cases.

According to the study, the frequency of acute exacerbation of SAD-ILD was diverse across 17 studies, ranging from 4.3% to 32.9%. The incident rate was 3.19 and 5.77 per 100 patient-years in 2 studies. In 5 studies, 90-day all-cause mortality was between 30% and 58.3%.

The development of acute exacerbation of SAD-ILD was significantly associated with older age at initial presentation and lower percentage of predicted diffusing capacity of the lung for carbon monoxide. At acute exacerbation, lower partial pressure of arterial oxygen/fraction of inspired oxygen ratio (PaO2/FiO2) was significantly linked with all-cause mortality.

“As PaO2/FiO2 reflects the severity of acute exacerbation and represents pulmonary state at the onset of the disease,” researchers wrote, “it makes sense that the index can portend the prognosis of acute exacerbation of systemic autoimmune disease-interstitial lung disease.”

—Jolynn Tumolo

Reference

Kamiya H, Panlaqui OM. A systematic review of the incidence, risk factors and prognosis of acute exacerbation of systemic autoimmune disease-associated interstitial lung disease. BMC Pulm Med. 2021;21(1):150.