Background/Purpose: We have limited knowledge of the characteristics of patients with interstitial pneumonia with autoimmune features (IPAF) that are associated with response to immunosuppression. Thus, we currently have no data to guide optimal IPAF patient selection for therapy or the optimal immunosuppressive regimen in IPAF. In this study, we use a rigorously phenotyped cohort (based on published IPAF criteria) to characterize features associated with response to immunosuppressive treatment.

Methods: We conducted a retrospective cohort study of 65 IPAF patients to evaluate for serologic, clinical, and radiographic characteristics associated with response to immunosuppression, defined as % relative functional vital capacity (FVC) decline of less than 10%, and absence of death or lung transplant within the first year of continuous immunosuppressive therapy. We used Fisher’s exact test, chi-squared test, and Mann Whitney U test, as appropriate, to evaluate for significant differences in characteristics between patients with significant progression and those who did not progress. Multivariate logistic regression was used to further evaluate significant variables while controlling for confounders.

Results: No baseline features predicted IPAF progression while on treatment, There was a trend of greater progression amongst men, ever smokers, and those with UIP radiographic pattern, although the differences were not statistically significant (Table 1). There was a statistically significant difference in the proportion of patients who were treated with combination therapy of mycophenolate mofetil (MMF) and prednisone whose disease did not progress.

Conclusion: Our study suggests that a combination therapy with MMF and prednisone may be associated with lack of progression in IPAF, including IPAF-UIP, although causality cannot be established. In the future studies, knowledge of cytokine profiles in different IPAF phenotypes may inform management in the early stages of the disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/variables-associated-with-response-to-therapy-in-patients-with-interstitial-pneumonia-with-autoimmune-features/