Background/Purpose: Recently the term “interstitial pneumonia with autoimmune features” (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Until now, only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD.
The aim of the study was to investigate the therapeutic choices in IPAF patients, their efficacy and safety.

Methods: Fifty-two patients (mean age at diagnosis 65.5±11.0 years, female/male ratio 29/23) were consecutively enrolled and prospectively followed for 45±31.6 months. Data about therapies, disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. A worsening of lung function was defined as a reduction of 10% compared to baseline of forced vital capacity (FVC) and diffusion lung capacity of CO (DLCO).

Results: An immunosuppressive therapy was prescribed in 15 patients (namely cyclophosphamide in 4, mycophenolate mofetil in 6 and azathioprine in 6, respectively), while 6 patients were treated with anti-fibrotic therapies (pirfenidone or nintedanib). Thirty-three patients taken corticosteroids, associated to other drugs in 18 patients, and alone in 15. Finally, no therapies were prescribed in 16 patients. At the end of follow-up FVC remained stable in 35% of patients, worsened in 50% and increased in 15%; DLCO remained stable in 25.8% of patients, worsened in 58.1% and increased in 16.1%.
Mean survival was 94.2±8.5 months, and no differences were recorded according to the kind of therapy, while survival was significantly associated to the lung function at baseline (FVC and DLCO were confirmed to be significantly associated to death at multivariate analysis).

Conclusion: The therapeutic strategy in IPAF patients reflects the etherogeneity of the disease. Waiting for specific trial in this population, treatment is empyrical and based on the baseline features of the patients. Despite well tolerated, both anti-fibrotic and immunosoppressive therapies seem to not influence the evolution of the diseas.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/therapeutic-strategies-and-survival-in-patients-with-interstitial-pneumonia-with-autoimmune-features/