Clinical implications of interstitial pneumonia with autoimmune features diagnostic criteria in idiopathic pulmonary fibrosis: A case control study

Sara Tomassetti; Claudia Ravaglia; Silvia Puglisi; Athol U Wells; Jay H Ryu; Marcello Bosi; Alessandra Dubini; Sara Piciucchi; Francesco Girelli; Paola Parronchi; Federico Lavorini; Elisabetta Rosi; Valentina Luzzi; Marco Matucci Cerinic; Venerino Poletti

doi:10.3389/fmed.2023.1087485

Clinical implications of interstitial pneumonia with autoimmune features diagnostic criteria in idiopathic pulmonary fibrosis: A case control study

Front Med (Lausanne). 2023 Feb 16:10:1087485. doi: 10.3389/fmed.2023.1087485. eCollection 2023.

Authors

Sara Tomassetti^{1

2}, Claudia Ravaglia³, Silvia Puglisi³, Athol U Wells⁴, Jay H Ryu⁵, Marcello Bosi³, Alessandra Dubini⁶, Sara Piciucchi⁷, Francesco Girelli⁸, Paola Parronchi¹, Federico Lavorini¹, Elisabetta Rosi⁹, Valentina Luzzi², Marco Matucci Cerinic¹, Venerino Poletti^{3

10}

Affiliations

¹ Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence, Italy.
² Interventional Pulmonology Unit, Careggi University Hospital, Florence, Italy.
³ Department of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy.
⁴ ILD Unit, Pulmonary Medicine, Royal Brompton Hospital, London, United Kingdom.
⁵ Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, United States.
⁶ Pathology Unit, GB Morgagni Hospital, Forlì, Italy.
⁷ Radiology Unit, GB Morgagni Hospital, Forlì, Italy.
⁸ Internal Medicine, GB Morgagni Hospital, Forlì, Italy.
⁹ Pulmonary Unit, Careggi University Hospital, Florence, Italy.
¹⁰ Department Respiratory Diseases & Allergology, Aarhus University Hospital, Aarhus, Denmark.

Abstract

Background: A subgroup of IPF patients can meet IPAF criteria (features suggesting an underlying autoimmune process without fulfilling established criteria for a CTD). This study was aimed to evaluate whether IPAF/IPF patients compared to IPF patients differ in clinical profile, prognosis and disease course.

Methods: This is a retrospective, single center, case-control study. We evaluated 360 consecutive IPF patients (Forlì Hospital, between 1/1/2002 and 28/12/2016) and compared characteristics and outcome of IPAF/IPF to IPF.

Results: Twenty-two (6%) patients met IPAF criteria. IPAF/IPF patients compared to IPF were more frequently females (N = 9/22, 40.9% vs. N = 68/338, 20.1%, p = 0.02), suffered more frequently from gastroesophageal reflux (54.5% vs. 28.4%, p = 0.01), and showed a higher prevalence of arthralgias (86.4% vs. 4.8%, p < 0.0001), myalgias (14.3% vs. 0.3%, p = 0.001) and fever (18.2% vs. 1.9%, p = 0.002). The serologic domain was detected in all cases (the most frequent were ANA in 17 and RF in nine cases) and morphologic domain (histology features) was positive in 6 out of 10 lung biopsies (lymphoid aggregates). Only patients with IPAF/IPF evolved to CTD at follow-up (10/22, 45.5%; six rheumatoid arthritis, one Sjögren's and three scleroderma). The presence of IPAF was a positive prognostic determinant (HR 0.22, 95% CI 0.08-0.61, p = 0.003), whereas the isolated presence of circulating autoantibody did not impact prognosis (HR 1.00, 95% CI 0.67-1.49, p = 0.99).

Conclusion: The presence of IPAF criteria in IPF has a major clinical impact correlating with the risk of evolution to full blown-CTD during follow-up and identifying a subgroup of patients with a better prognosis.

Keywords: circulating autoantibodies; disease behavior; idiopathic pulmonary fibrosis; interstitial lung diseases; interstitial pneumonia with autoimmune features; mortality.