Heterogeneous prognosis of individuals with desquamative interstitial pneumonia

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Desquamative Interstitial Pneumonia (DIP) is a rare and poorly described form of idiopathic interstitial pneumonia. We identified all patients with a diagnosis of DIP given in a multidisciplinary discussion in the frame of the French OrphaLung reference centers network. Among 77 screened patients, 25 patients were excluded (alternative diagnosis, n=17 ; no lung function test available, n=7; paediatric case, n=1). The main clinical characteristics at DIP diagnosis of the 52 patients included are presented in Table 1. After diagnosis, 9 patients (18%) quitted smoking, and pulmonary improvement was observed in 3 (33%) of them. Fourteen patients (27%) received corticosteroids, 7 (14%) antifibrotic drugs and 6 (12%) immunosuppressive therapy. After a median follow-up of 26 months (11-92 months), one patient died, 2 (4%) developed pulmonary hypertension and 8 (15%) developed lung cancer. FVC change was 22 mL/year (-85–101). FVC improved (>5% per years) in 9 patients (17%). Seven patients (13%) showed disease progression (loss of >5% of FVC per year). Our data emphasised the heterogeneous evolution of DIP. Analyses are underway to identify predictive factors for progression, with additional patients to be included. erj;66/suppl_69/PA1843/TB1 T1 TB1 Table 1 Quantitative variables are presented as median (range) and qualitative as number (%) Age at diagnosis (years) 54 (49–60) Male 44 (85) Current smokers 49 (94) Former smokers 3 (6) Pack-years 35 (26–46) Cannabis users 16 (31) Fibrogenic exposure 21 (40) Physical exam Dyspnea 36 (69) Velcro crackles 18 (35) Digital clubbing 23 (44) PFTs FVC % predicted 89 (79- 100) DLCO % predicted 60 (45-74) FEV1/FVC < 0.7 15 (29) BAL eosinophil count > 20 % 3 (6) Surgical lung biopsy 17 (33)

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