Rheumatoid arthritis (RA) is a systemic autoimmune disease whose main extra-articular organ affected is the lung, sometimes in the form of diffuse interstitial lung disease (ILD) and conditions the prognosis. A multicenter, observational, descriptive and cross-sectional study of consecutive patients diagnosed with RA-ILD. Demographic, analytical, respiratory functional and evolution characteristics were analyzed to evaluate the predictors of progression and mortality. 106 patients were included. The multivariate analysis showed that the diagnostic delay was an independent predictor of mortality (HR 1.11, CI 1.01–1.23, p = 0.035). Also, age (HR 1.33, 95% CI 1.09–1.62, p = 0.0045), DLCO (%) (HR 0.85, 95% CI 0.73–0.98, p = 0.0246), and final SatO2 (%) in the 6MWT (HR 0.62, 95% CI 0.39–0.99, p = 0.0465) were independent predictor variables of mortality, as well as GAP index (HR 4.65, 95% CI 1.59–13.54, p = 0.0051) and CPI index (HR 1.12, 95% CI 1.03–1.22, p = 0.0092). The withdrawal of MTX or LFN after ILD diagnosis was associated with disease progression in the COX analysis (HR 2.18, 95% CI 1.14–4.18, p = 0.019). This is the first study that highlights the diagnostic delay in RA-ILD is associated with an increased mortality just like happens in IPF.

Though idiopathic pulmonary fibrosis (IPF) is characterized by single-organ involvement, many comorbid conditions occur within other organ systems. Patients with IPF may present during evolution different complications and comorbidities that influence the prognosis and modify the natural course of their disease. In this chapter, we highlight common comorbid conditions encountered in IPF, discuss disease-specific diagnostic modalities, and review the current treatment data for several key comorbidities. The diagnosis and treatment of these comorbidities is a challenge for the pulmonologist specialized in interstitial lung diseases (ILDs). We will focus on pulmonary emphysema, lung cancer, gastroesophageal reflux, pulmonary hypertension, obstructive sleep apnea (sleep disorders), and acute exacerbation of IPF.

Introduction Fibrotic hypersensitivity pneumonitis (fHP) is an immune-mediated interstitial lung disease caused by sensitization to chronic allergen inhalation. This study aimed to determine prognostic indicators of progression and mortality in fHP. Methods This was a retrospective, multicenter, observational, cross-sectional cohort study of consecutive patients diagnosed with fHP from 1 January 2012 to 31 December 2021. Multivariate Cox regression analyses were used to calculate hazard ratios (HRs) with 95% confidence interval (CI) for predictors of progression and survival. Results A total of 403 patients were diagnosed with fHP: median (IQR) age 66.5 (14.0) years, 51.9% were women and 55.1% were never smokers. The cause of fHP was mainly fungal (39.7%) or avian (41.4%). Lung biopsy was performed in 269 cases (66.7%). In the whole cohort the variables that were related to mortality-lung transplant were older age (HR=1.08, p<0.001), FVC(% pred) (HR=0.96, p=0.001), lymphocytosis in BAL (HR 0.93,p=0.001), presence of acute exacerbation during follow-up (HR=3.04,p=0.001) and GAP (Gender, Age, Physiology) index (HR=1.96,p<0.01). It stands out in multivariate Cox regression analyses in the group of biopsied patients the presence of fibroblastic foci at biopsy (HR=8.39,p<0.001) as highly significant predictor for increased mortality-lung transplant. Also, GAP, lymphocytosis in BAL and age are predictors of progression (HR=1.26,p=0.009; HR=0.97,p=0.018, and HR=1.03,p=0.018, respectively). Conclusion the study identified several prognostic factors for progression and/or survival in fHP. The presence of fibroblastic foci at biopsy was consistent predictor for increased mortality and the presence of lymphocytosis in BAL was inversely related to mortality.