Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal disease. The treatment is challenging and nowadays a comprehensive approach based not only in pharmacological strategies is necessary. Identification and control of comorbidities, non-pharmacological treatment, prevention and management of exacerbations as well as other areas of care (social, psychological) are fundamental for a holistic management of IPF. Gastroesophageal reflux, pulmonary hypertension, obstructive sleep apnea, combined with emphysema, lung cancer and cardiovascular involvement are the main comorbidities associated with IPF. Non-pharmacological treatment includes the use of oxygen in patients with rest or nocturnal hypoxemia and other support therapies such as non-invasive ventilation or even a high-flow nasal cannula to improve dyspnea. In some patients, lung transplant should be considered as this enhances survival. Pulmonary rehabilitation can add benefits in outcomes such control of dyspnea, exercise capacity distance and, overall, improve the quality of life; therefore it should be considered in patients with IPF. Also, multidisciplinary palliative care programs could help with symptom control and psychological support, with the aim of maintaining quality of life during the whole process of the disease. This review intends to provide clear information to help those involved in IPF follow up to improve patients’ daily care.

Introduction: Connective tissue diseases (CTD), as Sjögren’s syndrome (SS), may involve the respiratory system and even debut as interstitial lung disease (ILD). The aim of this study was to evaluate the role of including minor salivary gland biopsy (MSGB) in the diagnostic approach of ILD. Methods: Retrospective study including ILD patients, CTD traits, and suspected primary SS, evaluated since 2013, which underwent an MSGB. Clinical, laboratory and histological data were collected. MSGB was considered positive when 1 or more focus score >50 lymphocytes/4mm3 was founded, except in patients under immunosuppressive treatment, in which the threshold was established in 20-50 lymphocytes/4mm3. Results: Thirty-six patients were included. Median age (±SD) was 70±10 years and 50% were female. Overall, 21 of the 36 MSGB (58%) were positive confirming SS diagnosis. In the MSGB positive group the patients were younger, with male gender predominance, UIP pattern (52%) and higher autoimmunity markers. Also, in 12 of those 21 patients (57%), the biopsy led to a therapeutic change. No statistically significant differences were found in the analyzed variables of the two groups, neither in the 1-year follow-up lung function tests. However, using the correlation statistic “Goodman and Kruskal’s lambda”, a correlation was found between the MGSB and the gender, age (under 75 years) and CT-scan pattern. Conclusions: The MSGB may be useful for SS diagnosis in patients with ILD and negative antibodies. Certain variables correlate with the positivity of the MSGB and should be confirmed in a larger prospective cohort.