| Key messages |
| According to the new classification, IIPs are categorised as major, rare and unclassifiable |
| MDD discussion is the “gold standard” for the diagnosis of the IIPs |
| For entities with heterogeneity in the clinical course and that are difficult to classify, a disease behaviour classification complementary to IIP classification has been proposed |
| IPF is a progressive and lethal fibrosing lung disease with an unpredictable course |
| Disease stratification aims to identify subgroups of patients who present similar characteristics and should be treated accordingly or enrolled in treatment trials |
| Gene polymorphisms related to host defence and cell repair are associated with the development of IPF |
| Markers of IPF progression have been identified by blood transcriptomic and proteomic studies |
| A combination of blood transcriptome with demographic and functional characteristics provides better information on the outcome of IPF |
| Early diagnosis and treatment of IPF comorbidities may have a positive effect on patients quality of life |
| Disease stratification and early identification of patients more likely to progress, and thus need more aggressive treatment, is also important in CTD-ILD |
| Predicting the clinical course of sarcoidosis, especially lung fibrosis and cardiac and neurological involvement, is still challenging |
| Potential novel genomic biomarkers, such as multi-gene signatures, can be useful to predict complicated sarcoidosis |
| In the presence of cardiac sarcoidosis, ICD therapy may be used as a component of an aggressive primary prevention strategy, especially in those patients with ventricular dysfunction |
| As second-line therapy in sarcoidosis, methotrexate and azathioprine are equally effective in terms of pulmonary function improvement and have a significant steroid-sparing effect |
IIP: idiopathic interstitial pneumonia; MDD: multidisciplinary diagnosis; IPF: idiopathic pulmonary fibrosis; CTD: connective tissue disease; ILD: interstitial lung disease; ICD: implantable cardioverter defibrillator.