A novel take on idiopathic pulmonary fibrosis disease progression: localised autoimmunity

It is well established that several of the interstitial lung diseases (ILDs) are driven by autoimmunity, such as rheumatoid arthritis-associated ILD and systemic sclerosis-associated ILD. These are therefore considered to be autoimmune diseases and frequently treated with immunosuppressive drugs. Idiopathic pulmonary fibrosis (IPF) is not viewed as an autoimmune disorder, but nevertheless, autoantibodies routinely studied in clinical practice are found in IPF and other types of idiopathic interstitial pneumonias. Serological autoreactivities, such as anti-cyclic citrullinated peptide antibody (anti-CCP), antinuclear antibody, rheumatoid factor (RF) and anti-neutrophil cytoplasmic antibody, have been reported in IPF [1] similar to patients with a connective tissue disease. Localised autoimmune responses in the lungs might contribute to disease progression in IPF