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Interstitial lung disease (ILD) is a group of lung diseases affecting the lung tissue and its associated lymphatics and small blood vessels. It may occur as part of an autoimmune injury to the lungs followed by an abnormal healing response, leading to fibrosis or scar tissue that replaces normal lung tissue. Idiopathic interstitial pneumonia is the term given to ILDs representing up to two-thirds of cases.
ILD has been sub classified into 7 subgroups:
Some of these subtypes are associated with autoantibodies seen in a spectrum of autoimmune diseases. The onset of ILD may herald progression to another autoimmune condition such a scleroderma, inflammatory myositis and others, or, the other diseases may develop ILD during their course. Autoantibodies can be useful ion distinguishing different subsets of ILD as well as serving as an indicator of another autoimmune disease evolving.