Type I interferon is a potent substance. As such, the induction, transmission, 
and resolution of the type I interferon-mediated immune response are tightly 
regulated. As defined, the type I interferonopathies represent discrete examples 
of a disturbance of the homeostatic control of this system caused by Mendelian 
mutations. Considering the complexity of the interferon response, the 
identification of further monogenic diseases belonging to this disease grouping 
seems likely, with the recognition of type I interferonopathies becoming of 
increasing clinical importance as treatment options are developed based on an 
understanding of disease pathology and innate immune signaling. Definition of 
the type I interferonopathies indicates that autoinflammation can be both 
interferon and noninterferon related, and that a primary disturbance of the 
innate immune system can "spill over" into autoimmunity in some cases. Indeed, 
that several non-Mendelian disorders, most particularly systemic lupus 
erythematosus and dermatomyositis, are also characterized by an up-regulation of 
type I interferon signaling suggests the possibility that insights derived from 
this work will have relevance to a broader field of clinical medicine.