We Need Better Classification and Terminology for “People at High Risk of or in the Process of Developing Lupus”

Systemic lupus erythematosus (SLE), like rheumatoid arthritis (RA) and other connective tissue diseases (CTDs), does not develop on the day it is diagnosed. Rather, as rheumatologists know, the onset can be days to years before SLE is clinically diagnosed. The recognition of SLE, a prototypic autoimmune disease with a striking heterogeneity and range of manifestations, can be a challenge1, 2. In some individuals, full-blown disease, with a “full house” of autoantibodies and aggressive, life-threatening disease, such as lupus nephritis or vasculitis, seems to develop overnight. In others, however, the onset is insidious and difficult to recognize and to distinguish from other conditions. The diagnosis of SLE among patients seen in consultation, most often for a positive antinuclear antibody (ANA) and one or two other non-specific symptoms, such as arthralgias, myalgias, fatigue or rashes, is one of the common clinical challenges faced by rheumatologists. The challenge is compounded by the fact that SLE is likely a spectrum of related diseases that share immunologic and clinical features.