Incomplete lupus syndromes

Patients who meet less than four criteria using either the American College of Rheumatology (ACR) or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) sets are generally considered to have incomplete lupus erythematosus (ILE). This is a heterogeneous group of patients with various clinical manifestations including skin rashes, arthritis, Raynaud's phenomenon and cytopenias. It is generally agreed that all ILE patients also have significant ANA positivity, but other more specific serologies are not common. For most ILE patients, the condition is less severe or organ-damaging than in patients with SLE; nephritis and neurologic manifestations are rare. While many ILE patients remain in this unclassified state indefinitely, a subset does progress to full SLE. For these patients ILE is a transitional and early disease phase during which it is possible to modifying environmental risk factors and use medications to improve outcomes. Development of algorithms or biomarkers to identify which ILE patients are most at risk for progression is an area of current investigation.