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stiffness. The renal criteria now include measurement of proteinuria by the urine
protein/creatinine ratio without the requirement of a time frame for collection.
In contrast to the older ACR criteria that included only seizures or psychosis as
neurologic manifestations of disease, the new criteria include many other neurologic
manifestations, including myelitis, peripheral or cranial neuropathy, mononeuritis
multiplex, and acute confusional state. The hematologic criteria have been subdivided
into three categories: hemolytic anemia, thrombocytopenia (<100,000/mm3), and either
leukopenia (<4,000/mm3) or lymphopenia (<1,000/mm3). Finally, the immunologic
criterion was expanded to include newly discovered antibodies present in SLE.

FIGURE 101.1 Adolescent girl with discoid lesions in malar distribution.

In 2018, a new system for the classification of adult SLE was proposed, with support
by the European League Against Rheumatism (EULAR) and the American College of
Rheumatology (ACR). The entry criterion for this classification is a positive ANA of
≥1:80 titer (HEp2 cells immunofluorescence). Specific clinical criteria (e.g., fever,
malar rash, synovitis in ≥2 joints) are divided into domains. There are seven clinical
and three immunologic domains. In each domain, the criteria are ordered from least to
most influential. There are also specific definitions for each criterion. The seven
clinical domains are constitutional, cutaneous, arthritis, serositis, hematologic, renal,
and neurologic. The three immunologic domains are other serologies, complement
proteins, and highly specific autoantibodies. The next step in developing this
classification system is to assign weights to the criteria, with an additive scoring
system. Only the most influential criterion in each domain will be scored. A threshold
will be set and those patients with a score above the threshold will be diagnosed as