Objective The Systemic Lupus Collaborating Clinics (SLICC) revised and validated the American College of Rheumatology (ACR) SLE classification criteria in order to improve clinical relevance, meet stringent methodology requirements and incorporate new knowledge in SLE immunology. Methods The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. SLICC validated the classification criteria in a new validation sample of 690 SLE patients and controls. Results Seventeen criteria were identified. The SLICC criteria for SLE classification requires: 1) Fulfillment of at least four criteria, with at least one clinical criterion AND one immunologic criterion OR 2) Lupus nephritis as the sole clinical criterion in the presence of ANA or anti-dsDNA antibodies. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications than the current ACR classification criteria (49 versus 70, p=0.0082), had greater sensitivity (94% versus 86%, p<0.0001) and equal specificity (92% versus 93%, p=0.39). In the validation set, the SLICC Classification criteria resulted in fewer misclassifications (62 versus 74, p=0.24), had greater sensitivity (97% versus 83%, p<0.0001) but less specificity (84% versus 96%, p<0.0001). Conclusions The new SLICC classification criteria performed well on a large set of patient scenarios rated by experts. They require that at least one clinical criterion and one immunologic criterion be present for a classification of SLE. Biopsy confirmed nephritis compatible with lupus (in the presence of SLE autoantibodies) is sufficient for classification.

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Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular changes including periungual telangiectasia, a photodistributed erythema or poikiloderma, and a scaly alopecia. Dermatomyositis has been linked to cancer, particularly ovarian cancer. Cancer-associated disease is more commonly found in older patients, and when present, is associated with a poor prognosis. A childhood form of the disease exists and is frequently complicated by the development of calcinosis. Dermatomyositis is a systemic disorder and whereas the skin and muscles are the most commonly affected organs, patients may have arthralgias, arthritis, oesophageal disease, or cardiopulmonary dysfunction. Recently described serological abnormalities, known as myositis-specific antibodies, add credence to the notion that this disorder is distinct from all other collagen-vascular diseases, and may lead to important discoveries about the pathogenesis of the inflammatory myopathies, which are not currently of practical use in the clinic or office. Management of the patient with myositis usually includes systemic corticosteroids with or without an immunosuppressive agent. Cutaneous disease is more difficult to manage, but antimalarials, methotrexate, and intravenous immunoglobulin are effective in small, often open-label, studies.

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