For patients who respond to initial treatment, the recommended maintenance therapy is lower immunosuppression, with either mycophenolate mofetil/mycophenolic acid or azathioprine for at least 3 years in combination with low-dose prednisone. SLE has improved markedly in recent decades because of earlier diagnosis and optimized treatment. Further research and randomized controlled trials are needed for the development of specifically targeted therapies. Systemic lupus erythematosus (SLE) is usually a heterogeneous autoimmune disease that may involve many different organs and display a variable clinical course. Compound 401 The diagnosis of SLE is based on characteristic clinical findings of the skin, joints, kidneys, and the central nervous system, as well as on serological parameters such as antinuclear antibodies (ANA), Compound 401 in particular antibodies to dsDNA (e1). The various clinical symptoms do not usually occur simultaneously and may develop at any stage of the disease. In the early stages, physicians from numerous disciplines often propose several differential diagnoses, or identify only one aspect of the disease without realizing the symptoms as part of SLE (1, e2). Fever, fatigue, and arthralgia are the most frequently occurring non-specific symptoms at disease onset; additional joint swelling or a “butterfly rash”particularly in women of childbearing ageshould prompt concern of SLE (2). The aim of this short article is usually to provide an updated review around the diagnosis and treatment of SLE, based on a selective survey of the Compound 401 literature in PubMed and the Cochrane Library, including current guidelines and the recommendations of experts with extensive experience in the management of this disease. Epidemiology and Prognosis The prevalence of SLE in Germany in the year 2002 was 36.7/100 000, with a 4:1 ratio of women to Rabbit Polyclonal to CCDC45 men (3). The prevalence of pediatric-onset SLE is probably lower by a factor of ten (e3). The disease often begins in puberty; if SLE is usually diagnosed in patients under the age of 5 years, a rare monogenic form may be present. The survival rate has risen significantly in recent decades (1955 vs. 2003: 5-12 months survival rate 5% vs. 95%; 10-12 months survival rate 0% vs. 92%), mainly due to earlier diagnosis and improved management (4, 5, e4). During the first years after the onset of SLE, mortality is usually increased mainly due to disease activity and bacterial infection as a result of high glucocorticoid dosage (e5, e6), while cardiovascular complications predominate in the period beginning 5 years after initial diagnosis (e7, e8). Classification criteria The criteria of the American College of Rheumatology (ACR), first published in 1982 and revised in 1997, can be applied for the Compound 401 classification of SLE (6, 7, e9). Four of the 11 criteria have to be fulfilled for a diagnosis of SLE. As 4 of the criteria include mucocutaneous lesions, the application of the ACR criteria without analysis of autoantibodies may result in an overestimation of SLE (8, e2). Therefore, the Systemic Lupus International Collaborating Clinics (SLICC) group developed a new set of classification criteria in 2012 (Box 1) (9). Currently both units of criteria (ACR and SLICC) are often applied simultaneously. Box 1 Classification of SLE: the Systemic Lupus International Collaborating Clinics (SLICC) Classification Criteria* Clinical criteria Acute cutaneous lupus erythematosus (including butterfly rash) Chronic cutaneous lupus erythematosus (e.g., localized or generalized discoid lupus erythematosus) Oral ulcers (on palate and/or nose) Non-scarring alopecia Synovitis ( 2 joints) or tenderness on palpation ( 2 joints) and morning stiffness ( 30 min) Serositis (pleurisy or pericardial pain for more than 1 day) Compound 401 Renal involvement (single urine: protein/creatinine ratio or 24-hour urine protein, 0.5 g) Neurological involvement (e.g., seizures, psychosis, myelitis) Hemolytic anemia Leukopenia ( 4000/L) or lymphopenia ( 1000/L) Thrombocytopenia ( 100 000/L) Immunological criteria ANA level above laboratory research range Anti-dsDNA antibodies Anti-Sm antibodies Antiphospholipid antibodies (anticardiolipin and anti- 2-glycoprotein I [IgA-, IgG- or IgM-] antibodies; false-positive VDRL [Venereal Disease Research Laboratory] test) Low match (C3, C4, or CH50) Direct Coombs test (in the absence of hemolytic anemia) *Modified (short form) after (9). For classification as SLE, four criteria (at least one of them clinical and at least one immunological) have to be fulfilled or lupus nephritis has to be diagnosed histologically in the presence of ANA or anti-dsDNA antibodies. The SLICC criteria are not.