The Diagnosis and Treatment of Systemic Lupus Erythematosus

Abstract

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with a prevalence of 36.7/100 000 in Germany and a female/male ratio of 4:1. The clinical course is variable, with a broad spectrum of organ manifestations; lupus nephritis develops in about half of all patients.

Methods: This review is based on a selective search of PubMed and the Cochrane Library, including current guidelines and expert recommendations.

Results: Assessment of clinical symptoms, laboratory findings, and optional biopsy results are the basis for early diagnosis of SLE. All patients should be treated with antimalarials as soon as the diagnosis is confirmed. In particular, hydroxychloroquine is associated with a higher rate of remission, fewer relapses, and reduced damage in the course of the disease, even in lupus nephritis. High-dose glucocorticoids should be given only when acutely indicated; immunosuppressives such as azathioprine, methotrexate, or mycophenolate mofetil may be administered to reduce glucocorticoids, according to the EULAR recommendations. Belimumab was recently approved as add-on therapy in autoantibody-positive SLE patients with high disease activity unresponsive to standard treatment. Short-term induction pulse therapy with low-dose intravenous cyclophosphamide, as well as continued mycophenolate mofetil treatment are advances in lupus nephritis.

Conclusion: The long-term prognosis for 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.

Figure 1

a) Discoid lupus erythematosus (DLE) on the right cheek and ear: scarring with hyperpigmented border and residual activity (preauricular erythema with keratosis on the earlobe and comedo-like lesions in the meatus); b) Discoid lupus erythematosus (DLE) on the scalp: confluence of several lesions with erythema, silvery-white adherent hyperkeratoses and scarring alopecia, as well as DLE on the ear; c) Subacute cutaneous lupus erythematosus (SCLE) on the back and the extensor surface of the arms: polycyclic confluence of annular erythematous lesions with collarette scaling at the inner border and central clearing; d) Joint deformation without radiologic erosions (Jaccoud arthropathy)

Figure 2

a) “Active“ urinary sediment in lupus nephritis: if more than 5% of all erythrocytes in the urine show the appearance of so-called acanthocytes (“Micky Mouse ears“), glomerulonephritis is probable. Simultaneous albuminuria supports the suspicion; b) Antinuclear antibodies (ANA; HEp-2 cell test): homogeneous pattern with positive mitoses