A few studies have been performed applying well-established SLE disease activity scores in cohorts of patients with CLE. Evaluation of 176 patients with CLE by the Systemic Lupus Activity Measure (SLAM) and follow-up for more than 5 years (mean, 9 years) showed that most had mildly to moderately active disease. Ninety-seven patients had localized discoid LE, 59 had disseminated DLE, and 20 had SCLE. With SLAM criteria, 85 patients (48%) had low-activity disease, 72 (41%) had mild-activity disease, 15 (9%) had moderate-activity disease, and only 4 (2%) had very active disease. Photosensitivity, alopecia, oral ulcers, and Raynaud's phenomenon may indicate a worse prognosis (Parodi et al. 2000). Among the 50 patients followed up, SLAM scores decreased in 27, remained the same in 14, and increased in 9. The systemic manifestations at follow-up were arthritis, elevated erythrocyte sedimentation rate, and lymphopenia. These data indicate a good prognosis for CLE. However, the SLAM is far from being fully satisfactory. LE-specific skin lesions are not subdivided into different categories, but malar rash, DLE, lupus profundus, and bullous LE are all placed in the same group. However, these specific LE skin lesions are not equivalent regarding prognosis. Disease activity measured by the Systemic Lupus Erythemato-sus Disease Activity Index (SLEDAI) demonstrated that cases of SLE with nonspecific skin lesions have higher disease activity than those with specific skin lesions, supporting the fact that the latter group has a more benign course, including patients with DLE/SCLE (Zecevic et al. 2001).
Well-documented follow-up studies in patients with CLE are rare. However, continuous follow-up in 34 patients with SCLE documenting a wide panel of clinical and immunserologic data revealed that none of these patients developed severe LE crisis or died during 3 years of observation. Remarkably, the clinical symptoms, which occurred significantly more often, were acrolocalized vasculitis and arthralgias,being regarded as markers indicating mild progression of the disease (Tebbe et al. 1994). Nevertheless, a lethal outcome of CLE seems to be rare; in 10 years of follow-up, 10% of the patients with SCLE died,but only one from LE complications (Sontheimer 1989).
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