In lupus erythematosus (ICD-10-GM L93.-: Lupus erythematosus; ICD-10-GM M32.-: Systemic lupus erythematosus) is a group of autoimmune diseases in which the formation of autoantibodies occurs. It belongs to the group of collagenoses.
According to the ICD-10, the following forms of lupus erythematosus can be distinguished:
- Systemic lupus erythematosus (SLE; ICD-10-GM M32) – severe multisystemic disease.
- Discoid lupus erythematosus (DLE; ICD-10-GM L93.0) – form of lupus erythematosus confined to the skin.
- Subacute lupus erythematosus cutaneus (SCLE; ICD-10-GM L93.1) – mild form of multisystem disease.
- Other localized lupus erythematosus (lupus erythematosus profundus; ICD-10-GML93.2).
In addition, there are intermediate forms as well as variants, but also drug-induced forms.
Systemic lupus erythematosus (SLE) represents the most severe form. It is a systemic disease of unclear genesis (origin) in which autoantibodies are formed that damage cells and tissues.
Discoid lupus erythematosus (chronic discoid lupus erythematosus, CDLE) is a form of lupus erythematosus confined to the skin (in 90% on the head).
Subacute cutaneous lupus erythematosus (SCLE) is a mild form of the disease. A familial clustering can be observed. Sex ratio: systemic lupus erythematosus: Males to females is 1: 4.Discoid lupus erythematosus: Men to women is 1: 2-3.Subacute cutaneous lupus erythematosus: Males to females is 1: 3 to 1: 6.Juvenile systemic lupus erythematosus: Girls hardly more common than boys.
Age of first manifestation of systemic lupus erythematosus: 5-55 years of age.
Peak incidence: systemic lupus erythematosus (SLE) occurs predominantly between the ages of 20 and 40 years. In childhood, the prevalence of SLE is probably a power of ten lower: in about 10-15% of cases, systemic lupus erythematosus begins in childhood and adolescence (juvenile systemic lupus erythematosus), in individual cases already in infancy.
The prevalence of systemic lupus erythematosus (SLE) is 36.7/100,000 (in Germany). The prevalence for subacute cutaneous lupus erythematosus is two to three times higher than for systemic lupus erythematosus (in Germany). The disease is more common in the black U.S. population than in the white population. In Central Africa, the disease is unknown.
The incidence (frequency of new cases) for systemic lupus erythematosus is approximately 5-10 cases per 100,000 population per year (in Germany).
Course and prognosis: The disease usually progresses in relapses, which may be interspersed with remission phases (symptom-free phases) lasting several months. In systemic lupus erythematosus, internal organs are frequently affected – lupus nephritis (inflammation of the kidneys) occurs in about 50% of patients. The chances of cure depend on the extent of kidney damage. Juvenile systemic lupus erythematosus is associated with severe courses as well as more frequent organ involvement. The prognosis for discoid lupus erythematosus is favorable because only the skin is involved in 95% of cases. Subacute cutaneous lupus erythematosus occupies an intermediate position with respect to prognosis.
The 10-year survival rate for systemic lupus erythematosus is ≥ 90%. Affected individuals usually die from cardiovascular disease (coronary artery disease (CAD), myocardial infarction (heart attack)), infection, uremia (occurrence of urinary substances in the blood above normal levels), neurologic complications, or thromboembolism (vascular occlusion).
