Lupus (in short)
- Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which is
autoimmune in origin and is characterized by the presence of autoantibodies directed
against nuclear antigens.
- It is a multi-system disease, and patients can present in
vastly different ways.
- Frequency varies with ethnicity, but is estimated to be about
1 per 1000 overall with a female to male ratio of 10:1.
- The clinical heterogeneity
of this disease mirrors its complex causes, which highlights the importance
of genetic factors and individual susceptibility to environmental factors.
- Lupus can
affect every organ in the body. The most common manifestations include rash, arthritis
and fatigue.
- At the more severe end of the spectrum, lupus can cause nephritis, neurological
problems, anaemia and thrombocytopaenia.
- Over 90% of patients with lupus have positive
anti-nuclear antibodies (ANA). Significant titres are accepted to be of 1:80 or greater.
- Lupus is a relapsing and remitting disease, and treatment aims are threefold: managing
acute periods of potentially life-threatening ill health, minimizing the risk of flares
during periods of relative stability, and controlling the less life-threatening, but
often incapacitating day to day symptoms.
- Hydroxychloroquine (Plaquenil) and non-steroidal anti-inflammatory
drugs are used for milder disease;
- corticosteroids and immunosuppressive therapies
are generally reserved for major organ involvement;
- anti-CD20 monoclonal antibody
is now used in patients with severe disease who has not responded to conventional
treatments.
- Despite enormous improvements in prognosis since the introduction of corticosteroids
and immunosuppressive drugs, lupus continues to have a significant impact on the mortality
and morbidity of those affected.
- Authors: Jessica J Manson and Anisur Rahman. Orphanet Journal of Rare Diseases