Dr. Syed Atiqul Haq
MBBS, MD, FCPS, FRCP (Edin.)
FCPS (Pak.)
Professor of Medicine
(Rheumatology Wing), BSMMU

APLAR-COPCORD Coordinator
Chairman, Standing Committee on Epidemiology

APLAR General Secretary, Bangladesh Rheumatology Society

Vice President, Association of Physicians of Bangladesh
   

INTRODUCTION

In Latin, Lupus means Wolf. The rash therefore was said to resemble the skin which had actually been bitten by a wolf. The name lupus erythematosus (red) is given due to the facial rash, called butterfly rash which is common in this disease. When it was found that many organs (systems) of the body were affected then it was renamed Systemic Lupus Erythematosus.

HISTORY

Hippocrates (460-375 BC) was the first to describe red ulcerating skin lesions which was thought to be SLE. Herbernus of Tours (916 AD) first introduced the term lupus. Dr. Robert Willian (1757-1812) described it as destructive and ulcerative skin disease. French Dermatologist Blett introduced the term lupus erythematosus and confirmed that this is distinct from other ulcerating skin disease. Finally, Moriz Kaposi (1872) divided lupus into the discoid & systemic forms and first to describe the butterfly rash.

DEFINITION

Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease, particularly prevalent in women, probably with a genetic predisposition with triggering from an environmental stimulus, resulting in the production of pathogenic autoantibodies and immune complexes which produce the pathologic features of the disease.

 

EPIDEMIOLOGY

SLE can occur at any age but has its onset primarily between age 16 and 55. About 90% patients are women. The prevalence of SLE in children and older adults is 1:100000. The prevalence varies with race, ethnicity and socioeconomic status. SLE is more common among American blacks, Hispanics and blacks in the Caribbean but it is seen infrequently in blacks in Africa. Asians are affected to approximately the same extent as American blacks. The disease appears to be common in China, Southeast Asia and India. Extrapolation of prevalence rate of SLE in Bangladesh is 0.54%.

AETIO-PATHOGENESIS

The cause of SLE remains elusive. It is likely that there is no single cause. Rather a complicated and multifactorial interaction among various genetic, environmental factors and sex hormones are probably involved. It is thought to be resulting from a disorder in the immune regulation system whereby an immune response is induced against host antigens and subsequently leads to inflammation and irreversible damage to target organs. An environmental trigger acts upon a genetically susceptible individual to create T cell and B cell defects that result in increased autoantibody production.

Genetic susceptibility

SLE occurs in the relatives of the patients with a frequency hundred-folds that in the general population. The monozygotic twin concordance rate is high. The first degree relatives of a proband case has 3% risk of developing SLE. The discovery of multiple chromosome regions conferring risk for SLE development supports the notion that SLE is a polygenic disease. An association of HLA DR2 and DR3 with SLE is a common finding in patients of different ethnicities. It has been estimated