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CHAPTER I
INTRODUCTION

1.1. Background
The term lupus (Latin for wolf ) was first used during the Middle Ages to
describe erosive skin lesions evocative of a wolf ’s bite. In 1846 the Viennese
physician Ferdinand von Hebra (1816–1880) introduced the butterfly
metaphor to describe the malar rash. He also used the term lupus
erythematosus and published the first illustrations in his Atlas of Skin
Diseases in 1856. Lupus was first recognised as a systemic disease with
visceral manifestations by Moriz Kaposi (1837–1902). The systemic form was
further established by Osler in Baltimore and Jadassohn in Vienna. Other
important milestones include the description of the false positive test for
syphilis in SLE by Reinhart and Hauck from Germany (1909); the description
of the endocarditis lesions in SLE by Libman and Sacks in New York (1923);
the description of the glomerular changes by Baehr (1935), and the use of the
term diffuse connective tissue disease by Klemperer, Pollack and Baehr
(1941). The beginning of the modern era in SLE was the discovery of the ‘LE’
cell by Hargraves, Richmond and Morton at the Mayo Clinic in 1948.
Systemic lupus erythematosus (SLE) is the prototypic multisystem
autoimmune disorder with a broad spectrum of clinical presentations
encompassing almost all organs and tissues.1
Systemic lupus erythematosus occurs worldwide. In the United States,
systemic lupus erythematosus is reported to be more common in women,
particularly black women, than in white men.14,16 One U.S. retrospective
study16 of medical records found that the disease is diagnosed 23 times more
often in black women than in white men. The prevalence of this disease is also
higher in Hispanic and Asian Americans.16 In addition, a familial
predisposition to systemic lupus erythematosus has been identified.2 A
prevalence study in India carried out in a rural population near Delhi found a
point prevalence of 3 per 100,000. This is a much lower figure than reported
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from the west varying from 12.5 per100,000 adults in England to 39 per
100,000 in Finland and 124 per 100,000 in USA. However, a fair number of
cases of SLE are encountered in any large hospital in India. Copcord Bhigwan
study found a crude incidence rate of 1 per 25,000 person years.3