Four main types of lupus exist: systemic lupus erythematosus, discoid lupus erythematosus, drug-induced lupus erythematosus and neonatal lupus erythematosus. Of these, systemic lupus erythematosus (SLE) is the most common and serious form of lupus. The history of SLE can be divided into three periods: classical, neoclassical, and modern.

The classical period began when the disease was first recognized in the Middle Ages and when a description of the dermatological manifestation of the disorder was recorded.  The term lupus (Latin for wolf) is attributed to 12th-century physician Rogerius, who used it to describe erosive facial lesions that were reminiscent of a wolf’s bite, and the classic malar rash, that resembled a wolf’s scratch. The first published illustrations of lupus erythematosus were included in von Hebra’s text, Atlas of Skin Diseases, published in 1856.

The neoclassical period was heralded by Moric Kaposi’s recognition in 1872 of the systemic manifestations of the disease. Kaposi wrote, “… experience has shown that lupus erythematosus (“erythematosus” is Latin for red) … may be attended by altogether more severe pathological changes … and even dangerous constitutional symptoms may be intimately associated with the process in question, and that death may result from conditions which must be considered to arise from the local malady.“ Kaposi proposed that there were two types of lupus erythematosus; the discoid form and a disseminated form. Furthermore, he enumerated various symptoms and signs which characterized the disseminated form including (1) subcutaneous nodules, (2) arthritis with synovial hypertrophy of both small and large joints, (3) lymphadenopathy, (4) fever, (5) weight loss, (6) anemia, and (7) central nervous system involvement. The existence of a systemic form of lupus was firmly established by the work of Osler in Baltimore and Jadassohn in Vienna in 1904.

The modern period began in 1948 with the discovery of the LE cell by Hargraves and colleagues. The investigators observed these cells in the bone marrow of patients with acute disseminated lupus erythematosus and postulated that the cell “… is the result of … phagocytosis of free nuclear material with a resulting round vacuole containing this partially digested and lysed nuclear material …“ This discovery ushered in the present era of the application of immunology to the study of lupus erythematosus. 

Two other immunologic markers were recognized in the 1950s as being associated with lupus: the biologic false-positive test for syphilis12 and the immunofluorescent test for antinuclear antibodies. Medical historians have theorized that people with porphyria (a disease that shares many symptoms with SLE) generated folklore stories of vampires and werewolves, due to the photosensitivity, scarring, hair growth, and porphyrin brownish-red stained teeth in severe recessive forms of porphyria (or combinations of the disorder, known as dual, homozygous, or compound heterozygous porphyrias). 

Useful medication for the disease was first found in 1894, when quinine was first reported as an effective therapy. Four years later, the use of salicylates in conjunction with quinine was noted to be of still greater benefit. This was the best available treatment until the middle of the twentieth century, when the treatment of systemic lupus was revolutionized by the discovery of the efficacy of adrenocorticotrophic hormone and cortisone by Hench. Corticosteroids have been the primary therapy for almost all patients with systemic lupus. Antimalarials have been used principally for patients with skin and joint involvement on the one hand and cytotoxic/immunosuppressive drugs have been used for patients with glomerulonephritis, systemic vasculitis, and other severe life-threatening manifestations on the other.

Two other major advances in the modern era have been the development of animal models of lupus and the recognition of the role of genetic predisposition to the development of lupus. The familial occurrence of systemic lupus was first noted by Leonhardt in 1954 and later studies by Arnett and Shulman at Johns Hopkins. Subsequently, familial aggregation of lupus, the concordance of lupus in monozygotic twin pairs, and the association of genetic markers with lupus have been described.


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