Lupus: The Essential Clinician's Guide (Oxford American Rheumatology Library), 2nd Ed

Chapter 1. The History of Lupus

Lupus (meaning “wolf” in Latin) was a Roman family name, and there was a St. Lupus who lived in central France in about A.D. 600.¹ How this name became connected with a disease is not known. Nevertheless, anecdotal case reports of what was probably the medical condition now called lupus appeared sporadically in writings from the tenth century on. Forms of cutaneous tuberculosis were also termed lupus in the early 1800s, and to this day, lupus vulgaris represents that condition. Several dermatological treatises in the 1830s and 1840s, particularly those by Pierre de Cazenave, provided illustrations and case presentations of what was certainly what is now known as lupus (Fig. 1.1).² Moritz Kaposi (for whom Kaposi’s sarcoma is named) first used the description “discoid” to describe cutaneous lupus in the 1860s.

Credit for describing “disseminated,” or systemic, lupus and connecting its rashes with organ involvement goes to Sir William Osler (1849–1919), who initially described our current concepts of the clinical aspects of the disease in three long articles published between 1872 and 1895.³ Between 1900 and 1950, pathological descriptions of cardiac, pulmonary, and central nervous system involvement increased our general knowledge.

In 1941, Paul Klemperer coined the term “collagen vascular disorders” to apply to a group of what are now known to be autoimmune conditions. This led to initiatives to classify this family of disorders. The first criteria for systemic lupus erythematosus (SLE) were published in 1971, followed by revisions in 1981 and 1997, and a new classification in 2012. Other related variants of lupus, such as drug-induced lupus (1945), neonatal lupus (which is not actually lupus), overlap syndromes, mixed connective tissue disease, antiphospholipid syndrome (1983), and incomplete forms of lupus-like inflammation (undifferentiated connective tissue disease), followed. By the late 1970s, a nosological compilation of cutaneous manifestations of lupus was put together by James Gilliam.

Although a few cases of false-positive syphilis serologies were reported as early as 1909 in lupus patients, and hypergammaglobulinemia associated with lupus in 1943, the single major advance in identifying lupus by blood testing was published in 1949. Malcolm Hargraves, a hematologist at the Mayo Clinic, found “globular antibodies taking purple stain” in the marrow aspirate of a child with undiagnosed disease. The discovery of the “LE cell” greatly advanced the field, as it became possible for the first time for a blood test to be used to diagnose lupus, and biopsies were no longer mandatory. The LE cell turned out to be a DNA-histone nucleoprotein. Efforts to improve identification of protein markers in lupus resulted in antinuclear antibody and anti-DNA testing becoming available in the late 1950s. (Advances are summarized in Table 1.1.) Insights into the role of complement in inflammation and advances in immune techniques allowed anti-Sm, anti-RNP, anti-ENA, anti-Ro (SSA), and anti-La (SSB) to be identified and elucidated in the 1960s.

Figure 1.1 The first modern illustration of cutaneous lupus, labeled “lupus erythemateux” (1856). Source: Wallace DJ, Lyon I. Pierre Cazenave and the first detailed modern description of lupus erythemotosus. Semin Arthritis Rheum. 1999;28:305–313. Reprinted with permission, Elsevier, 1999.

Table 1.1 First descriptions of components of lupus

The first lupus clinic in the United States was started by Marian Ropes in Boston in 1922; her treatment armamentarium included aspirin and dermato-logicals. The new availability of nitrogen mustard in 1947 and corticosteroids in 1948 revolutionized the treatment of SLE and raised the 50% five-year survival rate in 1948 to the current rate of 90% or better.

References

1. Benedek TG. Historical background of discoid and systemic lupus erythematosus. In: DJ Wallace and BH Hahn, eds. Dubois’ Lupus Erythematosus. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:2–15.

2. Hargraves MM. Production in vitro of the LE cell phenomenon: use of normal bone marrow elements and blood plasma from patients with acute disseminated lupus erythematosus. Proc Staff Mayo Clin. 1949;24:234–237.

3. Osler W. On the visceral complications of erythema exudativum multiforme. Am J Med Sci. 1895;110:629–646.