Systemic lupus erythematosus historical perspective

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

[Disease name] was first discovered by [scientist] in [year] during/following [event].

OR

[Disease name] was first described by [scientist] in [year].

Additional Sentence 1: In [year], the first [event] occurred/was first reported following/during [event].

Additional Sentence 2: In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Additional Sentence 3: There have been several outbreaks of [disease] which are summarized below.

Additional Sentence 4: [Disease name] was first described in [year] by [scientist].

Additional Sentence 5: [Risk factor or cause] was first discovered to be associated with [disease] in [year].

Additional Sentence 6: In [year], [scientist] was the first to discover the association between [risk factor] and development of [disease].

Additional Sentence 7: In [year], [gene] mutations were first identified in the pathogenesis of [disease].

The word lupus 

• The history of lupus erythematosus can be divided into three periods: the classical, neoclassical, and modern.
• The word lupus means wolf in Latin, as the destructive injuries the disease caused, brought to mind the bites of this animal. The ancient people used to believe that lupus patients could turn into wolfs especially during the exposure to sunlight. Probably this is as a result of lupus photo-sensitivity.
• Hippocrates firstly used the word "herpes esthiomenos" which was a definition for lupus lesions and thus Hippocrates is considered the first to have described cutaneous ulceration.
• Rogerius Frugardi (1230 ad) used the term lupus to describe erosive facial lesions for the first time scientifically.
• Then Giovanni Manardi (1530 ad) used the same pattern of ulceration to describe the lower extremity lesions and called it lupus as well.
• A British dermatologist, Robert Willan (1757-1808), described destructive lesions of the face and nose under the heading of lupus. Lupus willani which is the cutaneous tuberculosis or lupus vulgaris is named after him.
• Ferdinand von Hebra described an aggressive skin lesion with tissue destructive characteristics in 1845. Later in 1866, Ferdinand von Hebra used the "butterfly" to describe what is known as malar rash. He named the condition initially as seborrhea congestiva.
• Laurent Theodore Biett was the first one to describe lupus erythematosus, although he called it erythema centrifugum. Later his student, Pierre Louis Alphee Cazenave, published Biett's work in 1833.
• Cazenave described discoid lupus completely and called it "lupus erythematosus" disease, as a rare condition in 1851.
• In 1872, Kaposi was the first to describe the systemic signs of the disorder including arthritis, fever, anemia, lymphadenopathy, and weight loss.
• Kaposi and Cazenave were the first ones that clearly distinguished lupus erythematosus from lupus vulgaris or cutaneous tuberculosis, although both disease coexistance in some patients had led to a lot of confusion that time.
• Sir William Osler was the first to coin the term systemic lupus erythematosus. He discussed visceral complications of “erythema exsudativum multiforme” include cardiac, pulmonary, and renal problems as well as cutaneous lesions.
• Jonathan Hutchinson^{[[null 11],[null 12]]} was the first to describe the photosensitive nature of the malar rash
• Sequira and Balean were the first to describe acroasphyxia, or Raynaud phenomenon, and lupus nephritis in 1902.^{null 6}
• In 1908, Alfred Kraus and Carl Bohac were the firsts to describe pulmonary involvement in lupus.
• In 1923, Emanuel Libman and Benjamin Sacks were the firsts to describe noninfectious endocarditis due to lupus.
• Initially, the presence of cutaneous involvement was considered mandatory to the diagnosis of lupus erythematosus. However, George Belote and H.S. Ratner confirmed that the endocarditis of Libman-Sacks was a manifestation of the disease even without cutaneous involvement.
• Paul Klemperer, George Baehr, and A.D. Pollack^{null 15} described wire loop nephritis in 1935.
• Malcolm Hargraves^{null 16} discovered the LE cell. Serum from patients with lupus erythematosus was added to bone marrow preparations from normal subjects. When compared with control preparations, this induced the formation of clumps of polymorphonuclear leukocytes around amorphous masses of nuclear material.
• In 1954, Miescher and Fauconnet observed that absorption of lupus serum with nuclei prevented its ability to induce the LE cell phenomenon, suggesting that a globulin in the serum was reacting with, or destroying, the nuclei.
• This was followed by the demonstration by George Friou^{null 17} in 1958, that the substance in the serum of patients with lupus erythematosus that reacted to the nuclei of cells was gamma globulin, and the target in the nucleus was DNA complexed to histones. He called it the antinuclear factor and further described the indirect immunoflourescence test to detect antinuclear antibodies. These observations in the late 1950s clearly demonstrated an autoimmune pathologic process underlying lupus erythematosus and paved the way for a new area of research. Autoantibodies like nuclear ribonucleoprotein (nRNP), Sm, Ro, La, and anticardiolipin antibodies are useful in describing clinical subsets and understanding the pathogenesis of lupus and other autoimmune diseases.
• While trying to develop a serologic test for syphilis, Wasserman^{null 18} in 1906 first described a complement-fixing antibody that reacted with extracts from bovine hearts. In 1941, the relevant antigen was identified as cardiolipin,^{null 19} which became the basis for the Venereal Disease Research Laboratory (VDRL) test for syphilis. Screening for syphilis lead to the observation that many patients with systemic lupus erythematosus had a positive VDRL test, without any other clinical or serologic evidence of syphilis.^{null 20} Moore and Lutz^{null 21} reported the results of an investigation into the phenomenon of biologic false positivity in 1955. Lupus erythematosus developed in 7% of 148 subjects with false positive tests for chronic syphilis and a further 30% had symptoms consistent with collagen disease.

Leonardt, Arnett, and Schulman^{null 22} at Johns Hopkins University described the familial aggregation of lupus and concordance in monozygotic twins. The discovery of a lethal kidney disease in the New Zealand Brown White hybrid mouse in 1959 at Otago Medical School in New Zealand was another important breakthrough.^{null 23} This murine model has provided many insights into the mechanisms of immunologic tolerance, imunopathogenesis of autoantibody formation, development of glomerulonephritis, and the evaluation of newer therapeutic agents in lupus erythematosus. Drug-induced lupus erythematosus was first described in 1954 at the Cleveland Clinic with the antihypertensive, hydralazine. The first classification criteria for systemic lupus erythematosus were established in 1971 and required 4 of 14 criteria. In 1982, the criteria were revised by the American College of Rheumatology because of advances in serologic testing (ANA and anti-dsDNA) and improved biostatistical techniques.

1. 6348430
2. 7020464
3. PMCID: PMC3437177
4. PMCID: PMC2783313
5. Smith CD, Cyr M. The history of lupus erythematosus, from Hippocrates to Osler. Rheum Dis Clin North Am 1988;14:1-14.
6. Cazenave PLA, Schedel HE. Manual of the Diseases of the Skin. Burgess (trans). London, Henry Renshaw, 1852.
7. Hebra F. On Diseases of the Skin including the Exanthemata, Vol 1. Adams F (trans). London, The New Sydenham Society 1866.
8. Kaposi M. Lupus vulgaris. In: Hebra, F Kaposi M (eds). On Diseases of the Skin including the Exanthemata, Vol IV. Tay W (trans). London, The New Sydenham Society, 1875.
9. Osler W. On the visceral complications of erythema exudativum multiforme. Am J Med Sci 1895;110:629-646.
10. Hutchinson J. Lupus erythematosus. Med Times Gaz 1879;1:1-3.
11. Hutchinson J. On lupus and its treatment. Br Med J 1880;1:650-652.
12. Jadassohn J. Lupus erythematodes. In: Mracek F, editor. Handbuch der Hautkrankheiten. Wein, Alfred Holder, 1904, pp 298-404l.
13. Libman E, Sacks B. A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 1924;33:701.
14. Klemperer P, Pollack A, Baehr G. Diffuse collagen disease: acute disseminated lupus erythematosus and diffuse scleroderma. JAMA 1984;251:1593-1594.
15. Hargraves MM. Discovery of the LE cell and its morphology. Mayo Clin Proc 1969;44:579-599.
16. Friou GJ. Antinuclear Antibodies: diagnostic significance and methods. Arthritis Rheum 1967;10:151-159.
17. Wasserman A, Neisser A, Bruck C. Eine serodiagnosticsche reaktion bei syphilis. Dtsch Med Wochenschr 1906;32:745-746.
18. Pangborn MC. A new serologically active phospholipid from beef heart. Proc Soc Exp Biol Med 1941;48:484-486.
19. Haserick JR, Long R. Systemic lupus erythematosus preceded by false-positive serologic test for syphilis: presentation of five cases. Ann Intern Med 1952;37:559-565.
20. Moore JE, Lutz WB. The natural history of systemic lupus erythematosus: an approach to its study through chronic biological false positive reactions. J Chron Dis 1955;2:297-316.
21. Arnett FC, Shulman LE. Studies in familial systemic lupus erythematosus. Medicine (Baltimore) 1976;55:313-322.
22. Bielschowsky M, Helyer BJ, Howie JB. Spontaneous hemolytic anemia in mice of the NZB/BL strain. Proc Univ Otago Med School 1959;37:9.

Although the term “lupus” was not confined to a single disorder at that time [3], the peculiarity of the skin lesion was fully recognized, resulting in a variety of descriptive terms (e.g., “butterfly rash” [4]) which are in use until today. It was, however, not until Moritz Kaposi succeeded Hebra to the Chair of the Department of Dermatology at the Vienna University Hospital that the systemic nature of lupus was recognized: Kaposi reported in 1872 that certain patients suffering from lupus presented with a syndrome consisting of fever. arthritis, lymphadenopathy, and anemia [5]. It was this report which led to a clear separation of systemic or disseminated lupus erythemntosus (SLE) from the forms definitely limited to the skin. Publications by Jadassohn from Vienna [6] and by William Osler from Baltimore, Maryland, who discussed visceral complications of “erythema exsudativum multiforme” [7] — which rather would be recognized as SLE today [8] — gave additional evidence for the disseminated nature of the disease, while reports on idiopathic thrombotic thrombocytopenic purpura [9], endocarditis [10], and changes in renal histology [11], culminating in the classic study by Klemperer, Pollack, and Baehr on the pathomorphology of SLE in different organs [12], illustrated its puzzling variety of clinical manifestations.

1. 1. Hebra F (1845) Versuch einer auf pathologischer Anatomie gegründeten Einteilung der Hautkrankheiten. K.k. Gesellschaft der Ärzte in Wien 1: 43–52
2. 2. Cazanave PL, Chausit M (1851) Annales des maladies de la peau et de la syphilis, conference du 4 Juin 1851, Paris. 3: 297–299
3. 3. Holubar K (1980) Terminology and iconography of lupus erythematosus. Am J Dermatopathol. 2: 239–242PubMedCrossRef
4. 4. Hebra F (1846) Bericht über die Leistungen in der Dermatologie. In: Constatt, Eisenmann (eds) Jahresbericht über die Fortschritte der gesamten Medizin, 1845. Enke, Erlangen
5. 5. Kaposi M (1872) Neue Beiträge zur Kenntnis des Lupus erythematosus. Arch Dermatol and Syphilol 4: 36–78CrossRef
6. 6. Jadassohn J (1940) Handbuch der Hautkrankheiten. Mraeck, Berlin
7. 7. Osler W (1900) The visceral lesions of the erythema group of diseases. Br J Dermatol 12: 227–245
8. 8. Talbott J (1974) Historical background of discoid and systemic lupus erythematosus. In: Duboid EL (ed) Lupus erythematosus, 2nd ed. University of Southern California Press, Los Angeles
9. 9. Gitlow S, Goldmark C (1939) Generalized capillary and arteriolar thrombosis: report of two cases with a discussion of the literature. Ann Intern Med 13: 1046–1067
10. 10. Libman E, Sacks B (1924) A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 33: 701–737
11. 11. Keith N, Rowntree L (1922) Study of renal complications of disseminated SLE: a report of 4 cases. Trans Assoc Am Physicians 37: 487–502
12. 12. Klemperer P, Pollack A, Baehr G (1941) Pathology of disseminated lupus erythematosus. Arch Pathol Lab Med 32: 569–631

Historical Perspective

Etymology

There are several explanations ventured for the term lupus erythematosus. Lupus is Latin for wolf, and 'erythro' is derived from ερυθρόςTemplate:Polytonic, Greek for "red." All explanations originate with the reddish, butterfly-shaped malar rash that the disease classically exhibits across the nose and cheeks.

1. In various accounts, some doctors thought the rash resembled the pattern of fur on a wolf's face.
2. In other accounts doctors thought that the rash, which was often more severe in earlier centuries, created lesions that resembled wolf bites or scratches.
3. Stranger still is the account that the term "Lupus" didn't come from Latin at all, but from the term for a French style of mask which women reportedly wore to conceal the rash on their faces. The mask is called a "loup", French for "Wolf"
4. Another common explanation for the term is that the disease's course involves repeated attacks like those of a voracious predator, leaving behind the red blotches.

History

Medical historians have theorized people with porphyrias (a disease that shares many symptoms with Lupus) 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 disorders known as dual, homozygous or compound heterozygous porphyrias.

The history of lupus erythematosus can be divided into three periods: the classical, neoclassical, and modern. The classical period began when the disease was first recognized in the Middle Ages and saw the description of the dermatological manifestation of the disorder. The term lupus is attributed to the 12th century physician Rogerius, who used it to describe the classic malar rash. The neoclassical period was heralded by Móric Kaposi's recognition in 1872 of the systemic manifestations of the disease. The modern period began in 1948 with the discovery of the LE cell (the Lupus Erythematosus cell, a misnomer as it occurs with other diseases as well) and is characterised by advances in our knowledge of the pathophysiology and clinical-laboratory features of the disease, as well as advances in treatment.

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 to patients until the middle of the twentieth century, when Hench discovered the efficacy of corticosteroids in the treatment of SLE.