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 means wolf in Latin, as the destructive injuries the disease caused brought to mind the bites of this animal.[[null 1],[null 2]] The earliest usage of the term lupus in the English literature is in the 10th century biography of St. Martin, written in 963 AD. However, Hippocrates is generally considered the first to have described cutaneous ulceration; calling it herpes esthiomenos, which means, gnawing dermatosis.null 3 Several authorities have suggested that lupus was included under this name. Rogerius Frugardi (1230 ad) used the term lupus to describe erosive facial lesions and Giovanni Manardi (1530 ad) used the same to denote boils and ulceration of the lower extremity.[[null 1],[null 3]] In the superstitious Middle Ages, the grotesque appearance of some lupus sufferers brought the myth of werewolves to the minds of people. These were feared to be human beings who had strange powers to transform themselves into animals.null 1 It is obvious that fearful fantasy borders meaningful linguistics in such imagery. Local language use, beliefs, and related pathologic conditions that may have coexisted during the specific time have to be considered carefully in the study of the history of any disease. Rudolph Virchow, after an extensive review of the works of the Medieval and Renaissance periods, concluded that any process involving ulceration or necrosis of the lower limbs or face was loosely labeled lupus before the mid-19th century.null 4

Robert Willan (1757-1808), a British dermatologist, described destructive lesions of the face and nose under the heading of lupus. Cutaneous tuberculosis or lupus vulgaris was included under this classification and was named lupus willani after him. Thomas Bateman,null 5 who was Willan's student, completed his work after his untimely death. The first clear description of lupus erythematosus is credited to Laurent Theodore Biett of the Paris School of Dermatology, who called it erythema centrifugum.null 6 His student, Pierre Louis Alphee Cazenave, published Biett's work and coined the term lupus erythematosus in 1833. Cazenave classically described lupus as a rare condition, which appears most frequently in young females who are otherwise healthy, attacking the face chiefly. Round red patches, slightly elevated, about the size of a shilling, gradually increase in size and sometimes spread over the greater part of the face. The edges of the patches are prominent, and the center, which retains its natural color, is depressed. There is heat and redness but no pain or itching. It is essentially a chronic affection though its appearance would indicate otherwise.null 7 It is obvious that the disease being described by Cazenavenull 7 is discoid lupus. In 1866, Ferdinand von Hebranull 8 used the metaphor of a butterfly to describe the classic malar rash. He had initially named the condition, seborrhea congestiva.

The next stage in our understanding of this disease was largely due to the work of Moriz Kaposi and the Vienna School of Medicine. In 1872, Kaposinull 9first described the systemic signs of the disorder. These included fever, weight loss, lymphadenopathy, anemia, and arthritis. The name discoidal lupus, as pertaining to the exclusively cutaneous form of the disease, is credited to Kaposi. Kaposi and Cazenave clearly distinguished lupus erythematosus from lupus vulgaris or cutaneous tuberculosis, although there was a lot of confusion around that time due to the coexistence of both diseases in patients. In the same five years that Kaposi diagnosed 22 cases of discoid lupus erythematosus (DLE), 279 cases of lupus vulgaris were seen in his department, and, in fact, one of Kaposi's patients with DLE died as a result of pulmonary tuberculosis. Kaposi maintained that DLE had no relation whatsoever to tuberculosis, but this was disputed until the first part of the 20th century, when extensive pathologic studies dispelled this myth.null 6

Sir William Oslernull 10 coined the term systemic lupus erythematosus, which included his own recognition of cardiac, pulmonary, and renal problems in some patients, and observations of the cutaneous manifestations of lupus. Osler observed 29 patients from 1894 to 1903 who presented with erythema as visceral injuries. Only two of these patients clearly had lupus erythematosus. One was a 15-year-old girl with a photosensitive malar rash, joint pain, pleuritis, fever, and nephritis. The other was a 24-year-old woman who also had a malar rash, in addition to fever, chills, lymphadenopathy, and pulmonary involvement.null 10 Jonathan Hutchinson[[null 11],[null 12]] described the photosensitive nature of the malar rash, and Sequira and Balean described acroasphyxia, or Raynaud phenomenon, and lupus nephritis in 1902.null 6Jadassohn'snull 13 exhaustive review of discoid and systemic lupus in 1904 contributed greatly to our understanding of this disease. The descriptions of pulmonary involvement in lupus by Alfred Kraus and Carl Bohac in 1908, and of noninfectious endocarditis by Emanuel Libman and Benjamin Sacksnull 14 in 1923, are significant contributions to our current understanding of the disease. 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. Pollacknull 15 described wire loop nephritis in 1935.

The modern period of our understanding of this disease began in 1948, when Mayo Clinic hematologist Malcolm Hargravesnull 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 Friounull 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, Wassermannull 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 Lutznull 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 Schulmannull 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.

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  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.

Ferdinand von Hebra first described a typical skin lesion with aggressive and tissue destructive characteristics in 1845 [1], which — due to its appearance — was named by Pierre Louis Cazanave “lupus [latin wolf] erythematosus” in 1851 [2]. 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

The word lupus was first used to describe skin lesions after the fall of the Roman Empire in medieval times. Why the image of the wolf was evoked to describe certain skin lesions is not definitively known, but the lesions having the appearance of having been caused by the gnawing of a wolf as well as the destruction of adjacent skin have been suggested (9). One of the first written references is from 916 A.D. in the description of the healing of Eraclius, Bishop of Liege, at the shrine of St. Martin in Tours (see 8). Later Virchow (1821–1902) would conclude that the meaning of lupus to medieval writers was not clear.

At the beginning of the 19th century, Robert Wilan (1757–1812) of London and his student William Bateman (1778–1821) published his Manual of Skin Diseases, which provided an orderly classification of skin diseases based on appearance. The term lupus was reserved for diseases with destructive or ulcerative characteristics that appeared on the face (20 and see 8). This system thus included what would later be called lupus vulgaris and lupus erythematosus. Distinguishing these entities awaited Pierre Cazenave (1795–1877) and Henri Schedel (1804–1856), in Paris at the Saint Louis Hospital for skin diseases (7), who reported their own work as well as that of Laurent Biett (1781–1840), their mentor and a student of Bateman (8). In several articles and especially in the textbook Abrégé Pratique Des Maladies De La Peau, Cazenave and Schedel divided lupus into three types based on the type of visible destruction (7,8). In 1833, Cazenave reported Biett’s work in what is now regarded as the first description of what would become known as lupus erythematosus (7,21).

Ferdinand von Hebra (1816–1880) was in charge of a skin disease clinic at the General Hospital of Vienna beginning in 1841. By 1846 he had described 2 lesions in lupus, 1 consisting of round discs and another of confluent smaller lesions (22, also see 8 and 23). He also invoked the phrase ‘butterfly rash’ to describe lupus (24). Meanwhile, Cazenave and Schedel continued to publish editions of their textbook, each subsequent version with an expanded description of lupus. In the fourth edition of 1847, the term lupus erythemateux was first used (7,21); thus, dividing lupus into lupus erythemateux, tuberculous lupus, ulcerating lupus and lupus with hypertrophy (7,8,21). From this point on tubercular skin disease of the face (lupus vulgaris) and lupus erythematosus were clearly distinguished from one another (8,25). In 1856, Hebra published the first illustrations and used the Latinized version, namely, lupus erythematosus, in Altas of Skin Diseases (8,23,22). Jonathon Hutchinson (1828–1913), perhaps more noted for his contributions to syphilogy, first used the simile of a ‘bat wing’ to describe lupus erythematosus and also first noted photosensitivity (26 and quoted in 8).

Discovery

Landmark Events in the Development of Treatment Strategies

Impact on Cultural History

Famous Cases

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.

References

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