Systemic Lupus Erythematosus

Basic, Applied and Clinical Aspects

George C. Tsokos, George Tsokos

• Publisher: Academic Press
• Year: 2015
• eBook ISBN: 9780128020098
• Topic: Medicine
• Subtopic: Diseases & Allergies

Systemic Lupus Erythematosus combines basic science with clinical science to provide a translational treatment of the disease and is a useful reference for specialists in the diagnosis and management of patients with SLE, a tool for measurement of clinical activity for pharmaceutical development and basic research of the disease and a reference work for hospital libraries.- Provides the very latest overview of the pathogenesis of SLE- Distills current understanding of the cellular, molecular, genetic and environmental factors that instigate and drive the disease- Includes comprehensive coverage of clinical features, including fatigue, organ system manifestations, overlap syndromes, infections, and more- Conveys the very latest understanding of mechanisms of tissue damage, including immune complexes, antibodies, and other mechanisms that lead to organ damage- Discusses the latest treatment options on disease modifying or disease controlling agents- Provides 'one stop' coverage of all the latest scientific and clinical developments in SLE

Part I

Epidemiology and Diagnosis

Outline

Chapter 1. History of Systemic Lupus Erythematosus

Chapter 2. The Patient

Chapter 3. Epidemiology

Chapter 4. Measuring Disease Activity

Chapter 5. Disease Development and Outcomes

Chapter 6. Socioeconomic Aspects of Systemic Lupus Erythematosus

Chapter 7. Biomarkers in Systemic Lupus Erythematosus

Chapter 1

History of Systemic Lupus Erythematosus

Christine Konya, and Ziv Paz Division of Rheumatology & Lupus Center, BIDMC, Harvard Medical School, Boston, MA, USA

Abstract

Systemic lupus erythematosus (SLE) is one of the most complex autoimmune systemic diseases. SLE has been recognized as a defined clinical entity for more than 100 years. However, its cause remains unknown and its treatment options are still limited. The many faces of this disease make its history unique and fascinating. The history of SLE is the history of modern medicine. We describe the evolution from basic descriptive physical observations to a more in-depth understanding of disease pathophysiology and its implications for treatment and prognosis.

Keywords

History; Lupus

Declare the past, diagnose the present, foretell the future.

Hippocrates

When we interview and treat patients who were diagnosed with systemic lupus erythematosus (SLE), read their posts in social networks, follow their support groups and forums, we see how their hope brings them together. They find comfort in sharing their stories. The story of each SLE patient is different, but not only because the patients are different. The disease affects every single patient in distinct ways and changes its nature over time. The many faces of this disease make its history so unique and fascinating. Systemic lupus erythematosus is one of the most complex autoimmune systemic diseases. Its cause remains unknown and our treatment options are still limited, despite the fact that SLE has been recognized as a defined clinical entity for more than 100 years.

Hippocrates was thought to be the first to describe the cutaneous manifestations of SLE, calling it herpes esthiomenos (gnawing dermatosis).^1,2 It is not completely clear when the word lupus became synonyms with the disease. However, records indicate that it may have been Hebernus, the archbishop of Tours, France, in 855 AD, who was the first to use the word “lupus” to describe the disease in his writing: “The Miracles of Saint Martin.” He mentions the bishop of Liège who was suffering from a severely consuming disease called lupus in the common tongue, and was miraculously cured after spending time in the shrine of Saint Martin in Tours.³ Later, Paracelsus, a fifteenth century Swiss German renaissance physician who was known for his astute observations, considered SLE to be a skin disease with “greater blood supply” and recommended treatment with bloodletting, which was the treatment of most conditions in those days. He used the term “lupus” to define the cutaneous manifestations of the disease or, by his own words, “a hungry wolf eating flesh.”³

Rudolph Virchow (1821–1902) was the first to review systematically the history of SLE. In his writings, Virchow mentioned a reference from the old German book Margerita Medicine, published in 1502 by the German physician Johann Trollat von Vochenberg. In this reference, von Vochenberg suggested the use of caprifolin to treat the conditions of cancer and wolf, or as quoted (translated from German): “for the wolf and for cancer, caprifolin (honeysuckle).” Rudolf Virchow quoted even an older text dating back to the end of the thirteenth century and referred to Roger de Palma, from the school of Salerno: “Sometimes lupus arises in the thighs and the lower legs (and is) distinguished from cancer from the symptoms mentioned above.”⁴ In the middle ages, the term “lupus” was used to describe ulcerative or necrotizing skin diseases as it reminded the describers of the bite of a wolf. Superstitious people at that time related these skin conditions to the bite of a werewolf given the grotesque appearance of the ulcerative skin disease.^5,6

Robert Willan (1757–1808) and his student Thomas Bateman were British dermatologists who used the term “lupus” to describe a destructive skin disease preferentially affecting the face and the nose.⁷ In the mid-nineteenth century, the French dermatologist Laurent Theodore Biett used the term “erythema centrifugum” to describe these skin lesions.^2,8 The current name of the disease, lupus erythematosus, was given in 1833 by Biett’s student Pierre Louis Cazenave, who called it “lupus érythèmateux or lupus erythematosus.” Cazenave was also the first to describe other aspects of the disease. He appreciated the predilection to young females, the painless nature of the facial lesions, and other features of the disease. His exact words are still relevant today and many times used by rheumatologists to describe examination findings:

“In some circumstances (lupus) manifests itself at first as a violet rubefaction on part of the face, and mainly on the nose, which at the same time is rather swollen: over many months the color rises little by little; the surface becomes animated; a small ulcer forms and on top of it, a scab, which then thickens and covers the ulcer, which becomes progressively deeper. Lastly, the skin may get thinner in imperceptible stages and adopt the appearance of a scar, without there being tubercles or ulcers, and without displaying worse injuries than a livid color and, from time to time, a light and barely perceptible peeling.”⁹

Ferdinand von Hebra used the phrase “butterfly rash” to describe Cazenave’s skin findings of lupus erythematosus.¹⁰ In 1872 at the Vienna School of Medicine, Moritz Kaposi, Hebra’s son-in-law, was the first one who appreciated the systemic features of “lupus erythematosus” including lymphadenopathy, fever, and arthritis. Kaposi was also the one who coined the term “discoid lupus” to describe a specific form of cutaneous involvement. Even though cutaneous tuberculosis was a prevalent condition in these days, Kaposi believed that discoid lupus erythematosus was a separate entity, even though this notion was argued by others. Kaposi also used the term “disseminated lupus erythematosus” to describe widespread cutaneous involvement.¹¹

Sir William Osler (1849–1919) was the first to use the phrase “systemic lupus erythematosus.” Osler diagnosed 29 patients who presented with erythema and visceral injuries as SLE. Many of these cases correspond to our current descriptions of lupus erythematosus, even though there were no official criteria at that time. Osler also recognized that SLE can affect the kidneys, lungs, and the heart.¹² At the beginning of the twentieth century, Jonathan Hutchinson described the photosensitivity of the lupus skin rash.^13,14 In 1923, Emanuel Libman and Benjamin Sacks identified four cases of noninfectious endocarditis, of which three patients displayed cutaneous injuries of SLE. Later, it was confirmed that the endocarditis of Libman-Sacks could occur even without the cutaneous involvement.^15,16

This was the beginning of the modern era of SLE, and the way we clinically diagnose the disease today has not changed much since. However, even though the physical findings of the disease were known at that time, they were not specific. The diagnosis of SLE was still challenging due to a lack of objective diagnostic tests. ...

Table of contents

• Cover image
• Title page
• Table of Contents
• Dedication
• Copyright
• List of Contributors
• Introduction
• Part I. Epidemiology and Diagnosis
• Part II. Pathogenesis
• Part III. Mechanisms of Tissue Damage
• Part IV. Clinical Aspects of the Disease
• Part V. Antiphospholipid Syndrome
• Part VI. Treatment of the Disease
• Appendix
• Index