Lupus is a complex autoimmune disease where the body’s immune system mistakenly attacks its own healthy tissues. This can lead to widespread inflammation and tissue damage in various organs. The disease manifests differently in each individual, making its diagnosis and understanding a long and evolving journey. Exploring the historical progression of medical knowledge about lupus reveals how our comprehension of this intricate condition has transformed over centuries.
Early Historical Accounts
The earliest suspected descriptions of conditions resembling lupus can be traced back to ancient times, long before the disease was formally identified. Hippocrates, around 400 BC, documented symptoms consistent with skin ulcers, referring to them as “herpes esthiomenos,” meaning “gnawing skin disease.” These early accounts suggest that skin conditions with ulcerative lesions were observed and noted.
During the Middle Ages and the Renaissance, the term “lupus” was used more broadly to describe various ulcerative skin lesions, particularly on the limbs. In the 13th century, Rogerius Frugard, an Italian surgeon, used the term “lupus” to describe erosive facial lesions that were thought to resemble wolf bites. These historical records, though vague by modern standards, indicate an awareness of severe, persistent skin afflictions that align with some cutaneous manifestations of lupus.
The Naming and Initial Classification
The formal appearance of the term “lupus” in medical literature dates back to around 850 AD. This association with wolves led to the adoption of the Latin word “lupus,” meaning “wolf.” The term “lupus erythematosus,” combining “lupus” with the Greek word “erythema” (meaning redness), was first used in 1850 by French dermatologist Pierre Cazenave.
In the early 19th century, British dermatologists Robert Willan and Thomas Bateman published atlases of skin diseases, which included the first illustrations of patients with lupus lesions. These works helped in classifying skin disorders and distinguished lupus from other conditions. Austrian physician Ferdinand von Hebra, in 1846, further described the distinctive facial rash of lupus as a “butterfly” pattern and noted its chronic nature, which could remain dormant for years.
Understanding Systemic Manifestations
A significant shift in understanding lupus occurred with the recognition that it was not solely a skin condition but could affect internal organs. In 1872, Austrian dermatologist Moriz Kaposi published “New Contributions to Knowledge of Lupus Erythematosus.” He described the existence of two forms: discoid lupus, which was exclusively cutaneous, and a disseminated form that involved systemic complications such as subcutaneous nodules, joint pain, swollen lymph nodes, fever, weight loss, and anemia.
Kaposi’s work distinguished between discoid and systemic forms, paving the way for the concept of Systemic Lupus Erythematosus (SLE). By 1904, the systemic nature of lupus was established through the work of Sir William Osler and Jadassohn. Osler’s treatises detailed how SLE could affect various internal systems, including the central nervous system, muscles, skeleton, heart, and lungs.
Further pathological studies over the next three decades documented additional systemic manifestations, such as heart and kidney lesions. These observations led to the concept of “collagen vascular disease” proposed by Paul Klemperer and colleagues in 1941, which helped classify lupus as an autoimmune disorder.
Modern Insights and Diagnostic Advancements
The modern era of lupus research began in 1948 with Malcolm Hargraves’ discovery of the lupus erythematosus (LE) cell. This cell became an important diagnostic marker, although its detection required a bone marrow biopsy. This discovery led to a deeper understanding of the systemic inflammatory aspects of the disease.
Following the LE cell discovery, Peter Miescher identified antinuclear antibodies (ANAs) in 1954, and in 1957, Seligman recognized DNA as a primary target of these ANAs. The identification of ANAs was a significant breakthrough, as it revealed that lupus is largely driven by autoantibodies—antibodies that mistakenly attack the body’s own components. This led to the development of immunofluorescent ANA testing, a much simpler and more accessible diagnostic tool than bone marrow biopsies, which revolutionized lupus diagnosis and allowed for the identification of milder forms.
The introduction of corticosteroids in the 1950s improved treatment outcomes for SLE patients. Over time, the American College of Rheumatology (ACR) established classification criteria for SLE, with revisions in 1982 and 1997. The Systemic Lupus International Collaborating Clinics (SLICC) further updated these criteria in 2012, and the EULAR/ACR 2019 criteria now serve as a standard for clinical trials, emphasizing precise diagnosis and differentiation from other conditions.