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What Are Systemic Lupus Erythematosus (SLE) And Other Types Of Lupus?
Lupus is a long-term autoimmune disease in which the immune system attacks healthy tissue. It can cause a wide range of symptoms throughout the body. Medical treatment and home remedies can help manage it.
According to the Lupus Foundation of America, people report around 16,000 new cases of lupus in the United States each year, and up to 1.5 million people may be living with the condition in the U.S.
Lupus refers to a range of conditions that can cause symptoms throughout the body. It is an autoimmune condition, which means it happens when the body's immune system mistakenly attacks healthy body tissue. Symptoms can range from mild to life-threatening.
Systemic lupus erythematosus (SLE) is the most common type, and people often use the term lupus to refer to SLE. However, there are other types, depending on which part of the body lupus mainly affects.
In addition to SLE, there are cutaneous lupus (such as discoid lupus erythematosus [DLE]), drug-induced lupus (DIL), and neonatal lupus.
Systemic lupus erythematosusSLE accounts for 70% of lupus cases. It affects multiple organs and systems throughout the body. For this reason, SLE tends to be a more severe form of lupus. The symptoms can range from mild to severe.
SLE can cause inflammation in the:
This inflammation may happen in one of these areas or affect multiple areas at one time.
Discoid lupus erythematosusIn DLE, a type of cutaneous lupus, symptoms affect only the skin. DLE appears as circular lesions, typically on the scalp and face, although they can appear on other parts of the body, such as inside the ears.
The lesions tend to be red and may become thick and scaly. In some cases, the lesions lead to scarring and skin discoloration. If lesions scar on the scalp, hair may not regrow in that area.
DLE does not affect the internal organs, but around 10% of people with DLE go on to develop SLE, according to the Lupus Foundation of America. However, it is possible that these people already had SLE and their skin symptoms led to a DLE diagnosis first.
Subacute cutaneous lupus erythematosusSubacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body that have exposure to the sun. These lesions do not cause scarring but may cause skin discoloration.
Drug-induced lupusDrug-induced lupus (DIL) results from the long-term use of certain medications. The symptoms tend to be similar to those in SLE but are often less severe.
Over 100 medications have been identified as potential causes of DIL, including:
DIL typically goes away within 6 months of stopping the medication.
Neonatal lupusNeonatal lupus can affect newborns if their birth parent passes on antibodies for lupus or Sjögren's syndrome through the placenta during pregnancy.
If the birth parent has these antibodies — whether or not they have lupus — there is a 2% an infant will develop neonatal lupus.
At birth, they may have:
The skin symptoms usually go away within a few weeks, but some infants may have a congenital heart block, in which the heart cannot regulate a normal and rhythmic pumping action. This is a more serious complication, and the infant may need a pacemaker.
The symptoms of lupus will depend to some extent on the type of lupus.
American College of Rheumatology lists 11 criteria for describing lupus. If a person meets four or more of the criteria, they will consider a diagnosis of lupus.
The 11 criteria are:
The symptoms of lupus occur in times of flare-ups. Between flare-ups, people usually experience times of remission, when there are few or no symptoms.
A person with lupus may also experience:
Signs of lupus in femalesThe symptoms of lupus can vary widely, including between males and females.
Symptoms more commonly seen in females include:
Research suggests that, while males are less likely than females to have lupus, the symptoms tend to be more severe.
Symptoms more commonly seen in males include:
Lupus can also cause the following systems, depending on which organs it affects:
Other complications that can arise from lupus include:
A doctor may recommend delaying pregnancy until lupus has been under control for at least 6 months.
The following images show how some lupus symptoms can appear.
Lupus is an autoimmune condition, but the exact cause is unclear.
What goes wrong?The immune system protects the body from pathogens such as bacteria, but sometimes it mistakenly targets healthy tissue. This can cause inflammation, swelling, pain, and tissue damage.
Why does the immune system go wrong?Researchers do not yet entirely understand what causes lupus, but they believe it is caused by multiple factors.
One possible theory relates to cell death, a natural process that occurs as the body renews its cells. Due to genetic factors, the bodies of people with lupus may not properly clear cells that have died.
The dead cells that remain may lead to the production of autoantibodies, such as antinuclear antibodies (ANAs), that go on to attack the body, causing lupus symptoms.
ANAs are commonly present in people with autoimmune conditions such as lupus. They work by targeting the nucleus of the body's cells. The nucleus contains genetic material.
Risk factors: Hormones, genes, and environmentLupus may develop in response to several factors. These may be:
According to the American College of Rheumatology, 20% of people with lupus develop the condition before the age of 20, but it rarely appears before the age of 5.
In children, lupus may have more severe symptoms. Up to 50% of children with lupus have kidney symptoms.
Treatment for lupus will depend on the type of lupus. Options include:
Home remedies and lifestyle measures may help protect body systems and manage some symptoms.
While research is limited some evidence suggests a varied and balanced diet can help manage lupus. A doctor or dietitian can help a person with lupus make a diet plan that suits their needs.
Options may include:
Other lifestyle changes that may help include:
Diagnosis can be difficult because symptoms of lupus can resemble symptoms of other conditions.
A doctor will ask about symptoms, carry out a physical examination, and take a personal and family medical history. They may also request blood tests and other laboratory investigations.
Biomarkers are antibodies, proteins, genetics, and other factors that can show a doctor what is happening in the body or how the body is responding to treatment. They can help identify whether a person has a condition even when there are no symptoms.
Blood testsBlood tests can show whether certain biomarkers are present, and biomarkers can give information about which autoimmune disease, if any, a person has.
Further tests may include:
Here are some questions people often ask about lupus.
What is the main cause of lupus?Researchers do not currently know what causes lupus. However, they believe that environmental, genetic, immunological, and endocrine factors may play a role.
What are three triggers of lupus?The following three factors may trigger lupus:
Five symptoms that may indicate lupus include:
However, the signs and symptoms of lupus vary widely and will depend on the type of lupus.
What is the life expectancy of a person with lupus?Life expectancy will depend partly on the type of lupus. Statistics suggest that 85–90% of people with SLE will live at least 10 years after their diagnosis, and many will live much longer.
DIL usually resolves within a few weeks after stopping the drug that caused the reaction.
Lupus is an autoimmune condition that can affect a wide range of body systems. There are different types of lupus, but SLE is the most common type.
Some people may experience cycles of flare-ups and remissions, whereas others may have ongoing symptoms. The varied experiences of lupus can make it challenging for doctors to diagnose.
Once a person has a diagnosis, various treatment options can help manage symptoms, limit damage to body organs, and maximize a person's quality of life.
The History Of Lupus
The history of lupus can be divided into three periods: classical, neoclassical, and modern. This article concentrates on developments in the present century which have greatly expanded our knowledge about the pathophysiology, clinical-laboratory features, and treatment of this disorder.
Lupus in the classical period (1230-1856)The history of lupus during the classical period was reviewed by Smith and Cyr in 1988. Of note are the derivation of the term lupus and the clinical descriptions of the cutaneous lesions of lupus vulgaris, lupus profundus, discoid lupus, and the photosensitive nature of the malar or butterfly rash.
The word 'lupus' (Latin for 'wolf') is attributed to the thirteenth century physician Rogerius who used it to describe erosive facial lesions that were reminiscent of a wolf's bite. Classical descriptions of the various dermatologic features of lupus were made by Thomas Bateman, a student of the British dermatologist Robert William, in the early nineteenth century; Cazenave, a student of the French dermatologist Laurent Biett, in the mid-nineteenth century; and Moriz Kaposi (born Moriz Kohn), student and son-in-law of the Austrian dermatologist Ferdinand von Hebra, in the late nineteenth century.
The lesions now referred to as discoid lupus were described in 1833 by Cazenave under the term "erythema centrifugum," while the butterfly distribution of the facial rash was noted by von Hebra in 1846. The first published illustrations of lupus erythematosus were included in von Hebra's text, Atlas of Skin Diseases, published in 1856.
Lupus in the neoclassical period (1872- 1948)The Neoclassical era of the history of lupus began in 1872 when Kaposi first described the systemic nature of the disorder: "...Experience has shown that lupus erythematosus ... May be attended by altogether more severe pathological changes, and even dangerous constitutional symptoms may be intimately associated with the process in question, and that death may result from conditions which must be considered to arise from the local malady."
Kaposi proposed that there were two types of lupus erythematosus; the discoid form and a disseminated (systemic) form.
Furthermore, he enumerated various signs and symptoms which characterized the systemic form, including:
The existence of a systemic form of lupus was firmly established in 1904 by the work of Osler in Baltimore and Jadassohn in Vienna. Over the next thirty years, pathologic studies documented the existence of nonbacterial verrucous endocarditis (Libman-Sacks disease) and wire-loop lesions in individuals with glomerulonephritis; such observations at the autopsy table led to the construct of collagen disease proposed by Kemperer and colleagues in 1941. This terminology, 'collagen vascular disease,' persists in usage more than seventy years after its introduction.
Lupus in the modern era (1948-present)The sentinel event which heralded the modern era was the discovery of the LE cell by Hargraves and colleagues in 1948. The investigators observed these cells in the bone marrow of individuals with acute disseminated lupus erythematosus and postulated that the cell "...Is the result of...Phagocytosis of free nuclear material with a resulting round vacuole containing this partially digested and lysed nuclear material...".
This discovery ushered in the present era of the application of immunology to the study of lupus erythematosus; it also allowed the diagnosis of individuals with much milder forms of the disease. This possibility, coupled with the discovery of cortisone as a treatment, changed the natural history of lupus as it was known prior to that time.
Two other immunologic markers were recognized in the 1950s as being associated with lupus: the biologic false-positive test for syphilis and the immunofluorescent test for antinuclear antibodies. Moore, working in Baltimore, demonstrated that systemic lupus developed in 7 percent of 148 individuals with chronic false-positive tests for syphilis and that a further 30 percent had symptoms consistent with collagen disease.
Friou applied the technique of indirect immunofluorescence to demonstrate the presence of antinuclear antibodies in the blood of individuals with systemic lupus. Subsequently, there was the recognition of antibodies to deoxyribonucleic acid (DNA) and the description of antibodies to extractable nuclear antigens (nuclear ribonucleoprotein [nRNP], Sm, Ro, La), and anticardiolipin antibodies; these autoantibodies are useful in describing clinical subsets and understanding the etiopathogenesis of lupus.
First animal model developedTwo other major advances in the modern era have been the development of animal models of lupus and the recognition of the role of genetic predisposition to the development of lupus. The first animal model of systemic lupus was the F1 hybrid New Zealand Black/New Zealand White mouse.
This murine (mouse) model has provided many insights into the immunopathogenesis of autoantibody formation, mechanisms of immunologic tolerance, the development of glomerulonephritis, the role of sex hormones in modulating the course of disease, and evaluation of treatments including recently developed biologic agents such as anti-CD4, among others.
Other animal models that have been used to study systemic lupus include the BXSB and MRL/lpr mice, and the naturally occurring syndrome of lupus in dogs.
Genetic component recognizedThe familial occurrence of systemic lupus was first noted by Leonhardt in 1954 and later studies by Arnett and Shulman at Johns Hopkins. Subsequently, familial aggregation of lupus, the concordance of lupus in monozygotic twin pairs, and the association of genetic markers with lupus have been described over the past twenty years.
Molecular biology techniques have been applied to the study of human lymphocyte antigen (HLA) Class II genes to determine specific amino acid sequences in these cell surface molecules that are involved in antigen presentation to T-helper cells in individuals with lupus. These studies have resulted in the identification of genetic-serologic subsets of systemic lupus that complement the clinico-serologic subsets noted earlier.
It is hoped by investigators working in this field that these studies will lead to the identification of etiologic factors (e.G., viral antigens/proteins) in lupus.
Over the last decade or so, we have witnessed significant advances in the understanding of the genetic basis of lupus, and of the immunological derangements which lead to the clinical manifestations of the disease.
Advances have been made in the assessment of the impact of the disease in general, and in minority population groups, in particular and efforts are being made towards defining lupus biomarkers which may help both to predict disease outcome and to guide treatments.
Lupus therapies then and nowFinally, no discussion of the history of lupus is complete without a review of the development of therapy. Payne, in 1894, first reported the usefulness of quinine in the treatment of lupus. Four years later, the use of salicylates in conjunction with quinine was also noted to be of benefit.
Cortisone/corticosteroids were introduced for the treatment of lupus in the middle part of the 20th century by Hench. Presently, corticosteroids are the primary therapy for almost all individuals with lupus.
Antimalarials, used in the past principally for lupus skin and joint involvement, are now recognized to prevent the occurrence of flares, the accumulation of damage, and the occurrence of early mortality.
Cytotoxic/immunosuppressive drugs are used for glomerulonephritis, systemic vasculitis, and other severe life-threatening manifestations of lupus. Newer biologic agents are now used, either off-label or after approval by regulatory agencies in the U.S., Europe, and other countries.
Other potential drug products are being investigated as new disease pathways are being discovered.
Looking forwardThe history of lupus, although dating back at least to the Middle Ages, has experienced an explosion in this century, especially during the modern era over the past 60 years. It is hoped that this growth of new knowledge will allow a better understanding of immunopathogenesis of the disease and the development of more effective treatments.
Lupus: Why This Autoimmune Disease Causes Hair Loss
It was in 2014 when singer-actor Selena Gomez was diagnosed with lupus, an autoimmune condition that causes inflammation in different parts of the body. In 2017, the founder and owner of Rare Beauty revealed that she underwent a kidney transplant due to the disease. It took some time for people to connect the dots and understand that lupus is not a single ailment but comprises a wide range of symptoms and manifestations. Just like how the disease impacted Gomez' kidney, it can also affect other parts of the body, such as your hair health.
Speaking with the OnlyMyHealth team, Dr Monica Chahar, Chief Dermatologist and Director-Skin Decor, Dwarka, New Delhi, discussed lupus-related hair problems in detail and shared ways to manage the condition.
Also Read: Recognising the Red Flags: 20 Signs You Have an Autoimmune Disease
What Is Lupus?"Lupus, scientifically termed Systemic Lupus Erythematosus (SLE), is an autoimmune disorder where the immune system mistakenly attacks healthy tissues, resulting in inflammation and damage across various organs and systems," explained Dr Chahar, adding, "While lupus can affect any part of the body, it often targets the skin, joints, kidneys, heart, lungs, and blood cells."
According to the Lupus Foundation of America, at least 50 lakh people worldwide have some form of lupus, and around 10.5 lakh are Americans. Of the population affected by the condition, 9 in 10 people with lupus are women.
Besides gender, genetics is a common risk factor for lupus, meaning a family history of lupus can increase susceptibility to the disease. Moreover, environmental triggers and exposure to certain medications or infections can make people more susceptible to the development of lupus.
How Lupus Affects Hair HealthLupus can affect hair health. One of the most common manifestations is alopecia, a type of hair loss where individuals may experience thinning or loss of hair, often in patches. In some cases, hair loss may be temporary, but it can also become permanent for certain individuals. Most lupus-related hair loss is non-scarring, which is hair loss that occurs without permanent damage to the hair follicles.
Why does lupus lead to hair loss? Dr Chahar shared a few explanations listed below:
Inflammation:
Non-scarring hair loss arises when lupus causes inflammation around the scalp and hair follicles. This type of inflammation impacts not only scalp hair but also eyebrows, beards, and eyelashes.
Discoid sores/lesions:
These types of sores or lesions commonly occur on the scalp and face and usually do not hurt or itch. However, they can form anywhere on the body, potentially causing permanent scarring. When these lesions appear on the scalp, they damage hair follicles, resulting in permanent hair loss.
Medication:
Certain medications used in lupus treatment, like steroids and immunosuppressants, can contribute to hair thinning.
According to a review published in the Journal Clinical, Cosmetic and Investigational Dermatology, hair loss has been observed in up to 85% of SLE patients. The research further notes that non-scarring alopecia has been included as a criterion for the diagnosis of SLE according to the latest Systemic Lupus International Collaborating Clinics (SLICC) classification criteria.
Identifying Lupus Hair LossDr Chahar said, "Hair loss is not universal among lupus patients, but when it occurs, there's an uptick in hair shedding."
While losing up to 100 hairs daily is normal, lupus patients may experience more shedding depending on the illness's severity, she shared, adding, "Hair loss may manifest during washing or brushing, ranging from minor thinning to clumps of hair loss. The degree varies, with some losing between 55% and 100% of their hair."
Also Read: More Than 50% People With Autoimmune Disease Experience Depression And Anxiety: Study
Can Lupus Hair Loss Be Treated?Whether or not your hair will grow back depends on whether you have scarring on your scalp from lupus skin problems, according to the Lupus Foundation of America. If you do have scarring, it may be difficult to treat it. However, getting treatment can protect the hair you still have, the charity shared.
To manage lupus symptoms and potentially reverse hair loss, here are some strategies to follow:
Disease control: Dr Chahar recommended taking corticosteroids and immunosuppressants to aid in symptom management and inflammation control. Additionally, antimalarial drugs may be prescribed to reduce lupus flares, she said. However, it is important to consult your healthcare provider before opting any new treatment.
Avoid triggers: Sun exposure can exacerbate lupus symptoms, including hair loss, which is why you must protect your scalp by wearing hats or using sunscreen.
In addition, follow these do's and don'ts to manage your symptoms:
Do's
Don'ts
Hair loss can be a common occurrence for people suffering from lupus. The autoimmune condition leads to inflammation of the scalp, leading to hair thinning and, subsequently, hair loss. The key is to diagnose the condition early and to receive timely treatment. Remember, the hair loss associated with lupus can be reversed if there is no scarring on your scalp yet. Speak with your doctor to know the best way to approach it.
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