Lupus – Types, Causes, and Risk factors

Overview  

Lupus is one of the most intricate autoimmune diseases. It is a chronic autoimmune disease that brings about a variety of clinical manifestations due to its impact on multiple organ systems. The condition triggers pain in many parts of the body.

An autoimmune disease occurs when a person’s immune system attacks their own healthy tissues, causing inflammation and damaging body parts like the skin, joints, lungs, blood vessels and kidneys. Joint pain, rashes and tiredness are the usual symptoms of lupus. 

Systemic lupus erythematosus (SLE) is considered the most common type of lupus. The chronic inflammation associated with lupus can occur in various tissues and organs, making diagnosis difficult. 

People from any age group can develop this autoimmune disease, but the majority of lupus patients are women aged between 15 and 44. 

Lupus affects each individual differently, with symptoms being difficult to detect and varying from patient to patient. It’s even been regarded as “the disease with a thousand faces”. 

There is no cure for lupus at the moment, but treatment can help control the autoimmune response and reduce symptoms.

Different types of lupus  

Systemic lupus erythematosus 

According to an NCBI book titled ‘Systemic Lupus Erythematosus’, Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease that causes significant morbidity and mortality.

SLE makes up roughly 70% of all lupus cases and can range from mild to severe. While there is currently no cure, there are many things one can do to help manage lupus and possibly improve the quality of life. 

It involves the support of the medical team for timely treatment and care.

Nearly half the people with this type of lupus are prone to have injuries in any major organs like the brain, heart or kidneys.

Some of the more severe complications are as follows:

• Kidney inflammation can impair the body’s ability to filter waste from the blood. This increases the need for dialysis or a kidney transplant and resulting in a disease known as lupus nephritis.
• Inflammation of the nervous system and brain leads to brain fog, headaches and strokes.
• Hardening of the arteries or the accumulation of deposits on artery walls can lead to a heart attack

Cutaneous lupus erythematosus

Cutaneous lupus erythematosus (CLE) only affects the skin. Skin lupus accounts for approximately 10% of all lupus cases.

Acute cutaneous lupus erythematosus

Acute cutaneous lupus erythematosus (ACLE) is a common type of cutaneous lupus associated with systemic lupus erythematosus. 

The rash caused by acute cutaneous lupus has a distinct butterfly pattern that spreads across the nose and cheeks. 

This rash is red, flat and itchy, usually appearing after sun exposure. A rash may also appear on other body parts, such as the arms and legs.

Discoid lupus erythematosus 

Discoid lupus erythematosus (DLE) is one of the most prevalent types of chronic cutaneous lupus. It is an autoimmune skin condition among the lupus erythematosus spectrum of illnesses. 

Discoid lupus erythematosus is a chronic skin condition characterised by sores, inflammation and scarring that primarily affects the face, ears and scalp. It can also affect other body parts. 

It manifests as red, inflamed, painful, coin-shaped patches of skin with a scaly appearance. It is usually noticed on the scalp, cheeks and ears. Hair loss may occur if the lesions are on the scalp. 

The lesions may then develop into more profound scarring, and the centre areas may appear lighter in colour with a darker exterior than normal skin. These lesions can be prominent for years if not treated. 

This type of lupus accounts for 50-85% of CLE cases. Women are 2-3 times more prone to DLE than men. Although DLE can occur at any age, it is most prevalent in people between the ages of 20 and 40.

Subacute cutaneous lupus erythematosus 

Subacute cutaneous lupus is most often a symptom of systemic lupus, but it can also occur on its own. Lesions are rarely itchy or painful, and they occasionally cause scarring after they heal. 

Subacute cutaneous lupus brings on two types of lesions—papulosquamous and annular.

The first type—papulosquamous lesion, appears as red, scaly patches that resemble pimples but can quickly advance to cover large areas of skin. Sun exposure triggers lesions, which typically appear on the back, shoulders and chest.

Annular lesions are the second type of subacute cutaneous lupus lesions. These are flat pink circles with a red rim. These lesions vary in size and can appear anywhere on the body. Their appearance on the face is rare.

Drug-induced lupus 

As the name implies, medication reaction causes drug-induced lupus. Certain drugs that treat irregular heartbeat, high blood pressure and tuberculosis have been linked to drug-induced lupus. 

The symptoms resemble those of SLE. However, unlike SLE, drug-induced lupus does not cause organ or blood vessel damage. In most cases, the symptoms are mild and will go away if the patient stops using the medication that caused lupus.

This type makes up approximately 10% of all lupus cases. Excessive amounts of certain medications are the root cause of the disease. Symptoms of the disease usually disappear after 6 months of not taking the medication that caused them.

While SLE and cutaneous lupus are much more common in women, men are more likely to develop drug-induced lupus. This is because the medications most predominantly associated with drug-induced lupus are more frequently prescribed to men.

Neonatal lupus 

Neonatal lupus is a rare condition affecting infants born to women with lupus. The antibodies that the mother produces have an impact on the foetus in the womb. The baby may have a skin rash, liver issues or low blood cell counts when the child is born. 

These symptoms usually set out after 6 months and have no long-term consequences. Newborns of lupus mothers are more likely to have a very rare but severe heart defect.

Doctors can now identify the most at-risk mothers through proper testing, and the infant can be treated at or prior to birth.

Babies born with neonatal lupus are unlikely to develop chronic lupus. Though uncommon, neonatal lupus can cause heart and blood vessel problems. Severe complications are not known to occur.

Causes of Lupus  

Lupus develops through antibody attacks occurring in the normal tissue. This transpires into long-term inflammation and brings out tissue damage. The immune system holds these antibodies. The cause of the problem-causing antibody formation is still a mystery. 

A combination of internal and external factors like hormones, environment and genetics is thought to give rise to lupus.

Hormonal changes  

The body’s messengers—hormones regulate most functions of the body. Since most lupus cases occur in females, there is speculation about the link between lupus and estrogen.

Though women and men have estrogen, the hormone production is greater in women. Symptoms of lupus surface before menstruation or during gestation since estrogen production is at its peak during this phase.

This implies that oestrogen manages the severity of lupus in some way. However, no link has been established between oestrogen or any other hormone and lupus. 

Furthermore, studies of women with lupus who took oestrogen in the form of birth control pills or as postmenopausal therapy prove no notable increase in disease activity. 

Environmental factors  

Researchers believe that a random encounter with an environmental agent, such as a virus or a chemical, causes the disease. They have not yet identified a specific environmental agent, but the hypothesis remains viable.

Though the environmental factors that can trigger lupus and cause flares are not fully understood, the common invoking agents are ultraviolet light (UVA and UVB), infections Like the Epstein-Barr virus effects) and exposure to silica dust in the agricultural sector or industrial settings.

Family history  

The identification of 50 plus genes related to lupus is a new break in the research.

While most of these genes haven’t been shown to directly cause lupus, they may be a contributing factor. 

In the majority of cases, genes alone are insufficient. This is generally evident in twins raised in the same environment and possessing the same inherited features, but only one develops lupus. 

However, when one of two identical twins develops lupus, the other twin is more likely to develop the disease. It is shown that there is a 30 per cent chance for identical twins and a 5-10 per cent chance for fraternal twins to acquire the disease if one twin is affected.

Lupus can occur in people with no family history of the disease, but some family members are likely to have other autoimmune diseases.

Certain ethnic groups, including people of Asian, African, Native American, Native Hawaiian, or Pacific Island descent, are at a higher risk of developing lupus, which may be related to the common genes they share.

Symptoms of Lupus 

Fatigue 

Being a common complaint among lupus patients, fatigue accounts for about 67 – 90 % of SLE patients, as shown by an NCBI article. Fatigue is a troublesome sign, affecting the quality of life. 

Most people with lupus experience fatigue or tiredness throughout the day. For some, fatigue makes it difficult to perform daily tasks such as taking a shower or going to work.

Fever 

According to an article titled ‘Fevers in Adult Lupus Patients’, fever crops out in 36 – 86 % of SLE patients, making it a common manifestation of the disease.

Fever is on and off in lupus individuals. Many people with lupus experience a fever of more than 100 degrees Fahrenheit. This frequently arises due to inflammation or infection and is manageable with medications.

Joint pain 

Lupus can also bring about joint inflammation, which health care professionals refer to as inflammatory arthritis. 

Arthritis related to lupus sets in due to joint lining inflammation, known as synovitis.

It can unfold joint pain, tenderness, stiffness and swelling. Lupus arthritis typically affects joints located further away from the centre of the body, such as the fingers, elbows, wrists, knees, toes and ankles. 

A butterfly-shaped rash 

Malar rash, commonly referred to as butterfly-shaped rash, is noticeable on the cheeks and at the nose’s bridge. 

The rash is prominent post sun exposure. Being transient or progressing to other areas of the face skin depends on the severity of the disease.

The butterfly-shaped rash will not leave any scars, but one may notice some skin discolouration, such as dark or light spots.

Skin lesions 

Ring-shaped lesions: These lesions appear as a non-itchy red scar. Arms, neck, shoulders or certain exposed body parts usually have these scars. They may eventually discolour the skin.

Coin-shaped lesions: Occurring on the scalp and face, coin-shaped lesions are red and scaly. Though they are non-itchy, scars and discolouration on the skin are inevitable. These lesions can clog the hair follicles, triggering hair fall. Hair loss may become permanent if the lesions heal and form scars.

Shortness of breath 

Lupus can also bring on inflammation of the lining of the lungs. Deep breaths frequently aggravate pain, causing pleurisy. 

Inflammation can cause scarring in the lungs, resulting in shortness of breath. Pleuritis, or inflammation of the tissue that covers the lungs, is a common lung problem in people with lupus. 

While breathing, these inflamed lung surfaces can cause chest pain and shortness of breath due to pleuritis.

Dry eyes 

Dry eye syndrome is a frequent manifestation of lupus. People with dry eyes often describe the sensation as having sand in their eyes. 

It manifests itself when there is inflammation in the lacrimal glands, which prevents the natural moistening of the eye from occurring. 

Secondary Sjogren’s syndrome affects about 20% of people with lupus. It is a condition in which the tear glands do not produce enough tears to lubricate and moisten the eyes. Other moisture-producing glands are also similarly affected.

Signs of lupus in females 

Hair loss 

Many people with lupus go through skin issues, including rashes or scalp sores, which can lead to hair loss. Loss of hair and thinning hair can also be side effects of some lupus medications, such as steroids and immunosuppressants.

Sensitivity to sunlight 

Sun-sensitivity symptoms can pop up several days or even weeks after excessive sun exposure.

Only about one-third of lupus patients are sun sensitive, but photosensitivity is common in people with lupus— affecting about 40% to 70% of people with lupus. 

These individuals will find that exposure to UV rays from sunlight or artificial light aggravates their disease. 

This can give rise to signs like itching, skin rashes and burning. Too much sun exposure can also trigger flares in people with systemic lupus, causing joint pain, weakness and fatigue.

Mouth ulcers 

Lupus mouth ulcers are red with a white halo. They most usually appear on the inside the cheeks, the roof of the mouth or on the lips. Mouth ulcers in lupus patients may or may not be painful.

Arthritis 

Almost 95% of lupus patients will develop arthritis or arthralgias (joint pains) at some point during their disease’s progression.

Malar rash 

The malar or butterfly rash can occur immediately or after sun exposure, affecting approximately half of all lupus patients.

Signs of lupus in males 

Cardiovascular complications 

Lupus elevates the chances of developing heart disease, including coronary artery disease (CAD). Coronary artery disease may provoke a heart attack.

Lupus can also cause inflammation in various parts of the heart, resulting in endocarditis, myocarditis and pericarditis.

Low blood count 

The complete blood count (CBC) of many people with systemic lupus is abnormal. People with lupus generally have low white blood cell count (leukopenia) due to a weak immune system.

Weight loss 

Weight loss may occur involuntarily as a result of low appetite or digestive issues. It can also be a possible side effect of some lupus medications.

Kidney complications 

Lupus nephritis is a kidney complication that develops when lupus autoantibodies attack waste-filtering structures in the kidneys. 

This triggers kidney inflammation, which can lead to blood and protein in the urine, high blood pressure, impaired kidney function and, in severe cases, kidney failure.

Chest pain 

Lupus also stirs up inflammation of the heart and the sac that surrounds it, which can result in sharp chest pain. Inflammation of the outer lining of the lungs also occurs in lupus patients, causing aggravated pain during deep breaths.

Risk factors of lupus  

Gender

Women are more prone to developing lupus than men.

Age  

Symptoms and diagnosis more often occur between the ages of 15 and 44. Only 15% of people who are diagnosed with lupus will experience symptoms before the age of 18.

Race  

Lupus affects people of colour more frequently than Caucasians. This includes African Americans, Asian Americans, Hispanics/Latinos, Native Hawaiians, Native Americans and Pacific Islanders.

Family history

Blood relatives of lupus patients have a 5-13% chance of developing the disease. However, if their mother has lupus, only about 5% of their children will develop it.

Treatments for Lupus  

Hydroxychloroquine 

Hydroxychloroquine administration is to help control mild lupus-related symptoms such as skin and joint disease. Employing this medication is useful in treating fatigue and mouth sores.

Corticosteroids and immune suppressants 

Applying steroid creams directly on rashes treats them. Ointments are generally safe, particularly for mild rashes.

Low-dose steroid pills may be effective for mild or moderate lupus symptoms. The need for higher steroid doses occurs when injuries occur in the internal organs. Regrettably, high doses are also more likely to cause side effects. 

Immunosuppressive medications, such as azathioprine, cyclophosphamide and methotrexate, alleviate lupus symptoms by suppressing immune system activity. Azathioprine also manages lupus.

Belimumab 

Belimumab medication is a monoclonal antibody that inhibits the production of autoantibodies by white blood cells (lymphocytes). 

Autoantibodies are significant because they damage tissue. Belimumab treats lupus that has not shown any effects on other treatments and does not involve the kidneys. 

Diagnosis of Lupus  

Antinuclear Antibody (ANA) Test 

A positive result for the presence of these antibodies, which the immune system produces, indicates that the immune system is stimulated. 

While most people with lupus have a positive ANA test result, the majority of people who have a positive ANA test do not have lupus. A doctor may recommend more specific antibody testing if this test is positive.

Blood test  

Blood test counts white blood cells, red blood cells and platelets. Haemoglobin is also measured as a part of the complete blood test. The results could indicate if a person has anaemia, which is common in people with lupus. 

Lupus can also reveal a low white blood cell or platelet count.

Urine test  

A urine examination will reveal a high red blood cell count or elevated protein level. This implies kidney damage due to lupus. 

Precautions for lupus  

Staying out of the sun 

Prolonged stay in the sun irritates the skin of lupus patients and triggers skin issues. It is advisable to refrain from UV light exposure which is high between 10 AM to 4 PM.

Applying sunscreen every day 

Applying a generous layer of sunscreen with an SPF of 30 or higher offers broad-spectrum protection against both UVA and UVB rays. 

The Food and Drug Administration, along with the Centers for Disease Control and Prevention, recommend reapplying sunscreen every two hours or more frequently if swimming or sweating.

Wearing sun-protective clothing 

Skin protection from head to toe is mandatory. Wearing tightly woven clothing that covers the skin, a wide-brimmed hat and sunglasses are protective against sun rays.

When to see a doctor? 

A doctor visit becomes necessary when a patient experiences the following:

• Fever that is much higher than usual or persistent
• Unbearable abdominal pain 
• Persistent ache
• Shortness of breath
• Exhaustion
• Chest pain
• Excessive bruising or bleeding on the body 
• Unexplained rash
• A slew of symptoms, including severe headache and neck stiffness

A person previously diagnosed with lupus requires a doctor’s visit when the symptoms aren’t improving or have become worse even after following the proper treatment plan, including medications.

Conclusion

Lupus is a complicated autoimmune disease, and Systemic lupus erythematosus (SLE) is the most common type of lupus. Inflammation associated with lupus can be misinterpreted as symptoms of other diseases, making diagnosis difficult. 

There is no complete cure for lupus at the moment, but treatment can help control the symptoms. Regular doctor visits with a proper treatment plan can ease pain during the course of the disease.

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