Autoimmune Diseases That Can Be Fatal: Symptoms, Diagnosis and Treatment

Life and death with an autoimmune disease

Normally, your immune system fights off disease and protects your body from infection. However, when you have an autoimmune disease, your immune system instead mistakenly attacks healthy cells, tissues and organs, doing damage to the body.

There are more than 100 autoimmune diseases. According to the National Institutes of Health, up to 23.5 million Americans suffer from an autoimmune disease. The list of potentially fatal autoimmune diseases or those that can shorten your lifespan is long.

“Almost all autoimmune diseases decrease life expectancy,” says Dr. Betty Diamond, director of the Institute of Molecular Medicine at the Feinstein Institutes for Medical Research at Northwell Health in Manhasset, New York.

One notable exception is hypothyroidism or underactive thyroid, she says. Some studies suggest that hypothyroidism, in fact, prolongs lifespan. A study published in JAMA Internal Medicine suggests that people with low-normal thyroid function live up to 3.5 years longer than those with high-normal thyroid function. Researchers theorized that an underactive thyroid may promote energy conservation, which helps you cope with acute and chronic stressors, or that there could be a genetic factor linked with increased life span.

How autoimmune diseases affect lifespan

There are autoimmune diseases that have a very high rate of mortality, but they are very rare, according to experts. One example of that is autoimmune myocarditis, a rare autoimmune disease characterized by inflammation of the heart muscle, notes Dr. Virginia Pascual, director of the Gale and Ira Drukier Institute for Children’s Health at Weill Cornell Medicine in New York City.

According to a study the Netherlands published in the journal Rheumatology, systemic autoimmune diseases – meaning diseases that affect many organs, such as systemic sclerosis or vasculitis, lupus or scleroderma – have a mortality rate of about 15 persons per million of the population. Their findings also suggest that the mortality risk of systemic autoimmune diseases increases with age, which may be explained by the lifetime accumulation of damage to several vital organs and systems in the body.

Other diseases are more common in the population, like rheumatoid arthritis and Type 1 diabetes, that impact a greater number of people, Pascual says. Often, autoimmune disorders can be an underlying cause of death due to complications caused by multiple comorbidities, meaning they have more than one chronic condition occurring at the same time.

Overlapping autoimmune conditions

To complicate matters, some patients may have more than one autoimmune condition, which can be challenging to manage. For example, having myasthenia gravis – an autoimmune disease that affects the skeletal muscles – puts you at risk for other autoimmune diseases, says Dr. Sujata Thawani, an assistant professor in the division of neuromuscular medicine at New York University Langone Health in New York City.

Although research is ongoing, experts suggest it’s possible that genetic risk factors, environmental triggers or an already-vulnerable immune system contribute to a patient having more than one autoimmune disease.

Here are 10 autoimmune diseases that may impact life expectancy:

Autoimmune myocarditis

Autoimmune myocarditis is a rare autoimmune disorder characterized by inflammation in the heart muscle.

Myocarditis is often diagnosed in people in their 20s to 40s, and symptoms like an abnormal heartbeat, chest pain, shortness of breath, fatigue and fever can come on suddenly and without warning. The condition is underdiagnosed and may cause sudden death.

If you experience symptoms that present like a heart attack, it’s important to seek medical attention immediately for treatment and to evaluate possible causes, including the possibility of myocarditis. When it's diagnosed, drugs that suppress the immune system may be used to treat autoimmune myocarditis.

Multiple sclerosis

Nearly 1 million people in the U.S. are living with multiple sclerosis, according to a study funded by the National Multiple Sclerosis Society. This autoimmune disorder affecting the brain and spinal cord results from the immune system attacking a protective layer around the nerves in that area called the myelin sheath, causing a range of problems.

Symptoms of MS include:

• Difficulty with coordination and balance.
• Spasticity, commonly in the legs.
• Fatigue.
• Memory issues.
• Weakness.
• Bladder dysfunction and bowel problems.

Treatment improvements continue to inch life expectancy for those with MS closer to what’s typical for people without the neurological disorder. However, research published in the journal Neurology suggests people with MS live an average of seven years less than those without MS. Those with MS had a median age of survival of about 76 years compared to 83 years for a matched general population, according to the study.

A long-term study from Norway published in the Journal of Neurology, Neurosurgery and Psychiatry similarly found a seven-year shorter life expectancy for people with MS compared with the general population.

A cure for MS has not yet been identified. Treatment typically focuses on preventing the permanent damage that can occur in the central nervous system early in the disease. This can be combined with symptom management and maintaining a healthy lifestyle. Among other treatment options, drug therapies targeting B cells – a type of white blood cell used by the body’s immune system – have shown promise.

“B cell depletion therapy has been phenomenal for many patients with multiple sclerosis and is now standard of care, which it certainly wasn’t some years ago,” Diamond says.

Lupus

An estimated 1 million to 1.5 million people in the U.S. have lupus, an autoimmune condition characterized by facial rashes, continual fatigue and joint pain. About 90% of adults with lupus are women, according to the Centers for Disease Control and Prevention.

In addition to treating the autoimmune disorder itself, clinicians have to be mindful of treating other health problems caused by or related to the autoimmune disorder. For example, the most common type of lupus – systemic lupus erythematosus – causes widespread inflammation and impacts organs like the kidneys (lupus nephritis). Improved treatment of kidney disease, including the availability of kidneys for transplant, has helped improve the outlook for many with lupus.

Because lupus causes your body's immune system to mistakenly attack itself, immunosuppressive therapies work to lower this response. However, therapies that suppress the immune system also put patients at a higher risk of infection – a cause of death in some patients with lupus. That's why having better antibiotics and treatments for some of those infectious complications have also positively impacted survival rates for people with lupus.

As with other autoimmune conditions, various factors impact life expectancy for people with lupus, including ethnicity. In the U.S., lupus mortality is higher in Black, Hispanic and Asian populations.

Type 1 diabetes

Commonly diagnosed in kids, Type 1 diabetes is an autoimmune disease in which the immune system attacks the pancreas and prevents the body from making the insulin needed to properly control blood sugar.

There isn't a cure for Type 1 diabetes, but those with well-controlled diabetes can live long, full lives and have a good quality of life. However, Type 1 and other forms of diabetes, which aren't autoimmune diseases, can cause a variety of serious complications, particularly if it isn’t well-managed. Those can include kidney and heart disease, eye disease (diabetic retinopathy) and nerve problems.

Type 1 diabetes is managed with insulin to keep blood sugar within a healthy range rather than suppressing the immune system. Exemplifying the impact of lifestyle, proper management of Type 1 diabetes means also carefully considering diet, as well as exercising regularly. That includes controlling carbohydrate consumption and evenly spreading carbs across the day with meals and snacks.

Vasculitis

Vasculitis, or inflammation of the blood vessels, can occur on its own or be caused by other autoimmune disorders like lupus or rheumatoid arthritis. Vasculitis also decreases life expectancy, notes Dr. Ignacio Sanz, chief of the division of rheumatology at Emory University School of Medicine and director of the Lowance Center for Human Immunology at Emory University and Children's Healthcare of Atlanta.

With vasculitis, the body's immune system attacks the blood vessels and causes inflammation. Severe cases of the disease can cause damage to your organs or blood clots, leading to death.

Treatment involves using medications like corticosteroids to control inflammation and address any underlying disease. Biologic therapies may be used to deal with immune system issues that contribute to the condition.

To prevent infections that can occur when immunosuppressive treatments are used for any autoimmune disorder, patients are encouraged to stay up to date on vaccinations. That includes getting vaccinated to prevent the flu, shingles and pneumonia, as recommended by a doctor. You should also ask your doctor if receiving the COVID-19 vaccine is right for you and your particular autoimmune disease. Vaccination should be part of the standard of care with autoimmune diseases, Sanz says.

Myasthenia gravis

Myasthenia gravis is an autoimmune neuromuscular disease that causes muscular weakness. There are approximately 36,000 to 60,000 estimated cases in the U.S., but the disease is underdiagnosed and prevalence is likely higher, according to the Myasthenia Gravis Foundation of America.

“It’s considered a disease of the neuromuscular junction,” Thawani says. “It’s the space in which a muscle and a nerve are communicating. There’s a disconnect between that communication.”

Key proteins in the muscles are not getting appropriate signals from the nerve terminal, or nerve ending, she explains. As a result, the muscle gets fatigued more easily and becomes weaker.

Myasthenia gravis has a predilection for certain muscle groups:

• Ocular muscles that control eye movements and the eyelids
• Muscles in the larynx and pharynx involved with swallowing and speech.
• Head and neck muscles that provide support.
• Limb muscles, shoulder and hip girdle, muscles of the hands and feet.

Patients with myasthenia gravis can take steps to improve their outcomes, Thawani says. That includes getting their bone density checked regularly if they’re on chronic steroids, and also using vitamin D and calcium supplements, as well as getting moderate exercise, maintaining a normal body mass index and not smoking. Fatigue is common in patients with this disease, and addressing mental health is also important.

“Most people can have a normal life expectancy,” Thawani says. "MG has a prognosis of less than 5% mortality. Fifteen percent of people with MG can develop a myasthenic crisis, in which they develop severe weakness of respiratory muscles that leads to prompt treatment in an intensive care setting."

Unfortunately, 15% of patients have what is considered very treatment-resistant myasthenia gravis, Thawani says. That can be due to having associated muscular specific kinase (MuSK) antibodies, tumors of the thymus gland (thymomas) and complex comorbid conditions. The mainstay of treatment is immune suppression.

Rheumatoid arthritis

Rheumatoid arthritis mainly attacks your joints, commonly affecting the hands, wrists and knees. Affected joints become inflamed, causing damage to the joint tissue which can lead to long-lasting pain, trouble with balance and deformity of joints.

The joint disease affects up to 1% of the population, predominantly women, and can raise the risk for heart disease and lung disease, which may be attributed to systemic inflammation, according to a peer-reviewed study published in American Heart Journal. Based on research to date, having RA may decrease life expectancy by a decade or more.

RA is one of a number of autoimmune disorders called rheumatic diseases, or musculoskeletal conditions marked by inflammation. Most rheumatic diseases have a decreased life expectancy and increased mortality, Sanz says.

However, as with other autoimmune diseases, life span varies greatly with RA by the individual, and proper management of the disease can improve a person’s outlook. Treatment often includes the use of disease-modifying antirheumatic drugs (DMARDs) and self-care for arthritis symptoms, such as using a heating pad and pain relievers to ease joint discomfort.

Psoriasis

Just as rheumatoid arthritis can impact health well beyond inflaming joints, psoriasis is more than a skin disease. The autoimmune disorder is also associated with other serious issues, including an increased risk of diabetes, depression and heart disease.

As a result, depending on the severity of psoriasis, it may affect life expectancy and raise mortality risk. In a population-based cohort study of more than 3,600 patients in the U.K., researchers built on previous evidence that severe psoriasis is associated with excess mortality and increased risk of cardiovascular death.

The findings concluded that patients with severe psoriasis had an increased risk of death from a variety of causes, with cardiovascular death being the most common cause. Additionally, the study showed a significantly increased risk of death associated with kidney disease, infection and dementia in patients with severe psoriasis.

For that reason, experts say, patients and their doctors should discuss not only topical treatment to address scaly patches of skin, but if and when immunosuppressive therapy might be warranted.

Scleroderma

Thick, tight, hardened skin is just part of the damage from scleroderma, an autoimmune disease that can also impact connective tissue and internal organs. Scleroderma is considered fairly rare, affecting an estimated 75,000 to 100,000 people in the U.S. – in most cases, women between the ages of 30 and 50 – according to the American College of Rheumatology.

Localized scleroderma mainly affects the skin. With systemic scleroderma, scarring and inflammation can cause permanent damage to the kidneys, lungs, digestive system and other parts of the body.

“Many autoimmune diseases affect quality of life, but not so much life expectancy,” says Dr. John Varga, a distinguished professor of internal medicine and chief of the rheumatology division at the University of Michigan. “Scleroderma causes scarring in the lungs and scarring of the blood vessels, and both of those are leading causes of death. When that happens, which it does in a lot of patients, their life expectancy is pretty significantly affected.”

Survival estimates vary. For severe scleroderma, five-year survival rates range from about 75% to 80% but decrease significantly with each decade after diagnosis. For patients with localized disease, five-year survival is estimated at about 90%.

Mixed connective tissue disease

Mixed connective tissue disease (MCTD) is a rare autoimmune disease that can often be life-threatening. MCTD is sometimes called an "overlap disease" because it frequently mimics symptoms from other autoimmune disorders, including lupus, rheumatoid arthritis, scleroderma and myositis.

Early signs of the disease may include:

• Arthritis, or inflamed or swollen fingers or joints.
• Muscle weakness and fatigue.
• Shortness of breath and dry cough.
• Difficulty swallowing.
• Acid reflux.
• Rash.

According to the Arthritis Foundation, nearly everyone with MCTD has aching joints and the disease also inflames muscle fibers. Complications of the disease may include:

• High blood pressure.
• Heart or lung disease.
• Kidney damage.
• Anemia.
• Tissue death.

Many patients with MCTD can recover from the disease. However, a third can develop serious life-threatening complications. According to a study on the survival rate and prognostic indicators of MCTD in Hungary, pulmonary arterial hypertension was the leading cause of death in patients with MCTD. The survival rates at five, 10 and 15 years after diagnosis were 98%, 96% and 88%, respectively.

Treatment advancements mean many with autoimmune diseases now live longer.

While disease severity varies, improvements in treatment have greatly bolstered life expectancy and improved quality of life for many living with autoimmune conditions. Therapeutic advances include biological drugs that suppress the immune system activity behind a myriad of health problems for those with autoimmune diseases such as lupus and multiple sclerosis.

Frequently, as with other disease prevention and management, a combination of lifestyle improvements – like eating well, exercising regularly and getting adequate sleep – and medical management are recommended.

Because of the many overlapping symptoms and complexities of autoimmune diseases, some people may face challenges in receiving a prompt and accurate diagnosis.

"The most important thing is to find a physician who’s knowledgeable and experienced and with whom you work well, and together work out a treatment regimen that works for you,” Diamond says.

Depending on your condition, your symptoms may affect different systems of the body. Therefore, managing your autoimmune disease may mean seeing different types of specialists, such as:

• Dermatologist. Dermatologists may treat autoimmune diseases that predominantly affect your skin, such as psoriasis or scleroderma.
• Endocrinologist.Endocrinologists specialize in diseases related to your hormones. You may see an endocrinologist if you have certain autoimmune diseases like Type 1 diabetes.
• Rheumatologist.These doctors specialize in treating arthritis and other inflammatory and immune-related disorders. Rheumatologists may treat autoimmune conditions such as lupus or rheumatoid arthritis.

Some autoimmune conditions that may affect life expectancy:

• Autoimmune myocarditis.
• Multiple sclerosis.
• Lupus.
• Type 1 diabetes.
• Vasculitis.
• Myasthenia gravis.
• Rheumatoid arthritis.
• Psoriasis.
• Scleroderma.
• Mixed connective tissue disease.

Story Source:

By Christine Comizio
March 13, 2023, at 3:27 p.m.
This story was previously published at an earlier date and has been updated with new information.