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Lupus – A Kaleidoscope Disease

 

Our use of the term “kaleidoscope” is purposeful in characterizing the many diverse and shifting medical facets and symptoms of lupus as an illness as well as describing the variety and beauty of those living with the disease.

 

Introduction 

Our organization has used the metaphor of the butterfly from its very beginning, and, of course, it is the most widely recognized symbol for lupus around the world.  Did you know that a collection of butterflies is referred to as a “kaleidoscope?“  

Kaleidoscope Fighting Lupus chose the term “kaleidoscope” to be a part of our name because of the powerful symbolism that reflects both the beauty and importance of each individual as well as the colorful resilience of our community – of those living with lupus.   We want this metaphor to continue to represent us – all of us – as a community of beautiful and unique butterflies – a kaleidoscope. 

Additionally, as medical science advances and more discoveries are made, lupus is being broadly recognized as a collection of symptoms and autoimmune manifestations – not just a single, distinct disease. In order to describe this change, the medical community is referring to lupus as a “kaleidoscope” autoimmune disease – where causes and symptoms seem to move and change and involve many overlapping conditions.  

Why is lupus a kaleidoscope Disease?

Many individuals with systemic lupus often present with similar features of other autoimmune illnesses.  For example, he or she may go to the doctor and a diagnosis of lupus only to later have the diagnosis changed to scleroderma or rheumatoid arthritis.

Why does this happen?  Well, some people with lupus can also present with symptoms characteristic of one or more other connective tissue diseases.  When this occurs, your doctor may use the term “overlap” to describe your condition.  Individuals with lupus commonly have a collection of well-recognized overlap diseases.  This is one more reason for lupus to be referred to as a “kaleidoscope” disorder – one in which it exists within a collection of other similar or closely related connective tissue diseases that affect various parts of the body – each with its own causes and treatments.  

Here are of some of the most common overlapping conditions – the other facets to the lupus “kaleidoscope.”

Scleroderma

Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” derives from two Greek words: “sclero” meaning hard, and “derma” meaning skin. One of the most visible manifestations of the disease is hardening of the skin.

There are two main types of scleroderma: localized and systemic. 

Localized scleroderma:

Usually found only in the skin and muscles, rarely elsewhere. This form is usually mild with organs not being affected. Patients with localized scleroderma rarely develop systemic scleroderma.

Systemic scleroderma:

This type may affect connective tissue in various parts of the body including the skin, esophagus, gastrointestinal tract, lungs, kidneys, heart, and other internal organs. It can also affect the muscles, joints, and blood vessels.  The hardening of these organs and tissues can cause them to function less efficiently.

Treatment:

Treatment for scleroderma can include non-steroidal anti-inflammatory drugs and hydroxychloroquine (Plaquenil) typically help both lupus and scleroderma. Methotrexate, azathioprine, prednisone or cyclophosphamide help lupus and overlap syndromes, and perhaps scleroderma lung disease. Scleroderma may modestly benefit from d-penicillamine, relaxin, and minocycline, none of which help lupus. 

Rheumatoid Arthritis

Rheumatoid arthritis (RA)is a form of inflammatory arthritis and an autoimmune disease. In RA, almost exactly like lupus, for reasons no one fully understands, the immune system – which is designed to protect our health by attacking foreign cells such as viruses and bacteria – instead attacks the body’s own tissues, specifically the synovium, a thin membrane that lines the joints. As a result of the attack, fluid builds up in the joints, causing pain in the joints and inflammation that is systemic, meaning it can occur throughout the body. Also similar to lupus, RA can have periods of flares and remissions.

Symptoms can be different for each individual and affect different parts of the body. Some common symptoms are:

  • Feeling a warm sensation in the joints
  • Noticeable inflammation
  • Fatigue
  • Pain or swelling in the affected areas
  • Stiffness and/or a decreased range of movement

Rheumatoid arthritis is most often symmetrical, meaning if a joint on one side of the body is affected, the corresponding joint on the other side of the body may also be involved.  It usually affects small joints on the hands and feet first, like the knuckle joints on the fingers and base of the big toe.  Because the inflammation is systemic, fatigue, anemia, loss of appetite and low-grade fevers are common.

Medications used to treat rheumatoid arthritis are used to:

  1. a) help relieve symptoms and reduce inflammation (non-steroidal anti-inflammatory drugs and corticosteroids), and
  2. b) modify the disease or put it in remission (disease-modifying anti-rheumatic drugs and biologic agents such as Benlysta).

Your physician may recommend using two or more together. Some medications affect the immune system or have other side effects, making careful monitoring by your caregiver very important. Along with medications, working closely with your physician to structure a plan for moderate physical activity can alleviate symptoms and improve your general sense of well-being.

Mixed Connective Tissue Disease (MCTD)

Characterized by signs and symptoms that are expressed in a variety of disorders, mixed connective tissue disease is term used by the medical field for a broad group of conditions that can include several diseases — primarily lupus, scleroderma and polymyositis/dermatomyositis. Mixed connective tissue disease occurs most commonly in young women.

In mixed connective tissue disease, the symptoms of the separate diseases usually don’t appear all at once. Instead, they tend to occur in sequence over a number of years, which can make diagnosis more complicated.  Some of the early signs and symptoms often involve the hands: Fingers may swell, and the fingertips may become white or even numb.  Later, if the disease progresses, organs such as the lungs, heart and kidneys may also be affected.

Polymyositis is one of the more common connective tissue diseases. It features inflammation of the muscle fibers. Like lupus, the cause of the disease is not known. It begins when white blood cells (the immune cells that cause inflammation) spontaneously invade muscles. The muscles affected are typically those closest to the trunk or torso, resulting in weakness that can be severe. Also similar to lupus, polymyositis is more common in women, often “systemic” (occurring in many areas of the body other than just the skin which is then called “dermatomyositis”) and is considered a chronic illness with periods of increased symptoms (flares or relapses) and periods with minimal or no symptoms (remissions).

Drugs such as prednisone are often used in treatment.

Raynaud’s Phenomenon

Raynaud’s (ray-NOHZ) disease or Raynaud’s phenomenon is a problem with blood flow. It is a condition that causes some areas of your body— such as your fingers, toes, and the tip of your nose and ears — to feel numb and cold in response to low temperatures or stress. In most cases, this inconvenience and discomfort lasts only for a short time, while your body is reacting to the coldness, or stress.   In some cases, the skin of the finders and toes can also become very pale in color.  In Raynaud’s disease, the smaller arteries that supply blood to your skin narrow, limiting blood circulation to affected areas. Like many of the diseases listed as “overlap” diseases, women are more likely to have Raynaud’s disease.

Treatment of Raynaud’s disease depends on its severity and whether you have any other health conditions. For many sufferers of Raynaud’s, the management of triggers can minimize the attacks.  The most common trigger is cold, therefore, keeping the body warm, wearing gloves and avoiding cold temperatures can help.  Stress, smoking, caffeine, medications like any cold medicine with pseudoephedrine, as well as beta blockers, can also trigger an attack.

Sjogren’s Syndrome

Sjogren’s syndrome is a systemic autoimmune disease that affects the entire body, but is most often characterized by its effects on the moisture-producing exocrine glands. Symptoms of Sjogren’s may vary depending on the glands involved. The ones that are usually affected include the salivary glands of the mouth, and the lacrimal glands that produce tears, though many others can also be affected. Symptoms can include dry mouth, dry eyes and skin, joint pain and abnormal digestion.  These, in turn, can lead to other conditions such as tooth decay and damage to the cornea.

When Sjogren’s syndrome is associated with another disease such as SLE, the treatment includes treatment of the underlying disease as well and often involves immunosuppressive drugs.  Treatment also includes targeting specific symptoms.

  • For dry eyes, there are a number of eye drops on the market ranging from simple tear solution to thick methylcellulose solutions that are used during the night to protect eyes while sleeping.  Surgically, the tear ducts can be plugged to prevent drainage of any tears that are produced and subsequently keep the eyes lubricated.
  • Dry mouth symptoms are treated primarily through frequent sips of water, though there are medications available, such as pilocarpine (Salagen) and cevimeline (Evoxac), that stimulate saliva production.
  • Sucking on sugarless hard candies promotes saliva production.  These patients must keep meticulous care of their teeth, and visit the dentist frequently.
  • Digestion difficulties can sometimes be alleviated with the addition of pancreatic enzymes to the diet.

Things to Note with Lupus Overlap Diseases

As mentioned above, when one has lupus in combination with other connective tissue or autoimmune diseases, it is often called an overlap disease.  This is one of the main reasons that lupus can be so difficult to diagnose and, all too often, misdiagnosed.  This clustering of coexisting autoimmune diseases in patients with SLE can be called the kaleidoscope of autoimmunity… and can be explained in part by the co-association of genetic and epigenetic variables in each individual.  

Be aware of your symptoms that may indicate the development of an overlap disease so that they can be properly managed and treated.  The treatments for these syndromes are not always the same as those for lupus; therefore they require separate care and medications.  

In Conclusion: 

The term “kaleidoscope” in describing lupus may be new to you, but it has been used for years.  And we, at Kaleidoscope Fighting Lupus, are thrilled that through our name, we can bring even more awareness to this concept as we seek to continue to fight for medical advances for each one of our unique members’ needs and concerns.  

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References

Lorber, Gershwin, & Shoenfeld. (1994). The coexistence of systemic lupus erythematosus with other autoimmune diseases: The kaleidoscope of autoimmunity. Seminars in Arthritis and Rheumatism, 24(2), 105-113.
Lupus and Epigenetics (n.d.). Retrieved from
https://www.kaleidoscopefightinglupus.org/lupus-and-epigenetics/
Lupus overlap diseases:  What are some common diseases associated with lupus (n.d.). Retrieved from
https://www.kaleidoscopefightinglupus.org/lupus-overlap-diseases-what-are-some-common-diseases-associated-with-lupus/

 

Author:  The Kaleidoscope Fighting Lupus Team

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All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.