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Understanding Lupus

Lupus erythematosus is an autoimmune disease that presents as a skin disorder (subcutaneous or discoid lupus) or as a disorder that affects the internal organs or joints (systemic lupus). Lupus can be difficult to diagnose because the symptoms vary depending the specific organ or tissue involvement and it may not be the obvious cause of symptoms or organ dysfunction. This resource covers the history of lupus as a documented disease, an analysis of the disease including the different types of lupus and their causes, symptoms, diagnosis and treatment. An extensive range of resources to further research lupus is included in the final section. This resource is part of a collection of nursing resources, and is useful to nurses, nursing students, or other individuals interested in or working in the medical field.

History

Lupus erythematosus has been documented as early as Ancient Greece by the physician Hippocrates. Lupus is a Latin term that means wolf, and the disease was termed such because the appearance was similar to a wolf bite, according to the physician Rogerius in the 13th century. The malar rash may appear on the face over the bridge of the nose and across the cheek in a formation resembling a butterfly and it is often referred to as a “butterfly rash” for that reason. The first doctor to identify the condition currently regarded as lupus erythematosus was French doctor Pierre Cazenave in the 19th century.

Dr. Moritz Kaposi was the first to identify two separate forms of lupus, discoid (skin) and disseminated (systemic). In 1939, the photosensitive aspect of the disease was discovered to cause flares of the disease and cause rashes to worsen.

In 1948 the LE cell test was developed to diagnose lupus, with half to seventy-five percent of lupus patients testing positive. Discoveries such as the use of an immunosuppressive, cortisone (Prednisone) and anti-malarial to treat lupus were discovered in the fifties. Criteria for a lupus diagnosis were not developed by rheumatologists until 1971.

Other discoveries included a false-positive syphilis test in patients that are diagnosed with lupus and the use of the anti-nuclear antibody test, which does not indicate specifically that a person has lupus but that they have an autoimmune disorder.

Analysis

Lupus erythematosus is an autoimmune disease, in which the body’s antibodies that typically fight disease instead attack the body’s own organs or tissue. The cause of lupus is unknown, but the disease may be brought on in susceptible individuals by an external condition such as contracting a virus, emotional stress, or prolonged exposure to sunlight. The disease is believed to be genetic and overwhelmingly affects females, generally striking between 15 and 45, and is much more common in females of African, Asian or Native American descent. Symptoms can worsen prior to menstruation, leading researchers to conclude that estrogen may play an important role in the disease.

The illness is chronic and has no known cure. The disease ranges from mild to severe, depending on specific organ involvement. The disease typically vacillates between episodes of remission and no symptoms are present and “flares,” in which the symptoms worsen.

The disease may be present concomitantly with other disorders such as fibromyalgia.

The following sections will cover the different types of lupus, symptoms, diagnosis and treatment options.

Types of Lupus

There are four types of lupus: cutaneous lupus erythematosus (discoid lupus), systemic lupus erythematosus, drug-induced erythematosus and neonatal lupus.

Cutaneous or discoid lupus erythematosus (DLE) presents with scaly rashes on the skin, sores in the mouth lining, alopecia (hair loss) and plaque or sores on top of the rashes. Approximately 10 percent of people with DLE develop systemic lupus.

The symptoms of systemic lupus erythematosus (SLE) include a wide range of symptoms such as muscle pain, swollen joints and Raynaud’s disease. This form of lupus attacks the internal organs and tissues, which can potentially lead to organ failure and death. Systemic lupus may involve multiple organ groups, and has subcategories based on the specific system affected.

  • Gastrointestinal Manifestations of Lupus by Richard G. Quist, M.D. discusses the symptoms affecting the upper gastrointestinal system including the esophagus, the small intestine and colon and pancreas.
  • Lupus in the nervous system (central nervous system, autonomic nervous system and peripheral nervous system) may lead to numerous difficult symptoms.
  • Cardiopulmonary disease caused by the inflammation of tissue, linings, and blood vessels and the cardiovascular and pulmonary system.

Drug-induced lupus erythematosus (DIL) is triggered by prescription medications, including procainamide. The symptoms are similar to systemic lupus and the disease typically resolves itself within months of discontinuing the medication.

Neonatal lupus is the presence of lupus symptoms, such as rashes or liver problems, in an infant when the antibodies are passed from a mother with lupus to her child. The child does not contract lupus from the mother and the symptoms are usually temporary. Damage to the fetus or infant is rare.

Symptoms

Symptoms include the fatigue, low-grade fever, joint pain, the tell-tale malar rash (butterfly rash), photosensitivity, Raynaud’s phenomenon, dry eyes and dry mouth, chest pain, weight fluctuations, skin lesions, sores on the membranes in the nose or mouth and psychological symptoms such as depression or anxiety.

Diagnosis

Eleven specific criteria are used to diagnose lupus, including seven symptoms and four lab tests. Four of the eleven are required to point to lupus.

The symptoms are:

  • Butterfly or malar rash across the bridge of the nose and cheeks.
  • Raised, red skin rashes.
  • Photosensitivity, including worsening rashes from exposure to the sun.
  • Ulcers in the nose or mouth.
  • Arthritic swelling and pain in at least two joints.
  • Pleurisy or pericarditis.
  • Psychosis or seizure.

The lab tests include:

  • Positive ANA test
  • Antibodies to cardiolipin, double stranded DNA or Sm indicating immunologic disorder.
  • Low white cell blood or platelet count or hemolytic anemia.
  • High amounts of protein or cellular casts found in urine indicating renal disorder.

Treatment

Because there is no cure for lupus, the treatments used are primarily to alleviate discomfort from symptoms and preventing flares. Treatment will also depend on severity of the disease.

Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen sodium are prescribed to reduce inflammation; anti-malarial drugs are helpful to reduce the incidence of flares and corticosteroids.

If the disease is particularly aggressive, immunosuppressive drugs or higher doses of corticosteroids may be prescribed.

Complementary treatment such as acupuncture may relieve pain and other symptoms. Regular exercise, stress reduction and diet modifications (such as avoiding foods that cause inflammation) may be helpful.

Resources

This collection of resources include online medical communities that offer information about symptoms of the disease, diagnostic information, prognosis, slide shows of the symptoms, blogs and articles by professionals, lupus-focused publications and recommendations for healthy living. Other sources include online video tutorials and information about clinical trials for patients with lupus.

  • Information about systemic lupus erythematosus from PubMed Health. Causes, symptoms, tests and photos of the lesions to review and tutorial on antibodies.
  • Lupus Foundation of America offers an online community, publication dedicated to lupus, research information, information for professionals and latest news about lupus. An interactive tool shows how lupus affects the body.
  • The Mayo Clinic offers extensive information about lupus including causes, symptoms, diagnostic tests, and possible complications. Alternative medicine and home remedies are included in treatment options.
  • WebMD Lupus Center provides an overview of the disease, a slideshow of physical symptoms, discussions, online communities, blogs written by experts and common treatments. Recommended stress and pain-relieving exercises are included with other resources in their Lupus Today section.
  • The Lupus Site from Lupus UK provides information about light sensitivity, blood disorders, and fact sheets about symptoms, diagnoses and medications.  Online forums and information about living with lupus are also available.
  • TeensHealth from Nemours offers information about lupus geared towards teenagers.  The article discusses types of lupus in plain terms including causes, symptoms, and treatment. An audio version of article is offered.
  • Medline Plus provides information about lupus, including an interactive tutorial that is available in English and Spanish. A medical encyclopedia for related terms, multi-media tools and the latest news are offered.
  • Health information for women at womenshealth.gov covers frequently asked questions about lupus. Printer friendly PDF versions are available.
  • Medical News Today provides information about lupus including symptoms, diagnostic criteria, treatment options, and videos from patients living with lupus. Resource includes article archive and latest news about lupus.
  • The Lupus Research Institute is working to find a cure for lupus, conduct extensive research to discover better treatment options and promote awareness about the disease.
  • Clinical trials from the Lupus Research Institute provide options for lupus patients to participate in cutting edge treatments.
  • WebMD Video lupus tutorial overviews lupus, lupus symptoms.
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) extensive article on the Future Directions of Lupus Research covers etiology, genetic and environmental factors, mechanisms of disease, pediatric lupus and more.
  • The Lupus Magazine offers information for lupus patients from around the world. News, events, lifestyle articles, information about lupus written by lupus patients for lupus patients.
  • Lupus support group from Daily Strength is an online forum for lupus patients is free and anonymous.
  • SLE Lupus Foundation: Living with lupus, lupus research, events and ways to get involved, advocacy and support groups based in New York.