Use of the dental hygiene interventions of scaling of teeth and root planing including curetting surrounding tissue, orthodontic and restorative practices, and other invasive interventions for persons¹ with lupus.

Lupus, chiefly systemic lupus erythematosus

Terminology used in this Advisory

Resources consulted

• DermIS
• DermNet NZ
• Lupus Canada
• Lupus Foundation of America
• Lupus: MedlinePlus

Terminology varies among centres; the following is used for the purposes of this Advisory.

Lupus is a long-term autoimmune disorder that

1. is characterized by acute and chronic inflammation of various tissues of the body in which
   1. the immune system turns against tissues it normally protects
   2. the consequence is damage to body parts such as
      1. blood vessels
      2. brain 
      3. heart
      4. joints
      5. kidneys
      6. lungs
      7. skin.
2. is termed
   1. cutaneous lupus erythematosus (which includes discoid lupus) when only the skin/mucosa is involved 
   2. systemic lupus erythematosus when internal organs are involved
   3. drug-induced lupus erythematosus when lupus-like eruptions, usually temporary, are attributed to some medications (e.g. hydralazine, an anti-hypertensive)
   4. neonatal lupus erythematosus, an uncommon form which occurs in offspring of certain women with cutaneous or systemic lupus, and for which most manifestations largely resolve in 6 months after birth (although congenital heart block may remain)
3. historically was variously referred to as disseminated lupus erythematosus.

Other terminology used in this Advisory is as follows.

1. Autoimmune disorder (CDHO Advisory), a condition that 
   1. occurs when the immune system erroneously attacks and destroys healthy body tissue
   2. occurs as more than 80 different types of autoimmune disorder (CDHO Advisory)
   3. may be treated with immunosuppression (CDHO Advisory) to control or reduce the immune system’s response. 
2. Bullous systemic lupus erythematosus, a serious but rare disease in persons who have antibodies against their own mouth and skin.
3. Cutaneous lupus erythematosus, lupus erythematosus of the skin, an uncommon group of skin disorders which
   1. most often affect women aged 20 to 50
   2. also affect
      1. children
      2. the elderly
      3. males
   3. may also occur in sub-acute form
   4. may affect oral mucosa. 
4. Discoid lupus erythematosus, a chronic form of cutaneous lupus erythematosus involving
   1. a red, raised rash on the face, scalp, or elsewhere
   2. oral mucosa, lips, nose, and/or eyelids in some cases
   3. raised areas that may
      1. become thick and scaly
      2. lead to scarring
   4. durations of days or years
   5. possible recurrences.
5. Drug-induced lupus, caused by medications of various types, of which the symptoms and signs
   1. resemble those of systemic lupus erythematosus 
   2. typically disappear when the drug is stopped. 
6. Flare, an unpredictable episode of lupus, followed by remission, which may be triggered  by
   1. sunlight
   2. stress
   3. certain medications
   4. pregnancy.
7. Herpes simplex labialis (fever blisters)
   1. common in the general population
   2. when associated with lupus may require antiviral drugs.
8. Immune system
   1. is a network of cells, tissues, and organs that work together to defend the body against attacks by infection-causing biological agents, for which the body is otherwise an ideal environment, such as 
      1. bacteria
      2. fungi 
      3. parasites
      4. viruses 
   2. recognizes and destroys substances containing antigens, which are molecules
      1. mostly of proteins
      2. on the surface of cells, bacteria, parasites, fungi and viruses 
      3. of nonliving substances such as
         1. chemicals
         2. foreign particles 
         3. medications
         4. toxins 
   3. normally recognizes the body’s own cells’ antigens and, with the help of human leukocyte antigens, does not react against them. 
9. Lesion, a term variously and loosely used in medicine to refer to such things as
   1. any abnormality of tissue in the body, including the mouth and skin
   2. any localized abnormal structural change in a bodily part
   3. a mass especially before a definite diagnosis is established
   4. cancer
   5. an injury to living tissue, such as a cut or break in the skin. 
10. Mucosal discoid lupus erythematosus
    1. the most frequent form of cutaneous lupus erythematosus
    2. occurs on oral mucosa only in association with discoid lupus erythematosus
    3. most commonly affects the inner cheeks, often with associated lip lesions which, though usually asymptomatic, become painful when ulcerated. 
11. Opportunistic infections 
    1. occur because of a weakened immune system
    2. are a particular cause of death for people with acquired immunodeficiency syndrome (AIDS) because the human immunodeficiency virus (HIV) causes death by impairing the immune system.
12. Oral apthae, apthous stomatitis canker sores, a recurrent disease of the oral mucosa of unknown cause characterized by 
    1. small white round or oval ulcerative lesions
    2. two to eight crops of lesions occurring at one time
    3. lasting for 7 to 14 days and then healing without scarring.
13. Oral cancer, an increased risk for skin and mucosal cancer for immunosuppressed individuals, including people with lupus who are receiving immunosuppressive therapy.
14. Oral candidiasis (thrush), a common complication of immunosuppressive therapy, characterized by sharply defined patches of whitish layers which, when removed, leave an underlying erythematous base. 
15. Oral ulcer, an open lesion, often painful, inside the mouth or upper throat, an alternative name for 
    1. mouth ulcer
    2. aphthous ulcer 
    3. aphthous stomatitis, also known as a canker sore
    4. a cancerous ulcer.
16. Sicca syndrome
    1. a term reserved for the combination of dryness of the mouth and eyes, regardless of cause
    2. when accompanied by lymphocyte infiltration of the salivary glands is named Sjögren syndrome.
17. Sjögren syndrome (CDHO Advisory), a serious, systemic, chronic autoimmune disease that occurs with lupus and which
    1. is considered to be one of the most prevalent autoimmune diseases
    2. is often under-recognized and under-treated
    3. causes xerostomia
    4. most commonly attacks and damages the salivary, tear and mucous-secreting glands, resulting in dry mouth or swollen salivary glands
    5. may cause arthritis, painful weak muscles, neuropathy and debilitating fatigue
    6. may result in inflammation of the 
       1. blood vessels
       2. brain
       3. gastrointestinal system
       4. kidneys
       5. liver
       6. lungs
       7. thyroid gland.
18. Stevens–Johnson syndrome, a rare complication of mucosal herpes outbreaks
    1. triggered by medications, most commonly sulfa drugs, anticonvulsants, and analgesics 
    2. is characterized by 
       1. severe erosions of at least two mucosal surfaces with extensive necrosis of the lips and mouth
       2. purulent conjunctivitis.
19. Sub-acute cutaneous lupus erythematosus, skin lesions that 
    1. appear on parts of the body exposed to sunlight
    2. do not cause scarring.
20. Systemic lupus erythematosus, the form of the disease generally referred to as lupus
    1. usually first affects persons aged 15 to 45 years
    2. also occurs in childhood and in the later adulthood
    3. is characterized by 
       1. arthritis
       2. rash
       3. fever
       4. chest pain. 
21. Toxins, substances that
    1. are released by microorganisms such as bacteria 
    2. are created by plants and animals and that are also poisonous to humans
    3. include medications that are helpful in therapeutic doses but harmful when used in an excess amount.
22. Xerostomia, abnormal dryness of the mouth resulting from decreased secretion of saliva, variously caused by
    1. sicca syndrome
    2. Sjögren syndrome (CDHO Advisory)
    3. some medications.

Overview of lupus

Resources consulted

• College of Dental Hygienists of Ontario: Lupus Fact Sheet
• Lupus Canada
• Lupus Foundation of America
• Lupus: Mayo Clinic
• Lupus: MedlinePlus
• Systemic Lupus Erythematosus
• Stevens-Johnson Syndrome: DermIS
• What Is Lupus?: National Institute of Arthritis and Musculoskeletal and Skin Diseases
• Keeling SO, Alabdurabalnabi Z, Avina-Zubieta A et al. Canadian Rheumatology Association Recommendations for the Assessment and Monitoring of Systemic Lupus Erythematosus. The Journal of Rheumatology. 2018 Oct;45(10):1426-1439; DOI 10.3899/jrheum.171459
  http://www.jrheum.org/content/45/10/1426

Occurrence

Lupus 

1. occurs in the form of a complex disease characterized by 
   1. flares and periods of remission
   2. warning signs of a flare that include
      1. abdominal discomfort
      2. dizziness
      3. fever
      4. headache
      5. increased fatigue
      6. pain
      7. rash
2. occurs as 
   1. bullous systemic lupus erythematosus, a serious but rare disease in persons who have antibodies against their own mouth and skin.
   2. cutaneous lupus erythematosus, lupus erythematosus of the skin, an uncommon group of skin disorders
   3. discoid lupus erythematosus, a chronic form of cutaneous lupus erythematosus
   4. drug-induced lupus, caused by medications of various types, of which the symptoms and signs resemble those of systemic lupus erythematosus 
   5. mucosal discoid lupus erythematosus the most frequent form of cutaneous lupus erythematosus
   6. systemic lupus erythematosus, the most common form of the disease and generally referred to as lupus
3. occurs 
   1. in up to 60,000 Canadians (including up to 15,000 to 30,000 with SLE), mostly women in their childbearing years
   2. much more commonly in women than men
   3. at any age, but most often in persons between the ages of 10 and 50
   4. in persons of African and Asian origin more often than in persons of other races.

Cause

Lupus

1. is an autoimmune disorder of which the underlying causes 
   1. are not fully known
   2. likely comprise some combination of genetic, environmental, and possibly hormonal factors 
2. as drug-induced lupus is also caused by certain medications.

Risk factors

Lupus increases susceptibility to infection because

1. it directly impairs the immune system, which reduces the body’s defences against opportunistic infection
2. many of the medications used to treat it 
   1. suppress the function of the immune system
   2. have oral side effects. 

Signs and symptoms

of lupus 

1. vary from person to person
2. may derive from severe, even life-threatening comorbidities, complications or associated condition
3. may come and go
4. usually include pain and swelling of joints
5. commonly include
   1. chest pain associated with deep breathing
   2. fatigue, which is extreme
   3. fever without apparent  cause
   4. general discomfort, uneasiness, or malaise
   5. hair loss of an unusual nature
   6. lymph node swelling
   7. oral ulcers
   8. rash exacerbated by sunlight 
      1. over the cheeks and bridge of the nose, termed the “butterfly” rash 
      2. involving other regions of skin
   9. sensitivity to the sun
   10. swelling of the legs or around the eyes
6. may relate to specific organs 
   1. brain and nervous system
      1. headaches
      2. numbness
      3. tingling
      4. seizures
      5. vision problems
      6. personality changes
   2. gastrointestinal system
      1. abdominal pain
      2. nausea and vomiting
   3. heart, arrhythmias
   4. lungs
      1. blood in sputum
      2. dyspnea
   5. lymph glands, swelling
   6. skin
      1. patchy skin colour
      2. Raynaud’s disease
7. muscles, joints and limbs
   1. arthritis of the
      1. fingers
      2. hands
      3. knees
      4. wrists
   2. painful or swollen joints and muscle pain
   3. pale or purple fingers or toes in response to cold or stress. 

Medical investigation

of lupus involves

1. physical examination for the typical signs
2. investigations such as
   1. antinuclear antibody test
   2. complete blood count
   3. chest x-ray
   4. kidney biopsy
   5. urinalysis.

Treatment

of lupus 

1. lacks a cure but
   1. symptoms can be effectively controlled with medications
   2. most persons with lupus can lead active, healthy lives 
2. requires individualized regimens, which may include 
   1. for the milder manifestations 
      1. antimalarials
      2. low-dose corticosteroids 
      3. corticosteroid creams 
      4. nonsteroidal anti-inflammatory medications 
   2. for the more severe manifestations
      1. immunosuppressive drugs
      2. high-dose corticosteroids
   3. aggressive specialized treatment, including surgery, for comorbidities, complications or associated conditions 

Prevention

for lupus, no means of prevention exists but for its comorbidities, complications and associated conditions, preventive care is essential, such as

1. bone mineral density tests for osteoporosis
2. immunizations that are kept up to date 
3. preventive cardiac care
4. protective clothing, sunglasses, and sunscreen for exposure to sunshine
5. therapeutic and social approaches to counter depression and mood changes.

Prognosis

for lupus 

1. is related to its severity
2. has improved in recent years
3. during pregnancy, with appropriate treatment, is good for a normal pregnancy and delivery of a healthy infant, though the prognosis may be clouded by complications such as
   1. severe kidney disease
   2. heart disease 
   3. the presence of lupus antibodies.

Social considerations

Persons with lupus benefit from reliable information resources and opportunities to connect with others facing the challenges of this condition, resources include

• Lupus Foundation of Ontario
• Lupus Foundation of America 
• The National Institute of Arthritis and Musculoskeletal and Skin Diseases 

Multimedia and images

• Lesions of chronic discoid lupus erythematosus
• Stevens-Johnson Syndrome
• Systemic lupus erythematosus skin lesions

Comorbidity, complications and associated conditions

Comorbid conditions are those which co-exist with lupus but which are not believed to be caused by it. Complications and associated conditions are those that may have some link with it. Distinguishing among comorbid conditions, complications and associated conditions may be difficult in clinical practice. 

Comorbid conditions, complications and associated conditions for lupus are as follows.

1. Blood
   1. anemia of chronic disease (CDHO Advisory)
   2. hemolytic anemia
   3. thrombocytopenia
2. Central nervous system
   stroke (CDHO Advisory)
3. Circulatory system
   deep vein thrombosis
4. Heart
   1. coronary heart disease 
   2. endocarditis
   3. myocarditis
   4. pericarditis
   5. valvular heart disease, apparently arising from antibody action, which increases the risk of infective endocarditis
5. Kidney
   1. kidney failure
   2. lupus nephritis
6. Lungs
   1. pleural effusion
   2. pulmonary embolism

Oral health considerations

Adapted from

• American Heart Association (Prevention of Endocarditis Guidelines)
• Little JW, Miller CS and Rhodus NL. Little and Falace’s Dental Management of the Medically Compromised Patient (9^th edition). St. Louis: Elsevier; 2018.
• Lupus Foundation of America
• Oral health and the masticatory system in juvenile systemic lupus erythematosus

1. A person with lupus is at increased susceptibility to infection because
   1. it directly affects the immune system, which reduces the body’s defences against infection
   2. many of the medications used to treat it
      1. suppress the function of the immune system
      2. have oral side effects. 
2. With valvular heart disease
   1. the valve damage creates the risk of infective endocarditis with interventions that cause transient bacteremia, such as the Procedures
   2. antibiotic prophylaxis is not typically indicated for infective endocarditis for patients/clients receiving invasive dental procedures (as per the American Heart Association’s 2007 guidelines for patients/clients with SLE-associated valvular disease).
3. The oral mucosa is one of the most common sites of lupus lesions, though internists, rheumatologists, and even dermatologists, sometimes omit examination of the mucous membrane; lesions include
   1. bullous systemic lupus erythematosus. 
   2. mucosal discoid lupus erythematosus 
   3. oral apthae, which 
      1. affect up to 15 percent of the general population
      2. occur more frequently with lupus.
4. Non-specific mucosal lesions in lupus often occur secondary to immunosuppression or other therapies, variously including
   1. herpes simplex labialis
   2. oral cancer
   3. oral candidiasis 
   4. Stevens-Johnson syndrome
5. Initial research
   1. found statistical correlation between plaque and gingival bleeding and 
      1. duration of juvenile systemic lupus erythematosus
      2. cumulative dose of prednisone
   2. suggests an association between juvenile systemic lupus and inadequate oral hygiene, higher incidence of gingivitis and temporomandibular joint dysfunction.

1. Promoting health through oral hygiene for persons who have lupus.
2. Reducing the adverse effects, such as performing the Procedures on a patient/client during a flare by
   1. paying attention to the medical history
   2. using appropriate techniques of communication
   3. providing advice on scheduling and duration of appointments.
3. Reducing the risk that oral health needs are unmet.

Identified in the Dental Hygiene Act, 1991 – O. Reg. 218/94 Part III