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FACT SHEET: Adrenal Insufficiency (also known as “AI” and “adrenocortical insufficiency”; includes “primary adrenal insufficiency” [which includes “congenital adrenal hyperplasia” {“CAH”}, “congenital adrenal hypoplasia”, and “Addison’s disease” {also known as “autoimmune adrenalitis”}] and “secondary adrenal insufficiency”)

Date of Publication: June 20, 2019
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Is the initiation of non-invasive dental hygiene procedures* contra-indicated?

  • Possibly, due to decreased stress tolerance if adrenal insufficiency is untreated. 

Is medical consult advised?  

  • Yes, if previously undiagnosed adrenal insufficiency is suspected1. In particular, a suspected adrenal (Addisonian) crisis necessitates immediate referral for emergency medical care. 
  • Yes, if previously diagnosed adrenal insufficiency is suspected to be suboptimally managed.

Is the initiation of invasive dental hygiene procedures contra-indicated?**

  • Yes, this is a potentially life-threatening condition if untreated or sub-optimally managed. 

Is medical consult advised? 

  • See above. 

Is medical clearance required? 

  • Yes, to assess the patient/client’s ability to tolerate the procedures (i.e., underlying health status, stress tolerance, etc.).  
  • Yes, to assess, advise, and prescribe (as appropriate) with regard to possible corticosteroid supplementation prior to dental/dental hygiene procedures2.

Is antibiotic prophylaxis required?  

  • No, not typically for patients/clients taking physiologic corticosteroid replacement therapy for primary AI. However, antibiotic prophylaxis should be considered for patients/clients at risk of immunosuppression (e.g., prolonged use and/or high doses of systemic steroids, as may be the case in some patients/clients with secondary AI depending on the underlying cause). 

Is postponing treatment advised?

  • Yes, if patient/client has previously undiagnosed adrenal insufficiency or inadequately treated adrenal insufficiency.
  • Yes, if patient/client is medically unstable. Adrenal crisis in the operatory3 is a medical emergency, which necessitates cessation of dental/dental hygiene treatment, immediate contact with emergency medical services, and supportive management pending hospital transfer (e.g., placing patient/client in supine position with legs elevated, use of supplemental oxygen, and administration of intravenous or intramuscular hydrocortisone4). 
  • Yes, if patient/client has not complied with pre-medication (e.g., supplementary hydrocortisone or prednisone), when indicated and as directed by the prescribing physician.

Oral management implications

  • Adrenal insufficiency — in particular, deficiency in cortisol — can compromise the patient/client’s ability to adapt to a stressful situation, such as a dental/dental hygiene appointment.
  • Prevention is the best approach to adrenal crises. An appropriate clinical history is necessary to inform potential medical consult and clearance, as well as potential deferral of procedures.
  • Patients/clients with known Addison’s disease may wear an identification bracelet and carry a “stress dose” kit containing 100 mg hydrocortisone solution (adult dose) in a sterile syringe ready for use. During dental/dental hygiene treatment, this potentially life-saving drug should be readily available. 
  • For patients/clients with primary or secondary AI, or on chronic corticosteroid therapy, determination should be made whether corticosteroid supplementation is required before dental/dental hygiene treatment. The stage/degree of adrenal suppression, in conjunction with the type of procedure contemplated and the patient/client’s overall health and anxiety level, informs the medical decision to supplement or not. For most patients/clients, steroid supplementation is not indicated for dental hygiene procedures.
  • Dental/dental hygiene appointments should typically be scheduled for the morning when the patient/client is less tired and better able to deal with stress (and when endogenous cortisol levels are higher). 
  • The patient/client’s anxiety and emotional stress should be minimized in the dental/dental hygiene office. Implementation of a stress reduction protocol can reduce the risk of an adrenal crisis during dental/dental hygiene treatment.
  • Normal chair position can be used for patients/clients with well-controlled AI. However, postural hypotension is an ongoing risk in persons with chronic AI. 
  • For dental procedures, use of long-acting anaesthetic agents should be considered, and adequate treatment of post-operative pain ensured. Care should be taken to prevent iatrogenic fracture during surgery, because extended continuous glucocorticoid therapy thins the bones. Patients/clients with AI who undergo dental surgery should have their blood pressure monitored at frequent intervals. 
  • Exogenous glucocorticoids can interact with other drugs, including:
    • Antacids, which decrease bioavailability of prednisone;
    • Phenytoin, barbiturates, and rifampicin, which accelerate glucocorticoid metabolism; and
    • Insulin, some oral anti-diabetic medications, and some anti-hypertensive medications, for which requirements may be increased.
  • Patients/clients with adrenal insufficiency may be prone to delayed healing and increased risk of infection.

Oral manifestations

  • Diffuse or focal brown macular pigmentation may be seen on the oral mucous membranes in Addison’s disease (but not in secondary AI). The buccal mucosa is most commonly affected, but hyperpigmentation also occurs on the gingiva, palate, tongue, and lips. Intraoral pigmentation often precedes skin pigmentation.
  • Chronic mucocutaneous candidiasis and other mouth sores may occur in primary AI.
  • Angina bullosa hemorrhagica (ABH)5 may occasionally be seen in patients/clients with secondary AI who continually use inhalation corticosteroid therapy (e.g., for asthma or chronic obstructive pulmonary disease).

Related signs and symptoms

  • Adrenal6 insufficiency is an endocrine disorder characterized by inadequate production of adrenal glucocorticoids (e.g., cortisol and cortisone), and possibly also adrenal mineralocorticoids (e.g., aldosterone7 and 18-deoxycorticosterone) and androgens.  
  • Primary adrenal insufficiency8 of Addison’s disease is characterized by progressive destruction of the cortex of the adrenal gland with resultant deficiency of all of the adrenocortical hormones9; the underlying destruction may be idiopathic10, autoimmune, surgical, tumour-related, hemorrhage-related, hemochromatosis11-related, or infectious (including tuberculosis) in nature, and it can also be caused by infiltration of the adrenal cortex by sarcoidosis or amyloidosis12. Treatment involves corticosteroid replacement therapy13. Autoimmune-mediated Addison’s disease may be associated with other autoimmune diseases, including type I diabetes, pernicious anemia, and Graves’ disease.
  • Secondary adrenal insufficiency results from disease of the hypothalamus or pituitary gland (or the treatment of such disease); chronic administration of exogenous corticosteroids (the most common cause, including oral and inhalation formulations)14; or a critical illness (e.g., extensive burns, trauma, or systemic infection). Secondary AI is typically characterized by selective glucocorticoid deficiency with sparing of mineralocorticoid and androgen functions.  
  • Primary AI is uncommon, occurring in about 8 people per 1 million per year (with diagnosis peaking in the fourth decade of life), with a prevalence of approximately 4 to 14 people per 100,000 (equating to about 1300 persons in Ontario and 3300 persons in Canada). Secondary AI is 2 to 3 times more common than primary AI15, with diagnosis peaking in the sixth decade. Both types of AI are more common in women.
  • Clinical manifestations of primary AI tend to develop slowly, and they relate mostly to deficiencies of cortisol and aldosterone. Signs/symptoms include fatigue, muscle weakness, myalgia, decreased appetite, nausea, abdominal pain, weight loss, reduced resistance to stress and infection16, hypoglycemia, salt-craving, and bronzing and patchy hyperpigmentation of the skin and mucous membranes17. Aldosterone deficiency leads to sodium and water depletion with diuresis (increased urination) and secondary dehydration and hypotension. Hyponatremia and hyperkalemia are common.
  • Clinical features of secondary AI are generally less pronounced than in primary AI. Because of the relationship between secondary AI and the medical administration exogenous glucocorticoids, patients/clients may exhibit Cushingoid features such as puffy round face, excess fat on back of neck, central obesity, and bruising. 
  • Adrenal crisis (also known as Addisonian crisis and acute adrenocortical insufficiency) is a rare but potentially life-threatening complication in a patient/client with underlying primary or secondary AI. Sudden withdrawal of exogenous glucocorticoids is the most common cause, but it may also be precipitated by stress (such as that associated with infection, trauma, surgery, pain, and fear)18. Signs/symptoms are fairly rapid in onset, and they include nausea, vomiting, abdominal pain, fever (or hypothermia), hypoglycemia, mental confusion, tachycardia, and hypotension. Without prompt recognition and treatment, adrenal crisis leads to circulatory shock, coma, and death.
  • Osteoporosis can result from long-standing continuous glucocorticoid therapy.
  • While most persons with Addison’s disease can usually lead essentially normal lives with appropriate treatment, the need for supplemental glucocorticoids during periods of stress, illness, or trauma continues indefinitely.

References and sources of more detailed information


Date: October 24, 2018
Revised: October 30, 2019


FOOTNOTES

1 Diagnosis of adrenal insufficiency is confirmed through hormonal blood and urine tests that detect low cortisol levels. Laboratory assessment of suspected primary AI or longstanding secondary AI includes the synthetic adrenocorticotrophic hormone (ACTH) stimulation test, which is a provocative test of the hypothalamus-pituitary-adrenal (HPA) axis. Ultrasound imaging of the adrenal glands, along with antibody tests, may be used to delineate underlying cause of Addison’s disease. Similarly, computerized tomography (CT) scans and magnetic resonance imaging (MRI) may be used to assess the pituitary and hypothalamus following diagnosis of secondary AI.
2 Most patients/clients on chronic corticosteroid therapy (and therefore at risk of secondary AI) do not require supplemental doses of steroid for general, routine dental/dental hygiene procedures or even for most oral surgical procedures. In patients/clients with previously diagnosed (and managed) primary or secondary AI, current evidence indicates that routine dental/dental hygiene care and minor oral surgical procedures (including uncomplicated extractions) do not typically increase stress levels enough to precipitate an adrenal crisis. Thus, corticosteroid supplementation is not typically indicated for such care and procedures, particularly if patients/clients have taken their usual dose of glucocorticoid within 2 hours of the procedure. Major surgical procedures (particularly in patients/clients with primary AI), however, may require steroid supplementation ― usually a 2- to 4-fold increase in the usual dose of glucocorticoid on the day of dental treatment. For both types of AI, supplementation requirements could increase if the patient/client’s overall health is poor, if concurrent fear/anxiety exists, and if concurrent infection is poorly managed. A pregnant patient/client with Addison’s disease may also require increased supplementation.
3 Risk is associated with unrecognized adrenal insufficiency; poor health status and stability at the time of dental treatment; infection; pain; having undergone an invasive procedure or extraction that causes bleeding or discomfort; and having received a general anaesthetic containing a barbiturate. Although adrenal crisis is rare in dental/dental hygiene patients/clients, between 5% and 8% of patients/clients with primary AI require emergency glucocorticoid administration for treatment of adrenal crisis annually.
4 Ideally, 100 mg of hydrocortisone should be administered intravenously to the adult patient/client within 30 seconds. For the paediatric patient/client, hydrocortisone dosing is 2mg/kg (to maximum of 100 mg); alternatively, age-based dosing can be used (< 3 years = 25 mg; 3 to < 10 years = 50 mg; and 10 years and older = 100 mg).
5 ABH = painless, sturdy blood-filled blisters in the mouth that suddenly appear and rupture within 24 to 48 hours
6 The adrenal glands are small glands located on the superior pole of each kidney. The adrenal cortex (90% of the gland) produces and secretes corticosteroids. The adrenal medulla secretes catecholamines; namely, epinephrine and norepinephrine.
7 Aldosterone is essential for maintaining sodium and extra cellular fluid balance, exerting its effect primarily on the nephrons of the kidney, but also on the salivary glands and colon.
8 In contradistinction to adults, the most common cause of primary adrenal insufficiency in children is congenital adrenal hyperplasia, which is also the leading cause of atypical genitalia in female newborns. CAH encompasses a group of autosomal recessive disorders, each of which involves a deficiency of an enzyme involved in the synthesis of cortisol, aldosterone, or both. Deficiency of 21-hydroxylase accounts for more than 90% of cases.
9 Clinical features of adrenocortical insufficiency usually do not develop until 90% of the adrenal cortex is destroyed.
10 Idiopathic means a disease is “of unknown cause” or of “spontaneous origin”. While most cases of Addison’s disease were previously thought to be of idiopathic etiology, current evidence suggests that up to 80% of cases are caused by autoimmune disorders.
11 Hemochromatosis is a medical condition characterized by iron overload.
12 Amyloidosis is a rare disease characterized by the build-up of an abnormal protein (amyloid) in tissues and organs.
13 Cortisol is replaced with an oral synthetic glucocorticoid, such as hydrocortisone, prednisone, or dexamethasone. As a general rule, a normal adult secretes 20 mg of cortisol daily; thus, replacement therapy for patients/clients with Addison’s disease is about 20 mg of exogenous cortisol (i.e., hydrocortisone) daily. Aldosterone, if deficient, is replaced with oral mineralocorticoid such as fludrocortisone acetate.
14 Chronic administration of exogenous corticosteroids results in inhibition of the feedback loop between the hypothalamus, pituitary, and the adrenal glands. As a result, the pituitary gland does not produce enough adrenocorticotrophic hormone (ACTH), which normally stimulates the adrenal gland to produce cortisol (particularly in stressful situations). Synthetic exogenous glucocorticoids (cortisol-like) are used in the treatment of many diseases (e.g., systemic lupus erythematosus, rheumatoid arthritis, asthma, Crohn’s disease, ulcerative colitis, etc.), and they are also used on a long-term basis in patients/clients during immunosuppressive therapy for organ transplantation.
15 Up to 5% of North American adults use corticosteroid medications on a chronic basis and thus are at risk of secondary AI.
16 Cortisol is involved in homeostasis adaptation of cells to stress — it has anti-inflammatory and immunosuppressive effects; it mobilizes fatty acids from adipose tissue; it maintains vascular reactivity; it promotes neoglycogenesis in the liver; it increases glycemia; it inhibits bone formation; and it delays healing.
17 Melanic pigmentation is related to increases of both melanocyte stimulating hormone (MSH) and adrenocorticotrophic hormone (ACTH). Dermatologic darkening is most visible in scars, skin folds, and pressure points such as the knees, elbows, knuckles, and toes.
18 In these stressful situations, greater amounts of corticosteroids are required for the “stress response” than those afforded by endogenous production (i.e., cortisol) or exogenous therapy.


* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc.
** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.