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Addison's Disease

Addisons Disease

Addison’s disease is a chronic clinical disease characterized by the progressive decline in the function of the adrenal glands. This potentially fatal disease may result in hyperpigmentation, hypotension, and cardiovascular collapse. Addison’s disease is easily diagnosed clinically and by the use of hormonal assays that reveal an elevated adrenocorticotropic hormone (ACTH) with a low plasma cortisol level.


Presentation

Addison’s disease has a typical insidious and chronic onset which may take several months. The following signs and symptoms are usually presented by patients with primary adrenal insufficiency:

On occasions, the symptomatology of Addison’s disease may appear acutely or abruptly. The acute onset of adrenal insufficiency symptoms is clinically referred to as Addisonian crisis. The following signs and symptoms are typically observed in acute adrenal failure:

Fatigue
  • Forty-eight per cent of patients showed abnormal fatigue, while 61% had severe fatigue. The CIS fatigue scores were significantly higher compared to controls (P 0·01).[ncbi.nlm.nih.gov]
  • The patient noted a marked symptomatic improvement in morning symptoms, but with persistent fatigue during the day.[ncbi.nlm.nih.gov]
  • We report the case of a young man with a history of attention deficit/hyperactivity disorder and mild cognitive impairment who presented with chronic fatigue, anorexia and progressive darkening of the skin.[ncbi.nlm.nih.gov]
  • The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency.[ncbi.nlm.nih.gov]
  • Herein, we describe the case of a 50-year-old gentleman residing in western Sudan who presented with a 7-month history of generalized body weakness, easy fatigue, and frequent attacks of vomiting and diarrhea.[ncbi.nlm.nih.gov]
Weakness
  • Primary adrenal insufficiency or Addison's disease is a rare disorder often difficult to diagnose on presentation by the nature of its associated nonspecific symptoms, such as nausea or weakness.[ncbi.nlm.nih.gov]
  • Classically, patients affected with Addison's disease develop weakness, anorexia, electrolyte imbalances: decreased sodium and chloride with increased serum potassium resulting in hypotension, and hyperpigmentation of the skin and mucous membranes.[ncbi.nlm.nih.gov]
  • Herein, we describe the case of a 50-year-old gentleman residing in western Sudan who presented with a 7-month history of generalized body weakness, easy fatigue, and frequent attacks of vomiting and diarrhea.[ncbi.nlm.nih.gov]
  • A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation.[ncbi.nlm.nih.gov]
  • The physical examination was completely normal except for muscle weakness, hyperpigmentation on labial mucosa and skin in a patient. The laboratory tests revealed anemia and hypoglycemia.[ncbi.nlm.nih.gov]
Weight Loss
  • On review of history, the cause of vomiting and weight loss was questioned and combined with subsequent biochemical testing a diagnosis of Addison's disease was made.[ncbi.nlm.nih.gov]
  • Hyperpigmentation was observed in 76%, nausea and vomiting occurred in more than 40%, and weight loss was noted in 25%.[ncbi.nlm.nih.gov]
  • A 32-year-old Caucasian was admitted at 14 weeks of gestation with hypotension and weight loss.[ncbi.nlm.nih.gov]
  • The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency.[ncbi.nlm.nih.gov]
  • She reported weight loss and gradual increase in her skin pigmentation over a 1-year period. Aggressive fluid resuscitation was instituted. Hormonal tests showed primary adrenal insufficiency.[ncbi.nlm.nih.gov]
Fever
  • A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation.[ncbi.nlm.nih.gov]
  • A 39-yr-old male was referred because of extreme fatigue, weight loss, anorexia, nausea, and bouts of fever. During physical examination hyperpigmentation was seen. Laboratory tests showed a plasma cortisol of 0.02 micromol/l (08:30 h).[ncbi.nlm.nih.gov]
  • Symptoms include: fatigue muscle weakness joint or muscle pain fever weight loss nausea, vomiting, and or diarrhea (leading to dehydration) headache sweating changes in mood or personality, such as irritability, anxiety, or depression loss of appetite[familydoctor.org]
  • That can happen for many reasons, such as an illness, fever, surgery, or dehydration. You may also have a crisis if you stop taking your steroids or lower the amount of your steroids suddenly.[hopkinsmedicine.org]
Anorexia
  • Classically, patients affected with Addison's disease develop weakness, anorexia, electrolyte imbalances: decreased sodium and chloride with increased serum potassium resulting in hypotension, and hyperpigmentation of the skin and mucous membranes.[ncbi.nlm.nih.gov]
  • We report the case of a young man with a history of attention deficit/hyperactivity disorder and mild cognitive impairment who presented with chronic fatigue, anorexia and progressive darkening of the skin.[ncbi.nlm.nih.gov]
  • A 39-yr-old male was referred because of extreme fatigue, weight loss, anorexia, nausea, and bouts of fever. During physical examination hyperpigmentation was seen. Laboratory tests showed a plasma cortisol of 0.02 micromol/l (08:30 h).[ncbi.nlm.nih.gov]
  • They may suffer hypoglycemic symptoms some hours after a large carbohydrate meal but usually their anorexia prevents them from consuming sufficient carbohydrate to do this ...[herballegacy.com]
  • It is characterized by hypotension (low blood pressure), weight loss, anorexia, weakness, and sometimes a bronze-like melanotic hyperpigmentation of the skin.[patientslikeme.com]
Vomiting
  • On review of history, the cause of vomiting and weight loss was questioned and combined with subsequent biochemical testing a diagnosis of Addison's disease was made.[ncbi.nlm.nih.gov]
  • Hyperpigmentation was observed in 76%, nausea and vomiting occurred in more than 40%, and weight loss was noted in 25%.[ncbi.nlm.nih.gov]
  • New onset Addison's disease is rare in pregnancy but may present with prolonged vomiting and weight loss. Therefore adrenal failure should be included in the differential diagnosis of hyperemesis gravidarum.[ncbi.nlm.nih.gov]
  • After initial improvement with antimicrobial therapy, new symptoms appeared, consisting of persistent vomits, abdominal pain and hypotension, which led to the diagnosis of Addison's disease.[ncbi.nlm.nih.gov]
  • Herein, we describe the case of a 50-year-old gentleman residing in western Sudan who presented with a 7-month history of generalized body weakness, easy fatigue, and frequent attacks of vomiting and diarrhea.[ncbi.nlm.nih.gov]
Nausea
  • Hyperpigmentation was observed in 76%, nausea and vomiting occurred in more than 40%, and weight loss was noted in 25%.[ncbi.nlm.nih.gov]
  • Primary adrenal insufficiency or Addison's disease is a rare disorder often difficult to diagnose on presentation by the nature of its associated nonspecific symptoms, such as nausea or weakness.[ncbi.nlm.nih.gov]
  • We present a 51-year-old male patient, who underwent hemodialysis therapy for 8 years, diagnosed with Addison's disease after having myalgia, skin hyperpigmentation, weight loss, sweating, and nausea for the past few weeks.[ncbi.nlm.nih.gov]
  • A 39-yr-old male was referred because of extreme fatigue, weight loss, anorexia, nausea, and bouts of fever. During physical examination hyperpigmentation was seen. Laboratory tests showed a plasma cortisol of 0.02 micromol/l (08:30 h).[ncbi.nlm.nih.gov]
  • A 48 years old male presented in our medical outpatient department with a three day history of sudden onset of severe precordial chest pain that started while playing football which was associated with nausea, vomiting and difficulty in breathing.[ncbi.nlm.nih.gov]
Diarrhea
  • Herein, we describe the case of a 50-year-old gentleman residing in western Sudan who presented with a 7-month history of generalized body weakness, easy fatigue, and frequent attacks of vomiting and diarrhea.[ncbi.nlm.nih.gov]
  • Severe vomiting and diarrhea. Sudden, deep pain in the lower back, belly, or legs. Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission.[my.clevelandclinic.org]
  • Immediate medical attention is needed when severe infections, vomiting, or diarrhea occur, as these conditions can precipitate an Addisonian crisis. A patient who is vomiting may require injections of hydrocortisone instead.[en.wikipedia.org]
  • Nausea, vomiting and diarrhea occur in about 50 percent of all cases. Blood pressure is low and falls further when standing, causing dizziness or fainting.[healthcentral.com]
Abdominal Pain
  • After initial improvement with antimicrobial therapy, new symptoms appeared, consisting of persistent vomits, abdominal pain and hypotension, which led to the diagnosis of Addison's disease.[ncbi.nlm.nih.gov]
  • Seek emergency medical treatment if you experience the following signs and symptoms: Severe weakness Confusion Pain in your lower back or legs Severe abdominal pain, vomiting and diarrhea, leading to dehydration Reduced consciousness or delirium In an[mayoclinic.org]
  • Common symptoms include: weakness fatigue abdominal pain nausea weight loss low blood pressure darkened skin (in the case of Addison’s disease) salt craving (in the case of Addison’s disease) dizziness upon standing depression However, there are different[endocrineweb.com]
  • Symptoms and Causes The most common symptoms are fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain.[niddk.nih.gov]
Loss of Appetite
  • Symptoms and Causes The most common symptoms are fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain.[niddk.nih.gov]
  • Loss of appetite. Low blood glucose. Low blood pressure. Muscle weakness. Nausea. Patches of dark skin, especially around scars , skin folds, and joints. Sensitivity to cold. Unexplained weight loss. Vomiting. Worsening fatigue (extreme tiredness).[my.clevelandclinic.org]
  • You may experience: lack of energy or motivation (fatigue) muscle weakness low mood loss of appetite and unintentional weight loss increased thirst Over time, these problems may become more severe and you may experience further symptoms, such as dizziness[nhs.uk]
Oral Pigmentation
  • The patient presented with oral pigmented lesions, which were hypothesized to be mucosal pigmentation associated with Addison's disease.[ncbi.nlm.nih.gov]
Hypotension
  • As hypotension may be one of the few situations, in which NAAION may be treatable and the visual loss reversible, it is important to recognize and treat sustained episodes of hypotension in these individuals.[ncbi.nlm.nih.gov]
  • During admission for evaluation of hypotension, she was also detected to have hyponatremia. Hyponatremia and hypotension failed to improve despite fluid resuscitation.[ncbi.nlm.nih.gov]
  • She was hyperpigmented, hypotensive and hyponatraemic despite rehydration.[ncbi.nlm.nih.gov]
  • After initial improvement with antimicrobial therapy, new symptoms appeared, consisting of persistent vomits, abdominal pain and hypotension, which led to the diagnosis of Addison's disease.[ncbi.nlm.nih.gov]
  • Cardiovascular complications of the condition are usually limited to hypovolemic hypotension; however, we highlight here a rare, dramatic case of a fulminant adrenal crisis in a young man primarily marked by acute biventricular systolic failure and cardiogenic[ncbi.nlm.nih.gov]
Orthostatic Hypotension
  • Here are some key symptoms that can suggest Addison’s disease: When this condition first begins, you may experience fatigue, lightheadedness, and orthostatic hypotension (a drop in blood pressure when moving from lying down to either a sitting or standing[dummies.com]
  • On physical examination, these clinical signs may be noticed: Low blood pressure with or without orthostatic hypotension (blood pressure that decreases with standing) Darkening (hyperpigmentation) of the skin, including areas not exposed to the sun.[en.wikipedia.org]
  • Poorly managed or undiagnosed adrenal insufficiency Symptoms Inappropriate fatigue Depression Darkening of the skin Loss of appetite, nausea and/or vomiting Diarrhea Salt craving Orthostatic hypotension (light headedness upon standing) or incredibly low[addisonssupport.com]
  • . - Orthostatic hypotension (90%) - Electrolyte abnormalities: hyponatremia (88%) , hyperkalemia (64%) , hypercalcemia (6%) - Hyperpigmented skin and mucosa - Sparse axillary hair (more common in women), amenorrhea, decreased libido (both men and women[clinicaladvisor.com]
  • Other symptoms include orthostatic hypotension (a drop in blood pressure when standing, which can cause dizziness or fainting) and skin changes.[diabetesselfmanagement.com]
Muscle Weakness
  • The physical examination was completely normal except for muscle weakness, hyperpigmentation on labial mucosa and skin in a patient. The laboratory tests revealed anemia and hypoglycemia.[ncbi.nlm.nih.gov]
  • Symptoms of Addison's disease begin gradually and include chronic, worsening fatigue , muscle weakness, loss of appetite , and weight loss .[medicinenet.com]
  • Symptoms and Causes The most common symptoms are fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain.[niddk.nih.gov]
  • Clinical symptoms include ANOREXIA; NAUSEA; WEIGHT LOSS; MUSCLE WEAKNESS; and HYPERPIGMENTATION of the SKIN due to increase in circulating levels of ACTH precursor hormone which stimulates MELANOCYTES.[fpnotebook.com]
Myalgia
  • We present a 51-year-old male patient, who underwent hemodialysis therapy for 8 years, diagnosed with Addison's disease after having myalgia, skin hyperpigmentation, weight loss, sweating, and nausea for the past few weeks.[ncbi.nlm.nih.gov]
  • Chronic Addison's disease Typically, chronic primary adrenal insufficiency has an insidious onset and presents with many non-specific symptoms and signs: - Weakness and fatigability (99%), anorexia (99%), nausea/vomiting, abdominal pain, myalgias/arthralgias[clinicaladvisor.com]
Muscle Cramp
  • Symptoms of adrenal insufficiency include fatigue, nausea, anorexia, postural dizziness and muscle cramps. A history of weight loss, reduced strength and salt craving may also be found.[em-consulte.com]
Arthralgia
  • Chronic Addison's disease Typically, chronic primary adrenal insufficiency has an insidious onset and presents with many non-specific symptoms and signs: - Weakness and fatigability (99%), anorexia (99%), nausea/vomiting, abdominal pain, myalgias/arthralgias[clinicaladvisor.com]
Hyperpigmentation
  • Herein this case report, we focus on the subtle findings of diffuse hyperpigmentation and intermittent but repetitive "flu-like" symptoms in two patients to correctly identify the diagnosis of Addison's disease effectively and efficiently.[ncbi.nlm.nih.gov]
  • The physical examination was completely normal except for muscle weakness, hyperpigmentation on labial mucosa and skin in a patient. The laboratory tests revealed anemia and hypoglycemia.[ncbi.nlm.nih.gov]
  • Hyperpigmentation was observed in 76%, nausea and vomiting occurred in more than 40%, and weight loss was noted in 25%.[ncbi.nlm.nih.gov]
  • We herein report a case of Addison's disease caused by tuberculosis characterized by atypical hyperpigmentation, noted as exacerbation of the pigmentation of freckles and the occurrence of new freckles, that was diagnosed in the presence of active pulmonary[ncbi.nlm.nih.gov]
  • Reactive hyperpigmentation of the skin and mucous membranes may be found in Addison's disease and hyperthyroidism.[ncbi.nlm.nih.gov]
Alopecia
  • A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation.[ncbi.nlm.nih.gov]
  • Autoimmune thyroid disease • TSH; check free T4 and anti TPO antibodies if TSH outside reference range Type 1 diabetes • Fasting glucose/HbA1c Autoimmune parathyroid disease • Bone profile — check PTH if low calcium Vitiligo • Physical examination only Alopecia[bjgp.org]
  • Common autoimmune diseases associated with Addison’s Disease are: Chronic thyroiditis Graves’ disease Testicular dysfunction Hypopituitarism Hypoparathyroidism Type 1 diabetes Alopecia areata Hypogonadism Sjogren’s syndrome Vitiligo Pernicious anemia[dovemed.com]
  • In adulthood, PGA-1 can present with a wide spectrum of disease, including hypothyroidism, hypogonadism, alopecia, pernicious anemia, and vitiligo.[clinicaladvisor.com]
Amenorrhea
  • A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation.[ncbi.nlm.nih.gov]
  • […] weakness Low blood pressure Dizziness Darkening of the skin and mucous membranes (hyperpigmentation) Nausea and vomiting Craving salt Headache Sweating Low blood sugar level Loss of interest in sex (decreased libido) Irregular or absent menstruation (amenorrhea[innerbody.com]
  • Measuring serum calcium, phosphorus, glucose and thyrotropin Measure serum PTH if patient has hypocalcemia Hypogonadism should be investigated in post-menarchal female adolescents presenting in oligomenorrhea or amenorrhea by measuring FSH and LH.[pedclerk.bsd.uchicago.edu]
  • . - Orthostatic hypotension (90%) - Electrolyte abnormalities: hyponatremia (88%) , hyperkalemia (64%) , hypercalcemia (6%) - Hyperpigmented skin and mucosa - Sparse axillary hair (more common in women), amenorrhea, decreased libido (both men and women[clinicaladvisor.com]
  • ., amenorrhea, flushing, fatigue, poor concentration). 34 It is appropriate to offer these patients evaluation and counseling on other options for building a family. 35 Enlarge Print Table 4.[aafp.org]
Oligomenorrhea
  • Measuring serum calcium, phosphorus, glucose and thyrotropin Measure serum PTH if patient has hypocalcemia Hypogonadism should be investigated in post-menarchal female adolescents presenting in oligomenorrhea or amenorrhea by measuring FSH and LH.[pedclerk.bsd.uchicago.edu]
Dizziness
  • She presented with a four-week history of decreased energy, malaise and postural dizziness. Our patient described an unusual diet of liquorice sticks and soy sauce, consuming around 46 g of salt per week.[ncbi.nlm.nih.gov]
  • I don’t need an alarm to wake me up because my friend “dizzy” does it for me. (That is assuming that I slept at all). I wake up dizzy, like really dizzy, feeling very nauseous and my heart racing, I'm stuck on the tile-a-whirl dizzy.[reddit.com]
  • Nine days before admission she had collapsed because of dizziness and had a cerebral concussion and open fracture of her nasal bone, however, no further investigations were carried out at that time.[ncbi.nlm.nih.gov]
  • Common symptoms include: weakness fatigue abdominal pain nausea weight loss low blood pressure darkened skin (in the case of Addison’s disease) salt craving (in the case of Addison’s disease) dizziness upon standing depression However, there are different[endocrineweb.com]
  • Symptoms of adrenal crisis include: Abdominal pain Difficulty breathing Dizziness or lightheadedness Low blood pressure Reduced level of consciousness References Barthel A, Willenberg HS, Gruber M, Bornstein SR. Adrenal insufficiency.[mountsinai.org]
Asthenia
  • Adrenal crisis Adrenal crisis is characterized by Profound asthenia (weakness) Severe pain in the abdomen, lower back, or legs Peripheral vascular collapse Renal shutdown with azotemia Body temperature may be low, although severe fever often occurs, particularly[merckmanuals.com]
Apathy
  • ., apathy, depression) Electrolyte abnormalities (hyponatremia, hyperkalemia, hypoglycemia) Neutropenia, eosinophilia B.[clinicaladvisor.com]

Workup

Addison’s disease is primarily investigated by means of an extensive clinical history taking followed by a careful review of the presenting signs and symptoms. When the patients are suspected of having Addison’s disease the following confirmatory tests may be done to ascertain the diagnosis:

  • Serum electrolytes: This blood test will determine the present level of sodium and potassium in the serum because these electrolytes are usually affected in Addison’s disease.
  • Hormonal assays: The hormonal levels of ACTH and cortisol are likewise determined to support the suspicion of Addison’s disease.
  • ACTH stimulation test: This hormonal test monitors the level of cortisol produced in the body after the injection of a synthetic ACTH hormone [8]. A damaged adrenal cortex may not respond to ACTH stimulation at all.
  • Insulin-induced hypoglycemia test: This will test for secondary adrenal insufficiency or those caused by a pituitary dysfunction. This test measures the levels of blood glucose and cortisol levels at the different time intervals after the introduction of synthetic insulin. The normal response of the body is to decrease the glucose level and increase the cortisol level.
  • Imaging: Computed tomography (CT scan) of the abdomen can elucidate the size and pathology of the adrenal gland at the suprarenal region [9]. Magnetic resonance imaging (MRI) may demonstrate the conditions of the pituitary gland in the skull to rule out secondary causes of adrenal insufficiency.
Hyponatremia
  • During admission for evaluation of hypotension, she was also detected to have hyponatremia. Hyponatremia and hypotension failed to improve despite fluid resuscitation.[ncbi.nlm.nih.gov]
  • Based on the findings of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), we had considered that the cause of the hyponatremia was syndrome of inappropriate secretion of antidiuretic hormone (SIADH) due to active extrapulmonary tuberculosis[ncbi.nlm.nih.gov]
  • The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency.[ncbi.nlm.nih.gov]
  • An 82-year-old woman with miliary tuberculosis was treated with antituberculous chemotherapeutic agents including rifampicin (RFP), but she still demonstrated general malaise in addition to hyponatremia.[ncbi.nlm.nih.gov]
  • Preparation of such a patient for surgery should include treatment of hypovolemia, hyperkalemia, and hyponatremia .[openanesthesia.org]
Eosinophils Increased
  • Increased Imaging X-ray or CT Evidence of Calcification in adrenal area Renal TB Pulmonary TB Differential diagnosis Hyperpigmentation can result from bronchogenic carcinoma , ingestion of heavy metals (eg, iron, silver), chronic skin conditions, or[merckmanuals.com]

Treatment

The main goal in the treatment of Addison’s disease is to replace the deficient hormones in the right levels of steroid hormones that the adrenal glands are incapable of maintaining. The following are options used in the treatment of Addison’s disease:

  • Oral corticosteroids: Patients may be given oral hydrocortisone, prednisone, or cortisone acetate to replace the deficient cortisol hormone. In the same way, fludrocortisone may be given to replace the aldosterone deficiency.
  • Injectable corticosteroids: This steroid is given for the same indications. This option is resorted by patients if they are unable to tolerate oral corticosteroids [10].
  • Androgen replacement therapy: This regimen is given to women with low androgen levels. The oral dehydroepiandrosterone is preferentially used to address problems of libido and sexual satisfaction in women.
  • Sodium supplementations: Sodium may be amply given during activities that incurs heavy perspiration like exercises. Excessive diarrhea and vomiting among patients may also benefit from the sodium supplementations.

Prognosis

Addison’s disease presenting with significant adrenal hormone insufficiency can be life threatening if left untreated. Patients undergoing hormone replacement therapy with the deficient mineralocorticoid and glucocorticoid may live a normal life.

Etiology

The greater majority of cases of Addison’s disease or primary adrenal insufficiency is caused by the idiopathic atrophy of the adrenals, accounting for almost 70% of all cases. The leading theory postulated for the idiopathic atrophy of the adrenals points to an autoimmune origin. There are however, some less common causes of adrenal destruction which include granuloma, tuberculosis, histoplasmosis, amyloidosis, inflammatory necrosis, hemorrhage and tumorous growths. The most common cause of Addison’s disease among children is congenital adrenal hyperplasia (CAH).

Epidemiology

In the United States, the recent prevalence rate of Addison’s disease is up to 60 cases per one million population. Addison’s disease is relatively rare internationally. The increase in the mortality and morbidity rates is usually due to the delay in the replacement therapy of the mineralocorticoid and glucocorticoid hormones [2]. Although, primary adrenal insufficiency is already considered serious and potentially life threatening, concomitant illnesses like malignancies, cardiovascular diseases, and infectious diseases influences greatly the increased mortality rate among these patients [3].

The primary idiopathic and autoimmune form of Addison’s disease is more commonly seen in children and females. There is no racial predilection to Addison’s disease. The mean age of onset is 30 to 50 years old but may present earlier among the infantile cases of congenital adrenal hyperplasia.

Sex distribution
Age distribution

Pathophysiology

The pathogenesis of Addison’s disease or adrenal insufficiency is primarily due to the destruction of the adrenal cortex. Cortical destruction may either be inflammatory, necrotic, neoplastic, infectious, or hemorrhagic in nature. Signs and symptoms of adrenal insufficient usually present clinically when 90% or more of the adrenal cortex has been rendered dysfunctional or destroyed. The main systemic pathology is brought about by the lack of glucocorticoid and mineralocorticoid hormone secretion that is essential to the body.

Prevention

The congenital form of primary adrenal insufficiency cannot be prevented, although; new born screening may detect congenital adrenal hyperplasia in the first two days of life. The prompt diagnosis and hormone replacement therapy of Addison’s disease can prevent untoward complications and death.

Summary

Addison’s disease is a serious disorder that occurs when the adrenal glands are unable to produce sufficient hormones needed for proper metabolism. In this disorder, the hormones aldosterone and cortisol are insufficiently secreted from the suprarenal glands. For this reason, Addison’s disease is also referred to as primary adrenal insufficiency [1]. Hormonal treatment is the standard approach to this disease to mimic the natural function of the deficient adrenal hormone.

Patient Information

Definition

Addison’s disease is a serious disorder that occurs when the adrenal glands is unable to produce sufficient mineralocorticoid and glucocorticoid hormones needed for normal functioning.

Cause

The most common cause of Addison’s disease is idiopathic autoimmune adrenal atrophy. The other causes include granuloma, tuberculosis, histoplasmosis, amyloidosis, inflammatory necrosis, hemorrhage and tumors.

Symptoms

Patients usually present with anorexia, nausea and vomiting. Muscular fatigue and weakness are fairly common with hyperpigmentation of the skin. Signs of hypotension and hypoglycemia may also be observable.

Diagnosis

Diagnostics for Addison’s disease starts with blood test of electrolytes and hormones. ACTH stimulation tests and Insulin-induced hypoglycemia tests, imaging studies with CT scan and MRI may follow.

Treatment and follow-up

The cornerstone in the treatment of Addison’s disease is the active hormonal replacement of the deficient hormones. Glucocorticoids are replaced with oral and injectable steroids, mineralocorticoids are replace by fludrocortisone. Androgen replacement therapy for afflicted women can allay the signs of androgen deficiency.

References

Article

  1. Addison T. On the Constitutional and Local Effects of Disease of the Supra-renal Capsules. London, UK: Samuel Highley; 1855.
  2. White K, Arlt W. Adrenal crisis in treated Addison's disease: a predictable but under-managed event. Eur J Endocrinol. Jan 2010; 162(1):115-20.
  3. Kyriazopoulou V. Glucocorticoid replacement therapy in patients with Addison's disease. Expert Opin Pharmacother. Apr 2007; 8(6):725-9.
  4. Bergthorsdottir R, Leonsson-Zachrisson M, Oden A, et al. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab. Dec 2006; 91(12):4849-53.
  5. McBrien DJ. Steatorrhea in Addison's disease. Lancet. 1963; Vol I: 25-6.
  6. Barnett AH, Espiner EA, Donald RA. Patients presenting with Addison's disease need not be pigmented.Postgrad Med J. Nov 1982; 58(685):690-2.
  7. McBrien DJ. Steatorrhea in Addison's disease. Lancet. 1963; Vol I: 25-6.
  8. Kyriazopoulou V, Parparousi O, Vagenakis AG. Rifampicin-induced adrenal crisis in addisonian patients receiving corticosteroid replacement therapy. J Clin Endocrinol Metab. Dec 1984; 59(6):1204-6.
  9. Likhari T, Magzoub S, Griffiths MJ, et al. Screening for Addison's disease in patients with type 1 diabetes mellitus and recurrent hypoglycaemia. Postgrad Med J. Jun 2007; 83(980):420-1.
  10. Ma ES, Yang ZG, Li Y, et al. Tuberculous Addison's disease: morphological and quantitative evaluation with multidetector-row CT. Eur J Radiol. Jun 2007; 62(3):352-8.

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Last updated: 2018-06-22 07:49