Presentation
[…] it is commonly overlooked due the vagueness in its presentation. [pedclerk.bsd.uchicago.edu]
Case presentation A 37-year-old male soldier presented in 1997 with classical Addison's disease. He presented with fatigue, nausea, vomiting, weight loss, malaise and dizziness. [edmcasereports.com]
Possible presentations The most common is the child with known adrenal insufficiency who has an intercurrent illness. [kidshealthwa.com]
Presentation in part depends on the rapidity of loss of adrenal function: Acute : presentation may be as a crisis precipitated by infection, surgery or trauma. [patient.info]
Hypoglycemia is more commonly seen than in primary AI Pale skin without significant anemia Prepubertal growth deficiency, delayed puberty May present with clinical manifestations of pituitary or hypothalamic tumor, eg diabetes insipidus (DI) Secondary [specialty.mims.com]
Entire Body System
- Hypothermia
Haemochromatosis Congential causes Adrenal dysgenesis Adrenoleucodystrophy Impaired steroidogenesis Empty sella syndrome Autoimmune destruction Addisons's disease Traumatic destruction Trauma is a major cause of adrenal insufficiency Environmental factors Hypothermia [derangedphysiology.com]
As cortisol levels remain low, patients may develop severe hypotension, vascular collapse, acute renal failure, and hypothermia. [pharmacytimes.com]
- Asymptomatic
The patient remains asymptomatic and well. The patient also has an emergency supply of hydrocortisone for both oral and i.m. administration in the event of intercurrent illness. [edmcasereports.com]
- Severe Pain
During an addisonian crisis, affected individuals may develop a sudden loss of strength; severe pain in the lower back, abdomen or legs; vomiting and diarrhea potentially causing dehydration; and low blood pressure and loss of consciousness. [rarediseases.org]
Gastrointestinal
- Vomiting
If vomiting or diarrhoea treat as below. [kidshealthwa.com]
In major stress (temperatures above 38 degrees C and/or vomiting), the hydrocortisone dose should be increase to three to four times the normal replacement. [pedclerk.bsd.uchicago.edu]
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. [checkorphan.org]
DI) Secondary hypothyroidism Decreased libido and potency, amenorrhea Visual symptoms, headache Primary, Secondary & Tertiary Adrenal Insufficiency (AI): Fatigue Fever Weakness Salt craving Orthostatic hypotension, dizziness, hypovolemic shock Nausea/vomiting [specialty.mims.com]
EMERGENCY If you have been diagnosed with Addison’s disease and experience symptoms of severe lethargy, nausea, vomiting, dizziness, or dehydration, get immediate medical assistance. These may be indications of adrenal crisis. [healthcommunities.com]
- Nausea
[…] insipidus (DI) Secondary hypothyroidism Decreased libido and potency, amenorrhea Visual symptoms, headache Primary, Secondary & Tertiary Adrenal Insufficiency (AI): Fatigue Fever Weakness Salt craving Orthostatic hypotension, dizziness, hypovolemic shock Nausea [specialty.mims.com]
EMERGENCY If you have been diagnosed with Addison’s disease and experience symptoms of severe lethargy, nausea, vomiting, dizziness, or dehydration, get immediate medical assistance. These may be indications of adrenal crisis. [healthcommunities.com]
Metabolic decompensation Fatigue, anorexia, nausea, hyperpigmentation Increased ACTH and decreased 8 a.m. cortisol levels; high clinical suspicion needed for diagnosis 5. [aafp.org]
Common non-specific symptoms include fatigue and weakness as well as nausea, vomiting, anorexia, and weight loss. Deficiencies in cortisol synthesis typically lead to hypoglycemia. [pathwaymedicine.org]
[…] weakness Fatigue Dizziness Rapid pulse Dark skin (first noted on hands and face) Black freckles Bluish-black discoloration around the nipples, mouth, rectum, scrotum, or vagina Weight loss Dehydration Loss of appetite Intense salt craving Muscle aches Nausea [stanfordchildrens.org]
Cardiovascular
- Hypotension
Tertiary Adrenal Insufficiency (AI): Manifestations usually begin in the first 48 hours after steroid medication has been discontinued Similar to primary AI except that hyperpigmentation and dehydration are absent; Gastrointestinal (GI) symptoms and hypotension [specialty.mims.com]
refractory shock, poor response to inotropes/pressors fever confusion CHRONIC ADRENAL INSUFFICIENCY GENERAL: weight loss, arthralgia, myalgia CNS: fatigue, anorexia, mood change CVS: postural hypotension, syncope, salt craving SKIN: pigmentation, vitiligo [lifeinthefastlane.com]
Hypotension. Postural hypotension. Other situations which should provoke consideration of Addison's disease include: People with hypothyroidism in whom symptoms get worse when thyroxine treatment is commenced. [patient.info]
Measurement of plasma renin levels should be considered first in patients with continued salt craving or hypotension. [themedicalbiochemistrypage.org]
Other symptoms include vertigo, hypotension, depression, salt craving, and vitiligo (depigmented patches of skin). [pharmacytimes.com]
- Orthostatic Hypotension
Although there are classic clinical signs (eg, fatigue, orthostatic hypotension, hyperpigmentation, hyponatremia, hyperkalemia, and hypoglycemia) of adrenal insufficiency, its early clinical presentation is most commonly vague and undefined, requiring [ncbi.nlm.nih.gov]
[…] of pituitary or hypothalamic tumor, eg diabetes insipidus (DI) Secondary hypothyroidism Decreased libido and potency, amenorrhea Visual symptoms, headache Primary, Secondary & Tertiary Adrenal Insufficiency (AI): Fatigue Fever Weakness Salt craving Orthostatic [specialty.mims.com]
On physical examination, the following 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 [checkorphan.org]
Additional signs and symptoms include weakness, tiredness, dizziness, low blood pressure that falls further when standing ( orthostatic hypotension ), cardiovascular collapse, muscle aches, nausea, vomiting, and diarrhea. [en.wikipedia.org]
Musculoskeletal
- Muscle Weakness
Other common symptoms of adrenal hypofunction include fatigue that keeps getting worse, weight loss, muscle weakness, loss of appetite, headache and irregular or no periods in women. Causes Adrenal hypofunction has more than one cause. [livestrong.com]
weakness, sore throat, or infection Report tiredness, stomach pain, weakness, high/low blood sugar Do not d/c abruptly if taking chronically. [quizlet.com]
Symptoms may include: Muscle weakness Fatigue Dizziness Rapid pulse Dark skin (first noted on hands and face) Black freckles Bluish-black discoloration around the nipples, mouth, rectum, scrotum, or vagina Weight loss Dehydration Loss of appetite Intense [stanfordchildrens.org]
Symptoms of Adrenal Insufficiency Progressive fatigue and muscle weakness Nausea and vomiting Decreased appetite and weight loss Increased pigmentation of skin and mucous membranes (dark freckles, uneven patches of darkened skin, or unusually persistent [healthcommunities.com]
Secondary Adrenal Insufficiency (↓ACTH Levels) Pituitary disorders Hypothalamic disorders Withdrawal from pharmacological doses of corticosteroids Assessment Clinical features Shock Hypoglycaemia (confusion, coma) Muscle weakness Lethargy Vomiting Syncope [kidshealthwa.com]
Skin
- Hyperpigmentation
Fatigue Diarrhea Weakness Lethargy Fever Confusion Coma Anorexia Nausea/vomiting Metabolic acidosis Abdominal pain Patient with underlying chronic adrenal insufficiency (AI) who presents in crisis may show common symptoms of adrenal insufficiency (AI) Hyperpigmentation [specialty.mims.com]
It presents with hyperpigmentation, weakness, hypoglycemia, and seizures. [pedclerk.bsd.uchicago.edu]
Although there are classic clinical signs (eg, fatigue, orthostatic hypotension, hyperpigmentation, hyponatremia, hyperkalemia, and hypoglycemia) of adrenal insufficiency, its early clinical presentation is most commonly vague and undefined, requiring [ncbi.nlm.nih.gov]
In addition, Addison disease is associated with hyperpigmentation. [themedicalbiochemistrypage.org]
Metabolic decompensation Fatigue, anorexia, nausea, hyperpigmentation Increased ACTH and decreased 8 a.m. cortisol levels; high clinical suspicion needed for diagnosis 5. [aafp.org]
- Freckles
Symptoms may include: Muscle weakness Fatigue Dizziness Rapid pulse Dark skin (first noted on hands and face) Black freckles Bluish-black discoloration around the nipples, mouth, rectum, scrotum, or vagina Weight loss Dehydration Loss of appetite Intense [stanfordchildrens.org]
Symptoms may include: Weakness Fatigue Dizziness Dark skin Black freckles Bluish-black discoloration around the nipples, mouth, rectum, scrotum, or vagina Weight loss Dehydration Lack of appetite Muscle aches Nausea Vomiting Diarrhea Intolerance to cold [jerseyshoreuniversitymedicalcenter.com]
Symptoms of Adrenal Insufficiency Progressive fatigue and muscle weakness Nausea and vomiting Decreased appetite and weight loss Increased pigmentation of skin and mucous membranes (dark freckles, uneven patches of darkened skin, or unusually persistent [healthcommunities.com]
Black freckles may develop over the forehead, face, and shoulders, and a bluish black discoloration may develop around the nipples, lips, mouth, rectum, scrotum, or vagina. [msdmanuals.com]
Black freckles may develop on the forehead, face or shoulders in some cases. A variety of gastrointestinal symptoms may be present including nausea, vomiting, and abdominal pain. Diarrhea is less common, but may also occur. [rarediseases.org]
Neurologic
- Stroke
In addition, exposure to chronically increased levels of glucocorticoids can lead to an increased rate of diabetes, hypertension, high lipids, osteoporosis, doubling of cardiovascular risk and earlier death from heart attack, stroke, hip fracture etc. [csrf.net]
Workup
Autoimmune workup If the diagnosis of Addison’s appears to be a part of a larger autoimmune pathology, one should include a workup for other endocrine gland dysfunctions. [pedclerk.bsd.uchicago.edu]
^ Addison Disease~workup at eMedicine Further reading [ edit ] Bornstein, SR; Allolio, B; Arlt, W; Barthel, A; Don-Wauchope, A; Hammer, GD; Husebye, ES; Merke, DP; Murad, MH; Stratakis, CA; Torpy, DJ (February 2016). [en.wikipedia.org]
Further workup of anemia revealed hypochromic cells with anysocytosis on peripheral blood smear, serum iron 20 µg/dL, total iron binding capacity 108 µg/dL and iron saturation 22%. [asean-endocrinejournal.org]
Serum
- Hypoglycemia
Insulin-Induced Hypoglycemia Test: The insulin-induced hypoglycemia test is used to determine how the hypothalamus, pituitary and adrenal glands respond to stress. [ucsfhealth.org]
Although there are classic clinical signs (eg, fatigue, orthostatic hypotension, hyperpigmentation, hyponatremia, hyperkalemia, and hypoglycemia) of adrenal insufficiency, its early clinical presentation is most commonly vague and undefined, requiring [ncbi.nlm.nih.gov]
D40% was given with each episode of hypoglycemia, and fluid challenge with NaCl 0.9% following hypotension. [asean-endocrinejournal.org]
It presents with hyperpigmentation, weakness, hypoglycemia, and seizures. [pedclerk.bsd.uchicago.edu]
- Hyponatremia
[…] deficiency) due to salt wasting from mineralocorticoid deficiency Secondary disease: Hyponatremia alone. [openanesthesia.org]
(AI): Manifestations usually begin in the first 48 hours after steroid medication has been discontinued Similar to primary AI except that hyperpigmentation and dehydration are absent; Gastrointestinal (GI) symptoms and hypotension are less prominent Hyponatremia [specialty.mims.com]
Hyperkalemia and hyponatremia with a normal anion gap metabolic acidosis will be a feature of mineralocorticid deficiency, and will resemble the wanton use of spironolactone. [derangedphysiology.com]
Hyponatremia and hyperkalemia may also result form chronic renal insufficiency. Diagnosis Addison’s requires a high degree of suspicion and investigation. [pedclerk.bsd.uchicago.edu]
Although there are classic clinical signs (eg, fatigue, orthostatic hypotension, hyperpigmentation, hyponatremia, hyperkalemia, and hypoglycemia) of adrenal insufficiency, its early clinical presentation is most commonly vague and undefined, requiring [ncbi.nlm.nih.gov]
- Cortisol Decreased
The decrease in function in a specific adrenal steroidogenic enzyme leads to a decrease in cortisol production. [comprehensivephysiology.com]
- Cortisol Decreased
The decrease in function in a specific adrenal steroidogenic enzyme leads to a decrease in cortisol production. [comprehensivephysiology.com]
Treatment
Treatment may also include taking a medication that helps restore the body's level of sodium and potassium. [stanfordchildrens.org]
Treatment may also include taking fludrocortisone, a drug that helps restore the body's level of sodium and potassium. [jerseyshoreuniversitymedicalcenter.com]
They may also recommend medications, as well as radiation treatment, if your glands are producing too much of a hormone. Surgery is another treatment option for certain adrenal disorders. [healthline.com]
Hydrocortisone dose was therefore completely withdrawn and the patient remains well after 4 months of treatment. After treatment, his 0900 h ACTH level was initially elevated at 86 ng/l but has now normalised at 13 ng/l. [edmcasereports.com]
Long-term treatment with steroids leads to atrophy of the adrenal cortex; recovery of adrenal function may take months after steroid treatment is stopped. [healthcommunities.com]
Prognosis
[…] adrenalitis): enlargement of both adrenal glands, with necrotic centers and peripheral enhancing rims chronic : both adrenal glands appear small and atrophic, associated with calcifications ( adrenal calcification ) in granulomatous adrenalitis Treatment and prognosis [radiopaedia.org]
The prognosis for any patient with adrenal insufficiency will depend on the underlying cause. In those patients in whom the prognosis is not affected by the underlying pathology, replacement therapy should result in a return to health. [patient.info]
Prognosis - Addison's disease With proper medication, especially hormone replacement therapy, patients can expect to live relatively normal lives. Treatment - Addison's disease Corticosteroids to replace cortisols not secreted by the adrenal glands. [checkorphan.org]
Etiology
Overview Chronic Adrenocortical Insufficiency can be caused by a variety of etiologies. [pathwaymedicine.org]
Abstract Since Addison's classic and comprehensive description of the disease bearing his name, 1, 2 accepted etiologic factors have included tuberculosis and cytotoxic contracted adrenals (atrophy) almost exclusively. [annals.org]
Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common etiology overall. [radiopaedia.org]
[…] or equivalent for ≥4 weeks) or chronic glucocorticoid cream or inhaler application or long-lasting glucocorticoid injections into joints Tertiary AI is the inability of the hypothalamus to produce sufficient amount of corticotropin releasing hormone Etiology [specialty.mims.com]
Epidemiology
Epidemiology [ 1, 2 ] Primary insufficiency - this is a relatively rare condition. The annual incidence is about 1 in 10,000 people, with a prevalence in the UK of about 8,400. Across Europe, prevalence is estimated as 93-144 per million. [patient.info]
Pathophysiology
Study Group of the Italian Society of Endocrinology on the Pathophysiology of the Hypothalamic‐Pituitary‐Adrenal Axis. J Clin Endocrinol Metab 84: 440‐448, 1999. 116. [comprehensivephysiology.com]
The pathophysiology of this is not yet clear. It involves reduction in the production of glucocorticoids as well as reduced effect. [patient.info]
[…] the body, especially lung ), hemorrhage (e.g. in Waterhouse–Friderichsen syndrome or antiphospholipid syndrome ), particular infections ( tuberculosis, histoplasmosis, coccidioidomycosis ), or the deposition of abnormal protein in amyloidosis. [22] Pathophysiology [en.wikipedia.org]
Addison disease can be part of the autoimmune polyglandular syndromes (type 1 and 2), or it may present as an isolated disorder. 1 This article focuses on the diagnosis and treatment of Addison disease as an isolated disorder, with a focus on the pathophysiology [aafp.org]
Prevention
[…] of HIV HIV Transmission and Prevention in Specific Populations Systematic Review of HIV Behavioral Prevention Research in African Americans Lynae A. [hivinsite.ucsf.edu]
There is no way to prevent Addison’s disease. [healthcommunities.com]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
Preincubation of the microsomes with antisera to the 17 alpha-hydroxylase P-450 isozyme (P-450(17 alpha,lyase)) decreased 17 alpha-hydroxylase but not 21-hydroxylase activity and prevented the degradation of P-450 by 7 alpha-thio-SL. [molpharm.aspetjournals.org]
[…] depress the immune response, especially cell-mediated immune responses. [ Discussion of mechanism ] For this reason glucocorticoids are widely used in therapy: to reduce the inflammatory destruction of rheumatoid arthritis and other autoimmune diseases to prevent [biology-pages.info]