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The Canadian Veterinary Journal = La... Jan 2010Hypoadrenocorticism (Addison's disease) has been referred to as "the great pretender," due to its ability to mimic other common diseases in the dog and thereby represent... (Review)
Review
Hypoadrenocorticism (Addison's disease) has been referred to as "the great pretender," due to its ability to mimic other common diseases in the dog and thereby represent a diagnostic challenge. Naturally occurring hypoadrenocorticism is an uncommon canine disease. Young, female dogs are overrepresented. Hypoadrenocorticism typically results from immune-mediated destruction of all adrenocortical layers, resulting in deficiencies of min-eralocorticoids (aldosterone) and glucocorticoids (cortisol). A small number of dogs suffer from glucocorticoid deficiency only. Dogs suffering from hypoadrenocorticism may present in a variety of conditions, from a mildly ill dog to a shocky and recumbent dog. This review discusses etiology, pathophysiology, history, physical examination findings, and diagnostic findings in the Addisonian patient. A follow-up article (Part II) will discuss the definitive diagnosis and management strategies for these patients.
Topics: Addison Disease; Age Factors; Aldosterone; Animals; Dog Diseases; Dogs; Female; Hydrocortisone; Male; Physical Examination; Sex Factors
PubMed: 20357943
DOI: No ID Found -
BMC Cardiovascular Disorders Jan 2023Addison's disease which is due to dysfunction of the adrenal gland, with abnormal secretion of glucocorticoids and mineralocorticoids, is rare. By inducing inflammation... (Review)
Review
BACKGROUND
Addison's disease which is due to dysfunction of the adrenal gland, with abnormal secretion of glucocorticoids and mineralocorticoids, is rare. By inducing inflammation and disorders of water and electrolyte metabolism, Addison's disease may accelerate progression of co-existed cardiovascular diseases. Addison's disease combined with cardiovascular disease is infrequent, only 10 cases in the literature.
CASE PRESENTATION
We reported a 51-year-old male patient with unstable angina pectoris and hypotension. Changes on coronary angiography within 2 years suggested rapid progression of coronary artery disease in a patient with low cardiovascular risk. An additional clue of skin hyperpigmentation, fatigue and further examination confirmed the diagnosis of Addison's disease caused by adrenal tuberculosis. After hormone replacement treatment, the frequency and severity of the angina pectoris were alleviated significantly, as were hypotension, hyperpigmentation and fatigue.
CONCLUSIONS
The combination of Addison's disease and coronary artery disease in one patient is rare. Addison's disease can induce inflammation and disorders of water and electrolyte metabolism, which may further accelerate the course of coronary artery disease. Meanwhile, the hypotension in Addison's disease may affect the coronary blood flow, which may result in an increased susceptibility to unstable angina in the presence of coronary stenosis. So, we should analyze comprehensively if the coronary artery disease progress rapidly.
Topics: Male; Humans; Middle Aged; Addison Disease; Coronary Artery Disease; Inflammation; Fatigue; Hyperpigmentation; Hypotension
PubMed: 36709280
DOI: 10.1186/s12872-023-03079-0 -
The Journal of Clinical Endocrinology... May 2022Autoimmune thyroid disease is the most common endocrine comorbidity in autoimmune Addison disease (AAD), but detailed investigations of prevalence and clinical course...
CONTEXT
Autoimmune thyroid disease is the most common endocrine comorbidity in autoimmune Addison disease (AAD), but detailed investigations of prevalence and clinical course are lacking.
OBJECTIVE
This work aimed to provide comprehensive epidemiological and clinical data on autoimmune thyroid disorders in AAD.
METHODS
A nationwide registry-based study including 442 patients with AAD and autoimmune thyroid disease were identified through the Norwegian National Registry of Autoimmune Diseases.
RESULTS
Of 912 registered AAD patients, 442 (48%) were diagnosed with autoimmune thyroid disease. A total of 380 (42%) had autoimmune hypothyroidism. Of the 203 with available thyroid function tests at time of diagnosis, 20% had overt hypothyroidism, 73% had subclinical hypothyroidism, and 7% had thyroid levels in the normal range. Negative thyroid peroxidase antibodies was found in 32%. Ninety-eight percent were treated with levothyroxine, 5% with combination therapy with liothyronine or thyroid extracts, and 1% were observed without treatment. Seventy-eight patients (9%) were diagnosed with Graves disease (GD), of whom 16 (21%) were diagnosed with autoimmune hypothyroidism either before onset or after remission of GD. At the end of follow-up, 33% had normal thyroid hormone levels without antithyroid-drugs or levothyroxine treatment. The remaining had either active disease (5%), had undergone ablative treatment (41%), or had developed autoimmune hypothyroidism (21%).
CONCLUSION
The true prevalence of hypothyroidism in AAD is lower than reported in the current literature. Careful consideration of the indication to start thyroxin therapy is warranted. Long-term remission rates in GD patients with AAD are comparable to recent reports on long-term follow-up of patients without AAD.
Topics: Addison Disease; Graves Disease; Hashimoto Disease; Humans; Hypothyroidism; Thyroid Hormones; Thyroiditis, Autoimmune; Thyroxine
PubMed: 35226748
DOI: 10.1210/clinem/dgac089 -
European Journal of Endocrinology Feb 2021Over the last 10 years, evidence has accumulated that autoimmune Addison's disease (AAD) is a heterogeneous disease. Residual adrenal function, characterised by... (Review)
Review
Over the last 10 years, evidence has accumulated that autoimmune Addison's disease (AAD) is a heterogeneous disease. Residual adrenal function, characterised by persistent secretion of cortisol, other glucocorticoids and mineralocorticoids is present in around 30% of patients with established AAD, and appears commoner in men. This persistent steroidogenesis is present in some patients with AAD for more than 20 years, but it is commoner in people with shorter disease duration. The clinical significance of residual adrenal function is not fully clear at the moment, but as it signifies an intact adrenocortical stem cell population, it opens up the possibility of regeneration of adrenal steroidogenesis and improvement in adrenal failure for some patients.
Topics: Addison Disease; Adrenal Glands; Autoimmunity; Endocrine System Diseases; Female; Humans; Male
PubMed: 33306039
DOI: 10.1530/EJE-20-0894 -
Archivum Immunologiae Et Therapiae... Jun 2017Mitochondrial antiviral signaling (MAVS) protein is an intracellular adaptor molecule, downstream of viral sensors, retinoid acid-inducible gene I (RIG-I)-like receptors...
Mitochondrial antiviral signaling (MAVS) protein is an intracellular adaptor molecule, downstream of viral sensors, retinoid acid-inducible gene I (RIG-I)-like receptors (RLRs). Impaired antiviral cell signaling might contribute to autoimmunity. Studies have recently shown variations in genes encoding RLRs as risk factors for autoimmune diseases. We investigated whether MAVS coding polymorphisms are associated with Addison's disease (AD) and type 1 diabetes (T1D) in Polish population. We genotyped 140 AD, 532 T1D patients and 600 healthy controls for MAVS rs17857295, rs7262903, rs45437096 and rs7269320. Genotyping was performed by TaqMan assays. Distribution of the MAVS genotypes and alleles did not reveal significant differences between patients and controls (p > 0.05). This analysis did not indicate the association of the MAVS locus with susceptibility to AD and T1D.
Topics: Adaptor Proteins, Signal Transducing; Addison Disease; Adult; DNA Mutational Analysis; Diabetes Mellitus, Type 1; Female; Gene Frequency; Genetic Predisposition to Disease; Genotype; Humans; Male; Middle Aged; Poland; Polymorphism, Single Nucleotide; Risk Factors
PubMed: 27652379
DOI: 10.1007/s00005-016-0426-6 -
BMC Musculoskeletal Disorders Oct 2022To determine the relaxation times of the sodium nucleus, and to investigate the repeatability of quantitative, in vivo TSC measurements using sodium magnetic resonance...
Repeatability assessment of sodium (Na) MRI at 7.0 T in healthy human calf muscle and preliminary results on tissue sodium concentrations in subjects with Addison's disease.
OBJECTIVES
To determine the relaxation times of the sodium nucleus, and to investigate the repeatability of quantitative, in vivo TSC measurements using sodium magnetic resonance imaging (Na-MRI) in human skeletal muscle and explore the discriminatory value of the method by comparing TSCs between healthy subjects and patients with Addison's disease.
MATERIALS AND METHODS
In this prospective study, ten healthy subjects and five patients with Addison's disease were involved. Na-MRI data sets were acquired using a density-adapted, three-dimensional radial projection reconstruction pulse sequence (DA-3DPR) with a modification for the relaxation times measurements. Differences in TSC between muscle groups and between healthy participants were analysed using a nonparametric Friedman ANOVA test. An interclass correlation coefficient (ICC) was used as the repeatability index. Wilcoxon rank sum test was used for evaluation of differences in TSC between study participants.
RESULTS
The mean T in the gastrocnemius medialis (GM), the tibialis anterior (TA), and the soleus (S) was 25.9 ± 2.0 ms, 27.6 ± 2.0 ms, and 28.2 ± 2.0 ms, respectively. The mean short component of T, T were GM: 3.6 ± 2.0 ms; TA: 3.2 ± 0.5 ms; and S: 3.0 ± 1.0 ms, and the mean long component of T, T, were GM: 12.9 ± 0.9 ms; TA: 12.8 ± 0.7 ms; and S: 12.9 ± 2.0 ms, respectively. In healthy volunteers, TSC values in the GM were 19.9 ±0.1 mmol/L, 13.8 ±0.2 mmol/L in TA, and 12.6 ± 0.2 mmol/L in S, and were significantly different (p = 0.0005). The ICCs for GM, TA and S were 0.784, 0.818, 0.807, respectively. In patients with Addison's disease, TSC in GC, TA, and S were 10.2 ± 1.0 mmol/L, 8.4 ± 0.6 mmol/L, and 7.2 ± 0.1 mmol/L, respectively.
CONCLUSIONS
TSC quantification in a healthy subject's calf at 7.0 T is reliable; the technique is able to distinguish sodium level differences between muscles and between healthy subjects and Addison's disease patients.
Topics: Humans; Addison Disease; Magnetic Resonance Imaging; Muscle, Skeletal; Prospective Studies; Sodium
PubMed: 36266679
DOI: 10.1186/s12891-022-05879-5 -
Clinical and Experimental Immunology Jun 2014Steroidogenic enzyme autoantibodies (SEAbs) are frequently present and are markers of autoimmune premature ovarian failure (POF) in females with autoimmune Addison's...
Steroidogenic enzyme autoantibodies (SEAbs) are frequently present and are markers of autoimmune premature ovarian failure (POF) in females with autoimmune Addison's disease (AAD). The prevalence and significance of SEAbs in males with AAD have not yet been defined. We studied the prevalence of SEAbs in a large cohort of males with AAD and assessed the relationship between SEAbs positivity and testicular function. A total of 154 males with AAD (mean age 34 years) were studied. SEAbs included autoantibodies to steroid-producing cells (StCA), detected by immunofluorescence, and steroid 17α-hydroxylase (17α-OHAbs) and side chain cleavage enzyme (SCCAbs) measured by immunoprecipitation assays. Gonadal function was evaluated by measuring follicle-stimulating hormone (FSH), luteinizing hormone (LH), total testosterone (TT), sex hormone-binding globulin (SHGB), anti-müllerian hormone (AMH) and inhibin-B (I-B). Twenty-six males, 10 SEAbs((+)) and 16 SEAbs((-)), were followed-up for a mean period of 7·6 years to assess the behaviour of SEAbs and testicular function. SEAbs were found in 24·7% of males with AAD, with the highest frequency in patients with autoimmune polyendocrine syndrome type 1 (APS-1). The levels of reproductive hormones in 30 SEAbs((+)) males were in the normal range according to age and were not significantly different compared to 55 SEAbs((-)) males (P > 0·05). During follow-up, both SEAbs((+)) and SEAbs((-)) patients maintained normal testicular function. SEAbs were found with high frequency in males with AAD; however, they were not associated with testicular failure. This study suggests that the diagnostic value of SEAbs in males with AAD differs compared to females, and this may be related to the immunoprivileged status of the testis.
Topics: Addison Disease; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Child; Child, Preschool; Follow-Up Studies; Gonadal Hormones; Humans; Male; Middle Aged; Steroids; Testis; Young Adult
PubMed: 24666377
DOI: 10.1111/cei.12303 -
The Journal of International Medical... Dec 2021We report the case of a 47-year-old male patient with pigmentation of the head, face and hands, who was initially diagnosed as having primary adrenal insufficiency... (Review)
Review
We report the case of a 47-year-old male patient with pigmentation of the head, face and hands, who was initially diagnosed as having primary adrenal insufficiency (Addison's disease). Laboratory testing, imaging and physical examination revealed subclinical hypothyroidism, high circulating prolactin and oestradiol concentrations, gynaecomastia, lymphadenopathy, splenomegaly and weakness of both lower limbs. These findings led us to consider whether a single or multiple diseases were present in this patient. Indeed, Addison's disease can represent one aspect of a wider systemic disease. Therefore, we performed further examinations, and found high serum M protein (5.1%) and vascular endothelial growth factor [1005.30 pg/mL (normal range 0 to 142 pg/mL)] concentrations. As a consequence, we diagnosed polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. Consequently, when a single disease cannot fully explain the multiple symptoms and signs of one patient, clinicians should consider the possibility of the presence of a wider syndrome and undertake more detailed diagnostic testing.
Topics: Addison Disease; Humans; Male; Middle Aged; POEMS Syndrome; Splenomegaly; Vascular Endothelial Growth Factor A
PubMed: 34932395
DOI: 10.1177/03000605211066239 -
Proceedings of the Royal Society of... May 1974
Topics: Addison Disease; Adult; Autoantibodies; Blood Cell Count; Diarrhea; Exophthalmos; Female; Fluorescent Antibody Technique; Humans; Hyperthyroidism; Thyroid Function Tests; Vomiting
PubMed: 4600590
DOI: No ID Found -
Journal of Clinical Research in... Aug 2022Primary adrenal insufficiency (PAI) is a rare condition in children, and is potentially life-threatening. The most common cause is congenital adrenal hyperplasia, and...
OBJECTIVE
Primary adrenal insufficiency (PAI) is a rare condition in children, and is potentially life-threatening. The most common cause is congenital adrenal hyperplasia, and autoimmune etiology is the most frequent acquired cause in this age group. Symptoms are usually non-specific and, when suspected, investigation should include adrenocorticotropin hormone (ACTH) and morning serum cortisol measurement and, in some cases, a cosyntropin test to confirm the diagnosis. Prompt treatment is essential to prevent an adverse outcome.
METHODS
We retrospectively collected clinical and laboratory data from adrenal insufficiency due to autoimmune adrenalitis, observed from 2015 to 2020 in a pediatric endocrinology department of a tertiary care hospital.
RESULTS
Eight patients were identified, seven males and one female, with age at diagnosis between 14 and 17 years. The symptoms at presentation ranged from non-specific symptoms, such as chronic fatigue and weight loss, to a severe presentation, with altered mental status and seizures. The median duration of symptoms was 4.5 months. The diagnosis was confirmed by serum cortisol and plasma ACTH measurement and all were confirmed to have autoimmune etiology (positive anti-adrenal antibodies). At diagnosis, the most common laboratory abnormality was hyponatremia. All patients were treated with hydrocortisone and fludrocortisone. One patient presented with evidence of type 2 autoimmune polyglandular syndrome.
CONCLUSION
PAI is a rare condition in the pediatric age group. Due to non-specific symptoms, a high index of suspicion is necessary to establish a prompt diagnosis. Once an autoimmune etiology is confirmed, it is important to initiate the appropriate treatment and search for signs and symptoms of other autoimmune diseases during follow-up.
Topics: Addison Disease; Adolescent; Adrenal Insufficiency; Adrenocorticotropic Hormone; Female; Humans; Hydrocortisone; Male; Retrospective Studies
PubMed: 35633647
DOI: 10.4274/jcrpe.galenos.2022.2021-11-9