Addison's disease is a rare disease caused by insufficient production of glucocorticoids, mineralocorticoids, and androgens in the adrenal cortex. It occurs more frequently in women and develops most often between the ages of 30 and 50. About two-thirds of patients with Addison's disease may develop other autoimmune disorders in the context of autoimmune polyglandular syndrome (APS), including autoimmune thyroid disease (ATD), autoimmune gastritis, type 1 diabetes, premature ovarian failure (POF), vitiligo, or celiac disease. We reported a case of 51-year-old woman with complaints of weakness, nausea, vomiting, weight loss, frequent bowel movements, and hyperpigmentation. Laboratory examinations showed decreased level of morning cortisol, increased ACTH, increased FT4, decreased TSH, increased thyrotropin receptor antibody (TRab), and positive glutamic acid decarboxylase (GAD) 65. Patient was diagnosed with Addison's disease accompanied by autoimmune thyroid disease-causing' disease and type 1 diabetes mellitus, leading to autoimmune polyglandular syndrome (APS) type 2. After being given steroid, insulin, and anti-thyroid drugs therapy, the patient's condition improved.
Addison Disease

PURPOSE: Autoimmunity is an alternative etiology of gastric inflammation, the initiating event in the gastric carcinogenic cascade. This mechanism may be an increasingly important cause of gastric cancer with the waning prevalence of its primary etiologic factor, chronic Helicobacter pylori infection. MATERIALS AND METHODS: PubMed and EMBASE were searched up to September 2018. Autoimmunity and 96 specific manifestations were considered for associations with gastric cancer risk. Random effects analysis was used to calculate pooled relative risk estimates (RR) and 95% confidence intervals (CI). RESULTS: We found a total of 52 observational studies representing 30 different autoimmune diseases. Overall, the presence of an autoimmune condition was associated with a gastric cancer pooled RR of 1.37 (95% CI, 1.24 to 1.52). Among the 24 autoimmune conditions with two or more independent reports, nine were significantly associated with increased gastric cancer risk: dermatomyositis (RR, 3.69; 95% CI, 1.74 to 7.79), pernicious anemia (RR, 2.84; 95% CI, 2.30 to 3.50), Addison disease (RR, 2.11; 95% CI, 1.26 to 3.53), dermatitis herpetiformis (RR, 1.74; 95% CI, 1.02 to 2.97; n=3), IgG4-related disease (RR, 1.69; 95% CI, 1.00 to 2.87), primary biliary cirrhosis (RR, 1.64; 95% CI, 1.13 to 2.37), diabetes mellitus type 1 (RR, 1.41; 95% CI, 1.20 to 1.67), systemic lupus erythematosus (RR, 1.37; 95% CI, 1.01 to 1.84), and Graves disease (RR, 1.27; 95% CI, 1.06 to 1.52). CONCLUSION: Our analysis documents the wide range of autoimmune diseases associated with gastric cancer. These associations may reflect unreported links between these conditions and autoimmune gastritis. Further studies are warranted to investigate potential causal mechanisms.
Addison Disease ; Anemia, Pernicious ; Autoimmune Diseases ; Autoimmunity ; Dermatitis Herpetiformis ; Dermatomyositis ; Diabetes Mellitus ; Epidemiology ; Gastritis ; Graves Disease ; Helicobacter pylori ; Inflammation ; Liver Cirrhosis, Biliary ; Lupus Erythematosus, Systemic ; Prevalence ; Stomach Neoplasms

Vitiligo is a multifactorial disorder. Neural, biochemical, and autoimmune mechanisms have been hypothetically suggested as etiopathological contributors to this condition. Autoimmunity focuses primarily on genetic factors and the association between vitiligo and other autoimmune disorders including autoimmune thyroid disease, rheumatoid arthritis, psoriasis, type 1 diabetes, pernicious anemia, and Addison's disease. We describe a 35-year-old man with systemic lupus erythematosus who developed concurrent vitiligo and discoid lupus erythematosus suggesting the possible autoimmune association between these 2 different diseases.
Addison Disease ; Adult ; Anemia, Pernicious ; Arthritis, Rheumatoid ; Autoimmunity ; Humans ; Lupus Erythematosus, Discoid ; Lupus Erythematosus, Systemic* ; Psoriasis ; Thyroid Diseases ; Vitiligo*

BACKGROUND: Primary adrenal insufficiency (PAI) is a rare, potentially life-threatening condition. There are few Korean studies on PAI, and most have had small sample sizes. We aimed to examine the etiology, clinical characteristics, treatment, and mortality of PAI in Korean patients. METHODS: A nationwide, multicenter, registry-based survey was conducted to identify adults diagnosed with or treated for PAI at 30 secondary or tertiary care institutions in Korea between 2000 and 2014. RESULTS: A total of 269 patients with PAI were identified. The prevalence of PAI was 4.17 per million. The estimated incidence was 0.45 per million per year. The mean age at diagnosis was 49.0 years, and PAI was more prevalent in men. Adrenal tuberculosis was the most common cause of PAI in patients diagnosed before 2000; for those diagnosed thereafter, adrenal metastasis and tuberculosis were comparable leading causes. The etiology of PAI was not identified in 34.9% of cases. Of the patients receiving glucocorticoid replacement therapy, prednisolone was more frequently administered than hydrocortisone (69.4% vs. 26.5%, respectively), and only 27.1% of all patients received fludrocortisone. We observed an increased prevalence of metabolic disease and osteoporosis during the follow-up period (median, 60.2 months). The observed overall mortality and disease-specific mortality rates were 11.9% and 3.1%, respectively. CONCLUSION: The prevalence of PAI is significantly lower in Koreans than in reports from Western countries. The high frequency undetermined etiology in patients with PAI suggests the need to reveal accurate etiology of PAI in Korea.
Addison Disease* ; Adult ; Autoimmune Diseases ; Diagnosis ; Fludrocortisone ; Follow-Up Studies ; Glucocorticoids ; Humans ; Hydrocortisone ; Incidence ; Korea* ; Male ; Metabolic Diseases ; Mortality ; Neoplasm Metastasis ; Osteoporosis ; Prednisolone ; Prevalence ; Sample Size ; Tertiary Healthcare ; Tuberculosis

Adrenal tuberculosis is a relatively infrequent cause of primary adrenocortical insufficiency in developed countries. Isolated adrenal tuberculosis, especially with enlargement of adrenal glands, can cause diagnostic problems and requires differentiation from primary or secondary neoplasms. A 40-year-old woman presented to Soonchunhyang University Gumi Hospital with altered consciousness. She showed biochemical evidence of adrenal insufficiency without signs of tuberculosis. Abdominal computed tomography (CT) scan showed a calcified mass 3.3×2.3 cm in size and peripheral enhancement of the left adrenal gland. A laparoscopic adrenalectomy was performed for a diagnosis of left adrenal malignant tumor. The diagnosis of adrenal tuberculosis was confirmed by a final histological examination.
Addison Disease* ; Adrenal Glands ; Adrenal Insufficiency ; Adrenalectomy ; Adult ; Consciousness ; Developed Countries ; Diagnosis ; Female ; Gyeongsangbuk-do ; Humans ; Laparoscopy ; Tuberculosis*

An 80-year-old male with nausea and poor oral intake was referred for evaluation of hyponatremia. Primary adrenal insufficiency was diagnosed by a rapid adrenocorticotropic hormone (ACTH) stimulation test. The cause of the adrenal insufficiency was revealed to be adrenal tuberculosis presenting as a bilateral adrenal mass on computed tomography imaging. During the first few months of treatment, the size of the tuberculous mass increased and spread to an adjacent area, and further adrenal hormone replacement was needed. In addition, there was a newly developed tuberculous abscess in a nearby psoas muscle with a duodenal fistula. Thus, we report a case of a long-term clinical course of Addison's disease with changes in hormone replacement as a result of active adrenal tuberculosis, together with a review of the literature.
Abscess* ; Addison Disease* ; Adrenal Glands ; Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Aged, 80 and over ; Fistula* ; Humans ; Hyponatremia ; Male ; Nausea ; Psoas Muscles* ; Tuberculosis*

Adrenal hypoplasia congenita (AHC) is a rare inherited disorder of the adrenal gland caused by deletion or mutation of the dosage-sensitive sex-reversal AHC critical region on the X chromosome, gene 1 (DAX1) gene. The DAX1 gene is expressed in the adrenal cortex, the pituitary gland, the hypothalamus, the testis, and the ovary. Most affected infants present with failure to thrive, salt wasting, and hypoglycemic seizure in early life. Immediate mineralocorticoid and glucocorticoid replacement is essential. Most boys with AHC present with hypogonadotropic hypogonadism, resulting in failure to enter puberty and the need for testosterone treatment. However, a recent study revealed that the onset of puberty in boys with AHC can be variable, ranging from arrested or absent to precocious. We describe a case involving a newborn who presented with primary adrenal insufficiency due to a mutation of the DAX1 gene and was finally diagnosed with AHC.
Addison Disease* ; Adolescent ; Adrenal Cortex ; Adrenal Glands ; Adrenal Insufficiency ; Failure to Thrive ; Female ; Humans ; Hypogonadism ; Hypothalamus ; Infant ; Infant, Newborn* ; Ovary ; Pituitary Gland ; Puberty ; Seizures ; Testis ; Testosterone ; X Chromosome

Acute adrenal insufficiency (AI) is a life-threatening condition. While Addison’s disease (AD) is rare, in developing countries, tuberculosis (TB) still remains as the primary cause in 7 to 20% of cases. Urinary TB is also the third most common form of extrapulmonary disease. We report a case of 37-year-old male who presented with weakness, anorexia, weight loss, dysuria, flank pain and low grade fever. Examination revealed hypotension, hyperpigmentation, hyponatremia, hypoglycemia and low serum cortisol. He was diagnosed to have adrenal crisis due to Addison’s disease and extrapulmonary TB manifesting as urinary tract infection (UTI). He was treated with corticosteroids and anti-TB medications. Urologic reconstructive surgery was subsequently planned.
Addison Disease ; Tuberculosis

In cases of hyperkalemia with preserved renal function, the differential diagnoses that should be considered are drug-related disorders, primary tubular disease, and hormonal diseases including primary adrenal insufficiency. Addison's disease represents a rare disorder characterized by primary adrenal failure, general weakness, poor appetite, nausea, dizziness, and hyperpigmentation. It may also cause fatal adrenal crisis, involving hypotension, loss of consciousness, hyperkalemia, or hyperkalemic periodic paralysis under stressful conditions. We describe herein the case of a 54-year-old Korean male who developed Addison's disease, due to adrenal tuberculosis, in addition to painless thyroiditis, which led to hyperkalemic periodic paralysis.
Addison Disease* ; Appetite ; Diagnosis, Differential ; Dizziness ; Humans ; Hyperkalemia ; Hyperpigmentation ; Hyperthyroidism ; Hypotension ; Male ; Middle Aged ; Nausea ; Paralysis, Hyperkalemic Periodic* ; Thyroid Gland* ; Thyroiditis* ; Tuberculosis ; Unconsciousness

An adrenal crisis is a serious medical emergency. Most such crises develop in patients with Addison's disease when they are stressed. However, the clinical features of an impending adrenal crisis are nonspecific, delaying diagnosis and management. Adrenal tuberculosis is very rare among the extrapulmonary forms of tuberculosis. We herein report a case of an adrenal crisis triggered by bilateral adrenal tuberculosis. A 35-year-old man was admitted to our hospital because of drowsiness, fever, and hypotension. Abdominal computed tomography revealed bilateral adrenal enlargement, and computed tomography-guided adrenal gland biopsy allowed us to diagnose adrenal tuberculosis.
Addison Disease ; Adrenal Glands ; Adrenal Insufficiency ; Adult ; Biopsy ; Diagnosis ; Emergencies ; Fever ; Humans ; Hypotension ; Sleep Stages ; Tuberculosis* ; Tuberculosis, Endocrine