Secondary adrenal insufficiency associated with autoimmune disorders: a report of twenty‐five cases

Kasperlik-Załuska, A; Czarnocka, Barbara; Czech, W; Walecki, Jerzy; Makowska, Anna; Brzeziński, J; Aniszewski, Jaroslaw P.

doi:10.1046/j.1365-2265.1998.00611.x

Cited by 28 publications

(22 citation statements)

References 15 publications

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“…This observation is consistent with a recent Japanese review of the literature, which reported that 24% of cases of isolated ACTH deficiency were associated with hypothyroidism, most commonly Hashimoto's thyroiditis (6). Similarly, thyroid autoimmunity, with or without subclinical or overt hypothyroidism, was found in , 25% of Polish patients with secondary adrenal insufficiency of uncertain aetiology (2). As cortisol is a physiological regulator of thyrotrophin secretion (1), resolution of biochemical hypothyroidism following adequate glucocorticoid therapy has been reported (32 -34) and thyroid function should therefore be reassessed after adequate glucocorticoid replacement.…”

Section: Discussionsupporting

confidence: 79%

“…It is most commonly observed during or after chronic administration of exogenous glucocorticoids and in patients with hypothalamic-pituitary tumours, often in association with other pituitary hormone deficits (1). Presenting symptoms are variable and non-specific and include fatigue, reduced strength, myalgia, arthralgia, weight loss, anorexia, postural dizziness and neuroglycopaenia (1,2). The diagnosis is suspected by finding a low morning cortisol in the absence of adrenocorticotropic hormone (ACTH) elevation and is confirmed by the demonstration of suboptimal cortisol levels following dynamic testing.…”

Section: Introductionmentioning

confidence: 99%

“…An autoimmune origin has been postulated, due to its frequent coexistence with other autoimmune diseases, particularly primary hypothyroidism secondary to chronic lymphocytic thyroiditis (1,9). Among 102 Polish patients with secondary adrenal insufficiency of unknown aetiology, 25 reported associated autoimmune disorders: 23 exhibited evidence of thyroid autoimmunity, 14 had overt hypothyroidism, three had subclinical hypothyroidism and three had treated Graves' disease (2). Other autoimmune diseases included type 1 diabetes, vitiligo, pernicious anaemia, autoimmune thrombocytopaenia and premature ovarian failure (2).…”

Section: Introductionmentioning

confidence: 99%

“…Among 102 Polish patients with secondary adrenal insufficiency of unknown aetiology, 25 reported associated autoimmune disorders: 23 exhibited evidence of thyroid autoimmunity, 14 had overt hypothyroidism, three had subclinical hypothyroidism and three had treated Graves' disease (2). Other autoimmune diseases included type 1 diabetes, vitiligo, pernicious anaemia, autoimmune thrombocytopaenia and premature ovarian failure (2). Consistent with a probable autoimmune origin, ACTH deficiency is the most common endocrine abnormality reported in patients with autoimmune lymphocytic hypophysitis, present in up to 65% of cases (9 -11).…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of pituitary function in the fatigued patient: a review of 59 cases

Greenfield

Samaras²

2006

eur j endocrinol

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Objective: The aim of this study was to review the results of dynamic pituitary testing in patients presenting with fatigue. Methods: We reviewed clinical histories and insulin tolerance test (ITT) results of 59 patients who presented with fatigue and other symptoms of glucocorticoid insufficiency over a 4-year period. All patients referred for ITT had an early-morning cortisol level of ,400 nM and a low or normal ACTH level. Results: Peak cortisol and GH responses following insulin-induced hypoglycaemia were normal in only seven patients (12%). Median age of the remaining 52 patients was 47 years (range, 17-67 years); all but five were female. Common presenting symptoms were neuroglycopaenia (n ¼ 47), depression (n ¼ 37), arthralgia and myalgia (n ¼ 28), weight gain (n ¼ 25), weight loss (n ¼ 9), postural dizziness (n ¼ 15) and headaches (n ¼ 13). Other medical history included autoimmune disease (n ¼ 20; particularly Hashimoto's thyroiditis, Graves' disease and coeliac disease), postpartum (n ¼ 8) and gastrointestinal (n ¼ 2) haemorrhage and hyperprolactinaemia (n ¼ 13). 31 subjects had peak cortisol levels of , 500 nM (suggestive of ACTH deficiency; 18 of whom had levels , 400 nM) and a further six had indeterminate results (500 -550 nM). The remaining 15 subjects had normal cortisol responses (median 654 nM; range, 553-1062 nM) but had low GH levels following hypoglycaemic stimulation (5.9 mU/l; 3-11.6 mU/l). Conclusion: Our results suggest that patients presenting with fatigue and symptoms suggestive of hypocortisolism should be considered for screening for secondary adrenal insufficiency, particularly in the presence of autoimmune disease or a history of postpartum or gastrointestinal haemorrhage. Whether physiological glucocorticoid replacement improves symptoms in this patient group is yet to be established. European Journal of Endocrinology 154 147-157

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Section: Discussionsupporting

confidence: 79%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Evaluation of pituitary function in the fatigued patient: a review of 59 cases

Greenfield

Samaras²

2006

eur j endocrinol

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“…[2] Most cases are secondary to autoimmune process such lymphocytic hypophysitis. [3,4] It may also occur after traumatic head injury [5] and pituitary irradiation. [6] Genetic cases are encountered in neonatal and childhood isolated ACTH deficiency.…”

Section: Introductionmentioning

confidence: 99%

Transient Adenocorticotropic Hormone deficiency in an infant

Jurayyan

Issa

Jurayyan

2016

CRCP

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Background: Isolated Adenocorticotropin Hormone (ACTH) deficiency is a rare disorder, characterized by secondary adrenal insufficiency with a low or absent cortisol production, normal secretion of other pituitary hormones and absence of structural pituitary defects. Case summary: The patient was the product of a 28 weeks gestation, clomiphen induced to a 40-year-old, gestational diabetic mother. He was ventilated for 30 days, and was diagnosed to have grade IV Retina of Prematurity (ROP). He suffered from recurrent attacks of hypoglycemic, blood sugar of 1.6 mmol/L, low serum cortisol 65 nmol/L (normal; 150-630), and ACTH of 1.5 pmol/L (normal; 1.6-13.9), with suppressed serum insulin and normal thyroid, growth hormone and gonadal functions. Magnetic Resonance Imaging (MRI) was unremarkable. He was started on hydrocortisone 2.5 mg twice daily for two years, which was then slowly tapered and stopped. Later Serum cortisol was 175 nmol/L, and ACTH of 5.5 pmol/L with a normal shot ACTH stimulation test. Conclusions: This case of a premature baby who presented with recurrent hypoglycemia had an isolated ACTH deficiency that proved to be transient. The pediatricians need to be aware of the existence of such condition.

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Hahner¹,

Arlt

Allolio³

2003

Der Internist

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Acute adrenal insufficiency is a rare but life-threatening disorder that develops as a result of inadequate adrenal steroid production. Early diagnosis is key for effective and life-saving treatment of the affected patients. The main clinical features are non-specific, thus often leading to misdiagnosis and invasive diagnostic work up, in particular in patients with previously unknown disease. Adrenal crisis in patients with known chronic adrenal insufficiency is nowadays rare, but can only be prevented by structured patient education on stress-related glucocorticoid dose adjustment. Outcome of adrenal crisis is crucially determined by the physician's clinical competence and immediate initiation of specific treatment.

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Secondary adrenal insufficiency associated with autoimmune disorders: a report of twenty‐five cases

Abstract: The co-existence of autoimmune disorders with secondary adrenal insufficiency suggests an autoimmune aetiology for the ACTH deficiency.

Cited by 28 publications

References 15 publications

Evaluation of pituitary function in the fatigued patient: a review of 59 cases

Evaluation of pituitary function in the fatigued patient: a review of 59 cases

Transient Adenocorticotropic Hormone deficiency in an infant

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