Autoimmune Addison's disease

Muir, Andrew; Schatz, Desmond; Maclaren, Noel K.

doi:10.1007/bf00195978

Cited by 22 publications

(5 citation statements)

References 32 publications

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“…Instead, T cell-mediated immunity is thought to be the main pathogenic cause of AAD [8,9]. The histological picture, which shows destruction of the adrenocortical parenchyma and widespread mononuclear infiltration, suggests similarities to other classic endocrine autoimmune disorders like type 1 diabetes and autoimmune thyroiditis [10,11]. The 21OH autoantibodies have been shown to be class switched to the IgG1 subtype, implying a CD4þ T cell response against the respective protein [12].…”

Section: Introductionmentioning

confidence: 99%

The purification and application of biologically active recombinant steroid cytochrome P450 21-hydroxylase: The major autoantigen in autoimmune Addison's disease

Bratland

Bredholt

Mellgren

et al. 2009

Journal of Autoimmunity

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Section: Introductionmentioning

confidence: 99%

The purification and application of biologically active recombinant steroid cytochrome P450 21-hydroxylase: The major autoantigen in autoimmune Addison's disease

Bratland

Bredholt

Mellgren

et al. 2009

Journal of Autoimmunity

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“…Three steroidogenic P450 enzymes, 21-hydroxylase, l7ct-hydroxylase and the side chain cleavage enzyme, have been reported to represent the major targets involved in the pathogenesis of the autoimmune destruction of the adrenal gland (Muir et al, 1993; Weetman l995 Weetman 1997). Until recently, adrenal cortex antibodies (ACA) as detected by indirect immunofluorescence test and immunoblotting techniques were the most widely used procedures for the detection of adrenal specific autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

“…In both conditions organ-specific T-and B-cell responses directed to adrenal gland autoantigens have been reported by several studies (Muir et al, 1993;Weetman 1997). Although it is unlikely that autoantibodies are directly involved in the pathogenesis of the disease, adrenal antibodies are useful tools for accurate diagnosis and prediction of autoimmune adrenal failure (Scherbaum et al, 1982;Betterle et al, 1997).…”

Section: Introductionmentioning

confidence: 97%

“…The autoimmune destruction of the adrenal cortex is a common cause of primary adrenal insufficiency (Addison's disease). In adults the autoimmune disorder occurs either as adrenalitis affecting only the adrenal gland or as part of an autoimmune polyglandular syndrome (APS ) type II which may comprise up to 50% of the cases (Muir et al, 1993;Weetman 1995). APS II is defined as an association of Addison's disease with at least one other autoimmune endocrinopathy, e.g.…”

Section: Introductionmentioning

confidence: 99%

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Autoantibodies to adrenal cytochrome P450 antigens in isolated Addison's disease and autoimmune polyendocrine syndrome type II

Seißler

Schott

Steinbrenner

et al. 2009

Exp Clin Endocrinol Diabetes

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Adrenal P450 enzymes 21-hydroxylase (21OH), 17alpha-hydroxylase (17OH) and side chain cleavage enzyme (SCC) represent major target antigens in adrenal autoimmunity. To evaluate the diagnostic sensitivity of autoantibodies to recombinant adrenal antigens we established rapid and sensitive radioligand assays and compared the results with adrenocortical autoantibodies (ACA) as detected by the standard immunofluorescence test. A high prevalence of antibodies to 21OH (21OH-A) was observed in patients with isolated Addison's disease (IAD) and patients suffering from autoimmune polyendocrine syndrome type II (APS II). 21OH-A were found in 19 of 25 (76%) patients with IAD and in 34 of 40 (85%) patients with APS II. In contrast, antibodies to 17OH (17OH-A) as well as antibodies to SCC (SCC-A) were detected in 12 (30%) and 13 (33%) patients with APS II whereas only a few sera from patients with IAD had 17OH antibodies (n = 3) and SCC-A (n = 1), respectively (p < 0.0001). The majority of patients with 17OH-A (83.3%) or SCC-A (76.9%) were also found positive for 21OH-A and all three antibody specificities were positively correlated with the presence of ACA. Among 52 sera with ACA 49 (94.2%), 11 (21.2%), and 9 (17.3%) were positive for 21OH-A, 17OH-A and SCC-A, respectively. By combination of 21OH-A with 17OH-A all ACA positive individuals were identified. The availability of recombinant steroid P450 enzymes made it possible to develop radiobinding assays which allow simple, sensitive and quantitative detection of autoantibodies to defined adrenal autoantigens. We here demonstrate that autoantibodies to 21-hydroxylase are sensitive markers for autoimmune Addison's disease with and without polyglandular failure. The presence of 17OH-A or SCC-A may suggest the coexistence of or progression towards polyglandular autoimmunity.

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“…This proves that the condition runs mostly in the families of those people who have had a positive family history of autoimmune disorders. Before this evidence was found, tuberculosis was thought to be the major cause behind the occurrence of APS Type II [3].…”

Section: Introductionmentioning

confidence: 99%

Late Presentation of Neglected Autoimmune Polyglandular Syndrome

Murad

ALakkad

Adwan

et al. 2021

JAMMR

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A 60-year-old male patient, weighing 40 kg, having a BMI of 13, who was also a known case of Diabetes Mellitus Type II was shifted to the Orthopaedic Ward last month due to a fall, which led him to develop an intertrochanteric fracture in the femur. While the treatment of the patient was in an ongoing status for his fracture, his Diabetes was seen to be poorly controlled, with his blood glucose levels being constantly evaluated to be in a state of severe hypoglycaemia to slightly increased levels of blood glucose. On examination, the patient showed clear signs of being malnourished. He was in a bad and dishevelled state, he had creases on the palmar aspects of his hands and hyperpigmentation on his buccal mucosa. Furthermore, his lab reports revealed abnormalities in nearly every lab test ordered. Not only did he have elevated ACTH levels, but there was also a failure of Cortisol stimulation. His DEXA Scan showed that he was predisposed to develop severe osteoporosis and his malnourished condition was supportive of promoting that condition even more. The patient was diagnosed to be suffering from Autoimmune Polyglandular Syndrome, Type II. This is a rare condition of one of its kind where there is a pre-existence of autoimmune adrenal insufficiency along with either autoimmune thyroid disease or autoimmune diabetes mellitus. Since this condition is rare, it tends to often get overlooked upon diagnosis, leading to misdiagnosis because almost all of the presenting features or the symptoms of the prevalent endocrinological disorders present in this condition mimic either hypothyroidism, or diabetes, or adrenal insufficiency alone, and thus lead to further consequences when the condition does not resolve despite persistent treatment, such as the case in this patient. This paper reviews the background of the patient and the causes that possibly could have made him reach this advanced stage of the disease. The paper also reflects upon the disease, Autoimmune Polyglandular Syndrome Type II, as a whole and elaborates on the symptoms and signs which the patient tens to confuse with other endocrinological diseases. Lastly, this paper shall also review the appropriate management plan for the patient to ease his symptoms and accelerate his recovery process.

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Autoimmune Addison's disease

Cited by 22 publications

References 32 publications

The purification and application of biologically active recombinant steroid cytochrome P450 21-hydroxylase: The major autoantigen in autoimmune Addison's disease

The purification and application of biologically active recombinant steroid cytochrome P450 21-hydroxylase: The major autoantigen in autoimmune Addison's disease

Autoantibodies to adrenal cytochrome P450 antigens in isolated Addison's disease and autoimmune polyendocrine syndrome type II

Late Presentation of Neglected Autoimmune Polyglandular Syndrome

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