Pituitary autoantibodies in autoimmune polyendocrine syndrome type 1

Bensing, Sophie; Fetissov, Sergueı̈ O.; Mulder, Jan; Perheentupa, Jaakko; Gustafsson, Jan Åke; Husebye, Eystein S.; Oscarson, Mikael; Ekwall, Olov; Crock, Patricia; Hökfelt, Tomas; Hulting, Anna‐Lena; Kämpe, Olle

doi:10.1073/pnas.0610070104

Cited by 87 publications

(53 citation statements)

References 53 publications

(48 reference statements)

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“…In particular, antibodies against a 49 kDa antigen were detected in 60% of patients with SS but also in 17.8% of normal females with prior conception. Moreover, the 49 kDa pituitary protein was identified as a-enolase, an enzyme ubiquitously expressed; however, the importance of antibodies to enolase as markers of pituitary autoimmunity is still debated (18). Our results confirm that pituitary autoimmunity may play a role in inducing late hypopituitarism following post partum hemorrhage.…”

Section: Discussionsupporting

confidence: 77%

Anti-hypothalamus and anti-pituitary antibodies may contribute to perpetuate the hypopituitarism in patients with Sheehan's syndrome

Bellis

Keleştimur²,

Sinisi³

et al. 2008

European Journal of Endocrinology

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Objective: While anti-pituitary antibodies (APAs) were detected in some patients with Sheehan's syndrome (SS) suggesting an autoimmune pituitary involvement in the development of their hypopituitarism, hypothalamic cell anti-hypothalamus antibodies (AHAs) have not been investigated so far. Design: The aim of this study was to evaluate the presence of AHA and APA in SS patients to verify whether an autoimmune hypothalamic-pituitary process can contribute to their late hypopituitarism. Methods: Twenty women with SS with a duration of disease ranging from 3 to 40 years (median 25.5 years) were enrolled into the study. Out of 20 patients, 12 (60%) had panhypopituitarism and the others had partial hypopituitarism well corrected with appropriate replacement therapy. None of them had clinical central diabetes insipidus. AHA and APA were investigated by immunofluorescence method in all patients. In addition, a four-layer immunofluorescence method was used to verify whether AHA immunostained vasopressin-secreting cells (AVP-c) or not. Results: AHAs were found in 8 out of 20 (40%) and APAs in 7 out of 20 (35%) patients with titers ranging from 1:32 to 1:128 and 1:16 to 1:32 respectively; however, in none of these positive patients AHA immunostained vasopressin cells. None of controls resulted positive for both antibodies. Conclusions: Patients with SS, even many years after the onset of SS, can show antibodies to pituitary and/or hypothalamic but not AVP-secreting cells. Antibodies to unknown hypothalamic cells (releasing factor-secreting cells) other than APAs suggest that an autoimmune process involving both the hypothalamus and pituitary gland may contribute to late pituitary dysfunction in SS patients.

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Section: Discussionsupporting

confidence: 77%

Anti-hypothalamus and anti-pituitary antibodies may contribute to perpetuate the hypopituitarism in patients with Sheehan's syndrome

Bellis

Keleştimur²,

Sinisi³

et al. 2008

European Journal of Endocrinology

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“…Self-antibodies to pituitary proteins, such as the tudor domain-containing protein 6 (TDRD6), were also identified, but they do not seem to be associated with clinical hypopituitarism or hypophysitis in APS-1 patients (69).…”

Section: Self-antibodiesmentioning

confidence: 99%

Autoimmune polyendocrine syndrome type 1: case report and review of literature

Weiler

Silva

Lazaretti-Castro

2012

Arq Bras Endocrinol Metab

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SUMMARYAutoimmune polyendocrine syndrome type 1 (APECED) is a rare autosomal recessive disorder characterized by autoimmune multiorgan attack. The disease is caused by mutations in the autoimmune regulator gene (AIRE), resulting in defective AIRE protein, which is essential for selftolerance. Clinical manifestations are widely variable. Although the classic triad is composed by mucocutaneous candidiasis, hypoparathyroidism and adrenal failure, many other components may develop. Treatment is based on supplementation of the various deficiencies, and patients require regular follow-up throughout their lifespan. This article describes the case of a patient with the disease, and reviews literature data on the epidemiology, clinical course, immunogenetic aspects, diagnosis and treatment of the syndrome. Arq Bras Endocrinol Metab. 2012;56(1):54-66 SUMÁRIOSíndrome poliglandular autoimune tipo 1 é uma rara desordem autossômica recessiva caracterizada por ataque autoimune a diversos órgãos. A doença é causada por mutações no gene AIRE (autoimmune regulator), resultando em uma proteína AIRE defeituosa, proteína esta essencial para a manutenção da autotolerância. As manifestações clínicas são extremamente variáveis. A tríade clássica é composta por candidíase mucocutânea crônica, hipoparatiroidismo e insuficiência adrenal, porém diversos outros componentes podem estar presentes. A base do tratamento é a reposição das diversas deficiências, e os pacientes devem ser acompanhados por toda a vida. Este artigo descreve o caso de uma paciente com a síndrome e apresenta uma revisão sobre a epidemiologia, quadro clínico, aspectos imunogenéticos, diagnóstico e tratamento da desordem, de acordo com a literatura publicada. Arq Bras Endocrinol Metab. 2012;56(1):54-66

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“…This is the first evidence in patients with autoimmune GHD, diagnosed in childhood and treated with rGH replacement therapy until the transition age, of a shift of APA from a pituitary target at diagnosis (somatotrophs) to another pituitary target (gonadotrophs) at the observation in transition period, with consequent variation of the selective kind of hypopituitarism, from GHD to HH. The cause of this surprising behavior is at present unknown, perhaps because the true pituitary antigen(s) responsible for the autoimmune response have yet to be identified, despite several factors being suggested as possible autoantigens (22,23,24,25,26,27,28).…”

Section: Patients No Sexmentioning

confidence: 99%

Longitudinal behavior of autoimmune GH deficiency: from childhood to transition age

Bellis¹,

Bellastella²,

Maiorino³

et al. 2016

European Journal of Endocrinology

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Background: Some cases of apparently idiopathic GH deficiency (GHD) may be caused by pituitary autoimmunity. Objective: To study the variations in pituitary function and antipituitary antibodies (APA) from childhood to transition age in patients with apparently idiopathic GHD. Design: We conducted a longitudinal study. Patients and methods: Pituitary function and APA detection by immunofluorescence were investigated in 24 childhood patients with isolated GHD before starting recombinant GH therapy and after the stopping of this therapy in transition age. Sera of patients positive for APA were processed by double immunofluorescence to identify their pituitary target. Results: At diagnosis, 16 out of 24 patients were APA positive targeting only somatotrophs (group 1), while the remaining eight were APA negative (group 2). When retested off therapy, 12 out of 16 patients in group 1 persisted being APA positive, while the remaining four became negative with recovery of pituitary function. All patients in group 2 persisted being APA negative but still showing GHD. Of the 12 patients persistently APA positive, eight with confirmed GHD showed APA still targeting somatotrophs, whereas four showed APA targeting only gonadotrophs associated with isolated hypogonadotropic hypogonadism (HH). Conclusion: Patients with APA at middle but not at high titer in childhood may show a remission of autoimmune GHD in childhood after GH replacement therapy. As APA may shift their target in transition period, an early characterization of APA by double immunofluorescence is advisable in APA positive GHD patients showing delayed puberty, to allow an early diagnosis and an appropriate therapy, thus preventing the progression toward HH.

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Pituitary autoantibodies in autoimmune polyendocrine syndrome type 1

Cited by 87 publications

References 53 publications

Anti-hypothalamus and anti-pituitary antibodies may contribute to perpetuate the hypopituitarism in patients with Sheehan's syndrome

Anti-hypothalamus and anti-pituitary antibodies may contribute to perpetuate the hypopituitarism in patients with Sheehan's syndrome

Autoimmune polyendocrine syndrome type 1: case report and review of literature

Longitudinal behavior of autoimmune GH deficiency: from childhood to transition age

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