Diagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society

Takagi, Hiroshi; Iwama, Shintaro; Sugimura, Yoshihisa; Takahashi, Yutaka; Osuga, Yutaka; Akamizu, Takashi; Arima, Hiroshi

doi:10.1507/endocrj.ej19-0569

Cited by 28 publications

(50 citation statements)

References 26 publications

(36 reference statements)

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“…Having the number of IgG4 + cells as the only criterion, without storiform fibrosis or any exclusion criterion, may result in an overdiagnosis of the IgG4-RH. The reported prevalence of IgG4-RH is around 4% in patients with hypopituitarism and/or central diabetes insipidus, 14,27 whereas no conclusions regarding the prevalence of the IgG4-RH may be generated from our study. However,…”

Section: Discussioncontrasting

confidence: 69%

“…6 Histological criteria including the presence of more than 10 IgG4 + cells per HPF and IgG4 + /IgG + ratio of more than 40% are also included in the clinical guidelines for the diagnosis of IgG4-RH by the Japan Endocrine Society. 27 Recently, the American College of Rheumatology and the European League Against Rheumatism (ACR/ EULAR) published a more complex set of inclusion and exclusion criteria for the classification of IgG4-RD in the frequently affected organs, not including the pituitary gland. 28 In the present study, we aimed to describe the histopathological changes in the surgical specimens from patients with clinical signs of pituitary disease that fulfilled the criteria for IgG4-RH.…”

Section: Introductionmentioning

confidence: 99%

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Histopathological findings in the landscape of IgG4‐related pathology in patients with pituitary dysfunction: Review of six cases

Vasaitis

Wikström

Ahlström

et al. 2021

J Neuroendocrinology

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IgG4-related hypophysitis (IgG4-RH) is increasingly being reported as an isolated entity or, less frequently, as a manifestation of a multiorgan IgG4-related disease (IgG4-RD), in which typical histopathology is a cornerstone for the diagnosis. We aimed to describe the histopathological changes in the surgical specimens from patients with clinical signs of pituitary disease that fulfilled the current diagnostic criteria for IgG4-RH. Histopathological features were correlated with clinical and radiological findings. Of 19 patients with pituitary dysfunction and inflammatory changes in the surgical pituitary specimen operated on during 2011-2019, we identified five patients with typical IgG4-related pathology (lymphoplasmacytic infiltration with more than 10 IgG4-positive plasma cells per one high power microscopic field, representing at least 40% of all plasma cells and at least focal storiform fibrosis). One patient with diabetes insipidus and pachymeningitis with IgG4-related changes in a biopsy from the dura was also included. Additional histopathological changes that typically are not part of the IgG4-RH were observed: Rathke's cleft cyst in four and granulomatous changes in two patients. One patient had an elevated serum IgG4 level and systemic manifestations that could be associated with the systemic IgG4-RD. Our findings indicate that pure IgG4-RH is uncommon. All patients with pituitary dysfunction, beyond typical IgG4-related pathology, had other pathological findings that could trigger the secondary IgG4-response. Both primary pathology and secondary IgG4-related features should be reported in patients with pituitary dysfunction because their co-occurrence may cause atypical clinical and imaging features, and unexpected response to surgical and pharmacological treatment. The current criteria for the diagnosis of IgG4-RH can lead to overdiagnosis of IgG4-RH if additional pathological changes are not taken into consideration. The classification criteria of IgG4-RD proposed by the American College of Rheumatology/European League Against Rheumatism could help classify patients more properly as IgG4-RH if applied to the pituitary gland.

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Section: Discussioncontrasting

confidence: 69%

Section: Introductionmentioning

confidence: 99%

Histopathological findings in the landscape of IgG4‐related pathology in patients with pituitary dysfunction: Review of six cases

Vasaitis

Wikström

Ahlström

et al. 2021

J Neuroendocrinology

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“…Hypophysitis due to RCC is listed as one of the sella and parasellar diseases in the clinical guidelines of the Japan Endocrine Society [19]. Pathologically, hypophysitis is mainly classified into two forms: primary and secondary forms.…”

Section: Discussionmentioning

confidence: 99%

Two cases of symptomatic secondary hypophysitis due to Rathke’s cleft cysts treated with glucocorticoids: long-term follow-up

et al. 2021

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Rathke's cleft cyst (RCC) is a common incidental tumor in the hypothalamic-pituitary region. Some reports have shown that the clinical symptoms and endocrine functions of symptomatic RCCs are temporarily improved by glucocorticoid administration. However, it is still unknown whether glucocorticoid treatment is effective for symptomatic RCCs according to long-term observations. In this study, we describe the long-term clinical outcomes of two cases of glucocorticoid-treated biopsy-proven secondary hypophysitis caused by RCCs. We summarize the symptoms, imaging findings, and endocrine evaluations of two symptomatic RCC patients with concomitant hypophysitis before and after prednisolone treatment. In both evaluated cases, visual impairments and altered endocrine parameters were present due to chiasm and stalk compression; these outcomes improved after shrinkage of RCCs in response to prednisolone administration, and partial recovery of anterior pituitary hormone secretion was observed. However, in both cases, the deficits in anterior pituitary hormone secretion recurred, possibly due to persistent inflammatory infiltration in the RCCs and pituitary glands. After relapse of hypophysitis, anterior hormone secretion did not fully recover. In our cases of secondary hypophysitis caused by RCCs, prednisolone administration had an early effect of cyst shrinkage, followed by partial improvements in clinical symptoms and pituitary functions. However, long-term observation showed that prednisolone treatment did not contribute to complete improvement in anterior pituitary hormone dysfunction.

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“…Based on the above, the patient was diagnosed with AH, anterior pituitary hypofunction, SLE, NIDDM, and CVD. According to the clinical guidelines ( 6 , 7 ), the patient was treated with 30 mg/d prednisone, 80 mg/d testosterone undecanoate, and 50 µg/d levothyroxine for the treatment of anterior pituitary hypofunction; 0.4 g/d hydroxychloroquine for the treatment of SLE; 22 U/d premixed human insulin for the treatment of NIDDM; and 75 mg/d clopidogrel, 10 mg/d atorvastatin calcium tablets, 50 mg/d captopril and 100 mg/d metoprolol for the treatment of CVD. Three days after the treatment, the sodium level had risen to 134 mmol/L, and the patient’s symptoms resolved gradually.…”

Section: Case Presentationmentioning

confidence: 99%

Autoimmune Hypophysitis With Systemic Lupus Erythematosus: A Case Report and Literature Review

Xiang

Zhang

et al. 2020

Front. Endocrinol.

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Background: Autoimmune hypophysitis (AH) is a primary autoimmune inflammatory disorder of the pituitary gland, which usually presents as a mass in the sella turcica. Systemic lupus erythematosus (SLE) is another inflammatory disorder in which the immune system attacks healthy cells and tissues throughout the body. Although both diseases are autoimmune disorders, they rarely coexist, and the relationship between them is unclear. Case Report: A 66-year-old man was evaluated at the endocrinology clinic because of worsening fatigue, anorexia, drowsiness, and leg oedema. Examination revealed alertness impairment and lower limb oedema. Laboratory tests showed anterior pituitary hypofunction. The treatment approach, with glucocorticoids and immunosuppressive agents, resulted in long-term remission of symptoms of hypopituitarism and hyponatraemia. Conclusions: Our case demonstrates a potential association between AH and SLE. AH may need to be considered in the evaluation of SLE patients with headache, hyperprolactinemia, a pituitary mass, and hypopituitarism.

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Diagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society

Cited by 28 publications

References 26 publications

Histopathological findings in the landscape of IgG4‐related pathology in patients with pituitary dysfunction: Review of six cases

Histopathological findings in the landscape of IgG4‐related pathology in patients with pituitary dysfunction: Review of six cases

Two cases of symptomatic secondary hypophysitis due to Rathke’s cleft cysts treated with glucocorticoids: long-term follow-up

Autoimmune Hypophysitis With Systemic Lupus Erythematosus: A Case Report and Literature Review

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