Critical review of IgG4-related hypophysitis

Shikuma, Junpei; Kan, Kenshi; Ito, Ryo; Hara, Kazuo; Sakai, Hiroyuki; Miwa, Takashi; Kanazawa, Akira; Odawara, Masato

doi:10.1007/s11102-016-0773-7

Cited by 60 publications

(73 citation statements)

References 32 publications

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“…As with AIP, patients with IgG4-related hypophysitis tend to be middle-aged and older men [14, 71, 79–81]. …”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

“…IgG4-related hypophysitis most often presents with panhypopituitarism [81], although anterior hypopituitarism with a combination of hormone deficiencies or diabetes insipidus has also been reported [49, 82, 83]. A predictable constellation of symptoms resulting from pituitary insufficiency may occur (e.g.…”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

“…Pituitary stalk enlargement is the most common finding on MRI of hypophysitis secondary to IgG4-RD, although there may be isolated involvement of the pituitary, concomitant involvement of the pituitary and stalk, and/or pseudotumour formation [80, 81, 83, 84, 116, 117]. The pituitary lesions are usually hypointense on T2-weighted imaging and show homogeneous contrast enhancement on T1-weighted images [116–123].…”

Section: Diagnostic Approachmentioning

confidence: 99%

“…Patients with IgG4-RD (including those with neurological involvement) usually have an excellent but often unsustained clinical response to glucocorticoids [1, 8, 15, 29, 81, 82, 112, 143, 144, 150]. Responsiveness to glucocorticoids is characteristic early in the disease course, before the onset of significant tissue fibrosis ensues.…”

Section: Treatmentmentioning

confidence: 99%

“…However, for neurological manifestations of IgG4-RD (mostly pachymeningitis), an initial course of intravenous methylprednisolone (e.g. 500 mg–1 g for 3 days) has been used in an attempt to rapidly and effectively dampen the inflammatory response and prevent irreversible CNS damage [47, 65, 71, 81, 112, 154], although some authors have reported positive outcomes with relatively low doses of glucocorticoids in pachymeningitis [96] and replacement therapy in hypophysitis [80, 81, 121, 128, 155, 156]. …”

Section: Treatmentmentioning

confidence: 99%

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Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

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Opinion statementIgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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“…As with AIP, patients with IgG4-related hypophysitis tend to be middle-aged and older men [14, 71, 79–81]. …”

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

Section: Neurological Manifestations Of Igg4-rdmentioning

confidence: 99%

Section: Diagnostic Approachmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

See 3 more Smart Citations

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

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Infiltrative and Inflammatory Disorders of the Hypothalamus and Pituitary

Fernandes

Varlamov

2022

Pituitary Disorders Throughout the Life Cycle

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Hypophysitis and Granulomatous Pituitary Lesions in Systemic Diseases

Jaffrain-Rea¹,

Filipponi²

2019

Endocrinology

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In clinical practice, expansive sellar lesions are largely represented by primary diseases of the pituitary gland, mostly pituitary tumors, which can be managed by endocrinologists and/or multidisciplinary pituitary teams. Hypophysitis has long been considered as a rare disease, and is classified on the basis of pathological findings, and as primary or secondary. Nowadays, an enlarging spectrum of pituitary and/or hypothalamic inflammatory, granulomatous, or even neoplastic disorders can be recognized, which may be part of systemic conditions and require appropriate multidisciplinary management involving a variety of specialists. For example, hypophysitis is being increasingly recognized in the setting of systemic IgG4-related disorders or as side effects of immune checkpoints inhibitors in oncological patients. Lymphocytic hypophysitis may sometimes be part of autoimmune polyendocrine syndromes. Significant knowledge has been added in the last decade in the pathogenesis and management of histiocytic disorders, which may infiltrate the pituitary and/or the hypothalamus Infective causes of pituitary inflammation should not be underestimated and are increasingly recognized in immunocompromised patients. Based on updated literature on these topics, this chapter aims to place pituitary inflammatory, granulomatous, and histiocytic disorders in their systemic context and stimulate multidisciplinary collaboration in order to optimize their diagnosis and clinical management.

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Critical review of IgG4-related hypophysitis

Abstract: IgG4-related hypophysitis may have different clinical characteristics between genders. This survey may lack some information because the Japanese abstracts did not contain certain details.

Cited by 60 publications

References 32 publications

Neurological Manifestations of IgG4-Related Disease

Neurological Manifestations of IgG4-Related Disease

Infiltrative and Inflammatory Disorders of the Hypothalamus and Pituitary

Hypophysitis and Granulomatous Pituitary Lesions in Systemic Diseases

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