Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature

Uccella, Silvia; Amaglio, Cristina; Brouland, Jean‐Philippe; Bianconi, Eleonora; Ippolito, Silvia; Messerer, Mahmoud; Rouiller, Nathalie; Tanda, Maria Laura; Sessa, Fausto; Rosa, Stefano La

doi:10.1007/s00428-019-02564-2

Cited by 17 publications

(25 citation statements)

References 25 publications

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“…In accordance with the proposal of Leporati et al 13 and the biopsyproven cases of IgG4-RH published so far, [12][13][14][16][17][18][19][20][21][22][23][24][25][26] an increased number of IgG4 + plasma cells with or without fibrosis has been a basis for the diagnosis. In our cohort, all six patients fulfilled the criteria of Leporati et al 13 However, to induce remission in ANCA-associated vasculitis, more aggressive treatment strategies can be required.…”

Section: Discussionsupporting

confidence: 71%

“…[14][15][16] However, only about 30 of the published cases of IgG4-RH so far have been histologically confirmed in pituitary biopsy. [12][13][14][16][17][18][19][20][21][22][23][24][25][26] Thus, the whole spectrum of IgG4-related histopathological changes in the pituitary gland and sellar region is still unknown.…”

Section: Introductionmentioning

confidence: 99%

“…As the entity became better known among physicians, an increasing number of cases have been reported 14‐16 . However, only about 30 of the published cases of IgG4‐RH so far have been histologically confirmed in pituitary biopsy 12‐14,16‐26 . Thus, the whole spectrum of IgG4‐related histopathological changes in the pituitary gland and sellar region is still unknown.…”

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: Discussionsupporting

confidence: 71%

Section: Introductionmentioning

confidence: 99%

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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“…We speculate that the prolonged history of central DI in our patient could have been a primary clinical manifestation of IgG4‐RD. According to the literature, after the first report of pituitary involvement by IgG4‐RD in 2004, 22 more than 20 histopathological‐documented cases of “IgG4‐related hypophysitis” (IgG4‐RH) have been published till now 23 . More than half of the IgG4‐RH patients develop central DI 24,25 .…”

Section: Discussionmentioning

confidence: 99%

A challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

et al. 2020

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The IgG4‐related disease is a distinct, steroid‐responsive fibro‐inflammatory disorder of unknown etiology. This multiorgan disease is characterized by tumefactive lesions that contain rich infiltrations of IgG4‐positive plasma cells, with the pancreas, and the salivary and lacrimal glands being the main involved. The more common cardiovascular involvements include inflammatory peri‐aortitis, coronary arteritis, and pericarditis. Intra‐cardiac tumefactive lesions are rarely reported. Herein, we describe a challenging case of IgG4‐related disease with a long‐time lag between initiation of symptoms to proper diagnosis with biopsy‐proven cardiac and retroperitoneal and possible pituitary gland involvement. Concerning the rarity of the cardiac lesion in our case, we conducted a literature review of similar case reports.

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“…Reasonable precaution will be required, as the criteria necessitate judgment and diligence when used for any reason other than the assembly of a cohort for study and research. Unusual sites of infiltration have been reported, such as hypophysitis , thymus , temporal lobe , true ocular infiltrate , or aortic root involvement . Readers are reminded that classification criteria do not comprise the complete list of disease manifestations.…”

Section: Where We Started: the Blind Men And The Elephantmentioning

confidence: 99%

Classification of IgG4‐Related Disease: A Medical Marvel of Our Time

Bookman

2019

Arthritis & Rheumatology

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Disease heterogeneity in IgG4-related hypophysitis: report of two histopathologically proven cases and review of the literature

Cited by 17 publications

References 25 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

A challenging case report of IgG4‐related systemic disease involving the heart and retroperitoneum with a literature review of similar heart lesions

Classification of IgG4‐Related Disease: A Medical Marvel of Our Time

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