Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis

Lupi, Isabella; Cosottini, Mirco; Caturegli, Patrizio; Manetti, Luca; Urbani, Claudio; Cappellani, Daniele; Scattina, Ilaria; Martino, Enio; Marcocci, Claudio; Bogazzi, Fausto

doi:10.1530/eje-17-0123

Cited by 31 publications

(15 citation statements)

References 20 publications

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“…Notably, although with GC treatment, pituitary function/diabetes insipidus did not improve considerably (as shown in Table 4 ). Similar results were shown in a recent study of autoimmune hypophysitis [ 37 ]. Life-long hormone replacement therapy should be expected.…”

Section: Discussionsupporting

confidence: 92%

Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center

Liu

Wang

Zhang

et al. 2018

International Journal of Endocrinology

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This study aims to outline the clinical features and outcomes of IgG4-related hypophysitis (IgG4-RH) patients in a tertiary medical center. We reviewed clinical manifestations and imaging and pituitary function tests at baseline, as well as during follow-up. Ten patients were included. The mean age at diagnosis of IgG4-RH was 48.4 (16.0–64.0) years. An average of 3 (0–9) extrapituitary organs were involved. Five patients had panhypopituitarism, three had only posterior hypopituitarism, one had only anterior hypopituitarism, and one had a normal pituitary function. One patient in our study had pituitary mass biopsy, lacking IgG4-positive cells despite lymphocyte infiltration forming an inflammatory pseudotumor. Five patients with a clinical course of IgG4-RH less than nine months and a whole course of IgG4-RD less than two years were managed with glucocorticoids, while three patients with a longer history were administered glucocorticoids plus immunosuppressive agents. One patient went through surgical excision, and one patient was lost to follow-up. All patients showed a prompt response clinically, but only three patients had normalized serum IgG4 levels. Two patients who took medications for less than six months relapsed. Conclusions. IgG4-RD is a broad disease, and all physicians involved have to be aware of the possibility of pituitary dysfunction. Younger patients should be expected. The histopathological feature of pituitary gland biopsy could be atypical. For patients with a longer history, the combination of GC and immunosuppressive agents is favorable. Early and adequate courses of treatment are crucial for the management of IgG4-RH. With GC and/or immunosuppressant treatment, however, pituitary function or diabetes insipidus did not improve considerably.

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Section: Discussionsupporting

confidence: 92%

Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center

Liu

Wang

Zhang

et al. 2018

International Journal of Endocrinology

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“…Less frequently, these symptoms can also occur in adenohypophysitis as a consequence of stalk compression and impaired vasopressin transport [4,20,28], whereas hyperpolactinaemia is reported in 42-48 % [16,17] of LYH cases [9,20,29,30]. In a recent study including 12 patients with autoimmune-hypophysitis, DI had a negative prognostic role with regard to the response to glucocorticoids, since none of the patients with infundibulo-neuro-hypophysitis recovered, but our series was too small to make any accurate predictions [31]. The severity and rapid onset of LYH manifestations are not usually correlated with the degree of pituitary enlargement, suggesting that direct immune-mediated destruction of hormone secreting cells, rather than compression or architectural distortion, is responsible for its pathogenesis [4,9].…”

Section: Discussionmentioning

confidence: 67%

Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis

et al. 2018

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Primary hypophysitis (PH) is a rare disease with a poorly-defined natural history. Our aim was to characterise patients with PH at presentation and during prolonged follow-up. Observational retrospective study of 22 patients was conducted from 3 centres. In 14 patients, PH was confirmed histologically and in the remaining 8 clinically, after excluding secondary causes of hypophysitis. All patients had hormonal and imaging investigations before any treatment. Median follow up was 48 months (25-75%: 3-60). There was a female predominance with a female/male ratio: 3.4:1. Eight out of 22 patients had another autoimmune disease. Headaches and gonadal dysfunction were the most common symptoms. Five patients presented with panhypopituitarism; 17 patients had anterior pituitary deficiency, and 7 had diabetes insipidus. At presentation, 9 patients were treated surgically, 5 received replacement hormonal treatment, and 8 high-dose glucocorticoids from whom 5 in association with other immunosuppressive agents. Six patients showed complete recovery of pituitary hormonal deficiencies while 6 showed a partial recovery during a 5-year follow-up period. No difference was found between patients treated with surgery and those treated medically. The overall relapse rate was 18%. PH can be manifested with a broad spectrum of clinical and hormonal disturbances. Long-term follow-up is required to define the natural history of the disease and response to treatment, since pituitary hormonal recovery or relapse may appear many years after initial diagnosis. We suggest that surgery and immunosuppressive therapy be reserved for exceptional cases.

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“…However, PAH outcome after immunosuppressive treatment is not completely clarified: pituitary function and morphological improvements were observed between 15% and 90% of cases (8)(9)(10)(11)(12)(13)(14)(18)(19)(20). Treatment outcome seems influenced by pharmacological doses of glucocorticoids, short standing disease, and diagnosis of diabetes insipidus (20)(21)(22). Currently, the debate on the indication, benefits, optimal timing, and dosage of glucocorticoids for PAH treatment remains still open (23), and the therapies of hypophysitis present several disputes, without available guidelines or consensus of treatment protocol, according to the rarity of the disease, the lack of prospective studies, and the undefined natural history of the disease (24).…”

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confidence: 99%

Hypophysitis Outcome and Factors Predicting Responsiveness to Glucocorticoid Therapy: A Prospective and Double-Arm Study

Chiloiro

Tartaglione

Capoluongo

et al. 2018

The Journal of Clinical Endocrinology &Amp; Metabolism

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Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis

Abstract: Glucocorticoid therapy may improve anterior pituitary function in a subset of patients but has no effect on restoring posterior pituitary function. Diabetes insipidus appears as a negative prognostic factor for response to glucocorticoids.

Cited by 31 publications

References 20 publications

Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center

Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center

Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis

Hypophysitis Outcome and Factors Predicting Responsiveness to Glucocorticoid Therapy: A Prospective and Double-Arm Study

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