scholarly journals Clinical course of pituitary function and image in IgG4-related hypophysitis

2017 ◽  
Vol 2017 ◽  
Author(s):  
Takatoshi Anno ◽  
Fumiko Kawasaki ◽  
Maiko Takai ◽  
Ryo Shigemoto ◽  
Yuki Kan ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Adrenal Insufficiency ◽  
Clinical Course ◽  
Laboratory Data ◽  
Glucocorticoid Therapy ◽  
High Dose ◽  
Glucocorticoid Treatment ◽  
Serum Igg4 ◽  
Physiological Dose ◽  
Igg4 Level

Summary A 76-year-old man had a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism. Based on various findings including the swelling of the pituitary gland, increase of serum IgG4 level and abundant IgG4-positive plasma cell infiltration in immunostaining of the pituitary gland, we diagnosed this subject as IgG4-related hypophysitis. In general, a high-dose glucocorticoid treatment is effective for IgG4-related disease. His clinical symptom, laboratory data and adrenal insufficiency were almost improved without any therapy. The serum IgG4 level was decreased and pituitary size was normalized with hydrocortisone as physiological replacement. This case report provides the possibility that IgG4 level is decreased spontaneously or with physiological dose of glucocorticoid therapy. Learning points: We performed the pituitary gland biopsy and histochemical examination glucocorticoid therapy in a subject with IgG4-related hypophysitis. This case report provides the possibility that IgG4 level is decreased spontaneously or with a physiological dose of glucocorticoid therapy. We reported the clinical course of IgG4-related hypophysitis without a high-dose glucocorticoid treatment, although there were a few reports about the retrospective examination. Although the patient had still higher IgG4 level compared to normal range, his clinical symptom disappeared and his laboratory data were improved. We should keep in mind the possibility of IgG4-related hypophysitis when we examine one of the uncertain causes of a hypopituitarism including adrenal insufficiency, hypogonadism and hypothyroidism.

scholarly journals Serum IgG4 Concentration is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis

2022 ◽  
Author(s):  
Shoichiro Mukai ◽  
Naotaka Sakamoto ◽  
Hiroaki Kakinoki ◽  
Tadamasa Shibuya ◽  
Ryosuke Moriya ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Predictive Marker ◽  
Predictive Biomarker ◽  
Complete Response ◽  
Glucocorticoid Therapy ◽  
Smoking History ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Glucocorticoid Treatment ◽  
Female Ratio ◽  
Serum Igg4

Abstract Objectives: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and identify its clinical biomarker. Methods: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR and partial response groups: PR), and each parameter was compared statistically.Results: Male-female ratio was 5: 1, and median age at diagnosis was 69 (33-86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6mg/dL vs 255mg/dL, 206mg/dL, P=0.0059 and 0.0078). ROC analysis was performed between the non-responder (NC) and responder groups (CR+PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC was 0.793 and the values of sensitivity and specificity were 0.85 and 0.64, respectively, under the cut-off values of 67.6mg/dL. Conclusions: In the majority of iRPF patients, glucocorticoid therapy resulted in a favorable response. Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.

scholarly journals P.089 Recovery from chronic secondary adrenal insufficiency in patients with pituitary disorders

2016 ◽  
Vol 43 (S2) ◽  
pp. S41-S41
Author(s):  
V Munro ◽  
B Tugwell ◽  
S Doucette ◽  
DB Clarke ◽  
A Lacroix ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Tumour Size ◽  
Pituitary Surgery ◽  
Complete Recovery ◽  
Glucocorticoid Therapy ◽  
Hormone Deficiency ◽  
Secondary Adrenal Insufficiency ◽  
Glucocorticoid Treatment ◽  
Acth Secreting

Background: Patients with pituitary disorders may be placed on steroid replacement for secondary adrenal insufficiency (SAI), generally after pituitary surgery; however, data regarding recovery of long-term SAI are lacking. We conducted a study to assess the longer term recovery rate of SAI in patients with pituitary disorders. Methods: We identified all SAI patients from prospectively entered data in the Halifax Neuropituitary Database from November 1, 2005 to September 30, 2014, who had required glucocorticoid therapy for >3 months, and a minimum follow-up of 6 months. Exclusion: ACTH-secreting adenomas; peri-operative glucocorticoid treatment only; glucocorticoids for non-pituitary conditions. Results: 55 patients fulfilled the criteria, 41 (75%) of which had transsphenoidal surgery. Nine (16.4%) patients had complete recovery of SAI over a median of 20 months (range: 8–51). Smaller tumour size and initial cortisol >175 nmol/L had increased likelihood of recovery; those with secondary hypogonadism or growth hormone deficiency were less likely to recover. Conclusions: This is the first study to examine long-term recover of SAI in patients with pituitary disorders: approximately 1 in 6 patients recover adrenal function, up to 5 years after diagnosis. Consequently, patients with SAI should undergo regular testing to prevent unnecessary chronic glucocorticoid therapy.

scholarly journals Advances in IgG4-related Hepatobiliary Disease

2020 ◽  
Vol 25 (2) ◽  
pp. 100-106
Author(s):  
Sung-Hoon Moon
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Quantitative Polymerase Chain Reaction ◽  
High Serum ◽  
Hepatobiliary Disease ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Polymerase Chain

The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.

scholarly journals AB0353 ADRENAL INSUFFICIENCY AFTER GLUCOCORTICOID TREATMENT OF GIANT CELL ARTERITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1201.2-1201
Author(s):  
A. Hočevar ◽  
R. Jese ◽  
J. Kramarič ◽  
M. Tomsic ◽  
Z. Rotar
Keyword(s):  
Adrenal Insufficiency ◽  
Giant Cell Arteritis ◽  
Giant Cell ◽  
Cortisol Level ◽  
Adrenal Function ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Systemic Glucocorticoid ◽  
The Mean ◽  
Corticotropin Stimulation

Background:Adrenal insufficiency is frequently neglected and underappreciated, potentially severe complication of systemic glucocorticoid therapy.Objectives:We aimed to evaluate the prevalence of glucocorticoid induced adrenal insufficiency in giant cell arteritis (GCA).Methods:We analysed adrenal function data in a cohort of GCA patients diagnosed between July 2014 and July 2019, in whom discontinuation of methylprednisolone therapy was planned. Adrenal function was tested by Corticotropin stimulation test (CST). To perform the CST, methylprednisolone was substituted with hydrocortisone (20mg qd in three divided doses) for one to four weeks before the test. Adrenal insufficiency was defined as cortisol level <450 nmol/l measured 30 minutes after the corticotropin injection; additionally, the result of the CST was defined as borderline when the cortisol level 30 minutes after corticotropin injection was between 450 nmol/l and 500 nmol/l.Results:Adrenal function was tested in 74/215 GCA patients before definite methylprednisolone withdrawal (after a median 13.5 (12.9 – 22.4) months of glucocorticoid therapy). The mean (SD) methylprednisolone dose, prior to substitution with hydrocortisone and subsequent CST, was 3.1 (1.6) mg. Adrenal insufficiency was detected in 36/74 patients (48.6%); additionally, 10/74 patients (13.5%) had a borderline CST result. Seventeen patients with either adrenal insufficiency or borderline CST result, had a repeated CST after median (IQR) 11.6 (8.9; 12.6) months. Adrenal insufficiency persisted in 11/17 (64.7%) patients, and 1/17 patients had a borderline CST. A third CST was performed in 4/12 patients with abnormal second CST after median (IQR) 8.3 (6.9; 10.6) months. Adrenal function recovered in one patient, while the adrenal insufficiency persisted in the remaining 3 patients.Conclusion:Adrenal insufficiency is a common and potentially long-lasting glucocorticoid induced adverse event in GCA patients.Disclosure of Interests:None declared

scholarly journals Two cases of immunoglobulin G4 (IgG4)-related hypophysitis diagnosed without pituitary biopsy

2020 ◽  
Vol 11 ◽  
pp. 204201882092455
Author(s):  
Wanlu Ma ◽  
Xi Wang ◽  
Min Nie ◽  
Junling Fu ◽  
Jiangfeng Mao ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Hypogonadotropic Hypogonadism ◽  
Hormone Secretion ◽  
Disease Diagnosis ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Igg4 Level

Background: Immunoglobulin G4-related hypophysitis (IgG4-RH) is a rare disease, diagnosis of which typically depends on histopathology following an invasive pituitary biopsy, possibly leading to permanent hypopituitarism. Herein, we report two cases of IgG4-RH with favorable responses to glucocorticoids. One of them was multiple organs involved and treated with glucocorticoids and methotrexate. Methods: We retrospectively review clinical features, radiological images, and treatment of two cases with IgG4-RH. In addition, literature on IgG4-RH was comprehensively reviewed and a new therapeutic strategy for IgG4-RH was provided. Results: A 45-year-old man presented with diabetes insipidus for 6 months. Pituitary magnetic resonance imaging (MRI) indicated thickening of pituitary stalk. His serum IgG4 was 13,500 mg/l and hormonal evaluation revealed isolated growth hormone deficiency. Pituitary biopsy was denied by the patient due to fears of permanent pituitary damage. Treatment with prednisone and methotrexate (MTX) for 1 week led to improvement in sellar images and reduction in IgG4 level. His IGF1 (insulin-like growth factor-1) recovered after a 4-month treatment. The second case is a 43-year-old woman presenting with diabetes insipidus and amenorrhea for 20 months. Her pituitary MRI was similar to the patient above. Her serum IgG4 level was 5980 mg/l and hormonal measurement confirmed isolated hypogonadotropic hypogonadism. After 2 weeks of prednisone, the sellar images improved. After 3 months of treatment, her pituitary MRI was normal, IgG4 level had decreased to near normal range, and menstruation resumed. Literature review found additional patients with IgG4-RH, who were treated successfully without invasive pituitary biopsy in a manner similar to our cases. Therefore, we discuss the necessity of invasive pituitary biopsy for IgG4-RH. Conclusion: For suspected IgG4-RH with pituitary hormone deficiency, biopsy-induced hypopituitarism may be avoided by using diagnostic glucocorticoid treatment. Impaired pituitary hormone secretion may be recovered in response to steroid therapy. Improved pituitary MRI after 1–2 weeks of glucocorticoid treatment may provide diagnostic evidence of IgG4-RH.

scholarly journals Glucocorticoid induced adrenal insufficiency

BMJ ◽  
2021 ◽  
pp. n1380
Author(s):  
Alessandro Prete ◽  
Irina Bancos
Keyword(s):  
Adrenal Insufficiency ◽  
Withdrawal Syndrome ◽  
Adrenal Function ◽  
Glucocorticoid Therapy ◽  
Patient Counseling ◽  
Glucocorticoid Treatment ◽  
Factors Affecting ◽  
Function Recovery ◽  
Underlying Disorder ◽  
Glucocorticoid Metabolism

ABSTRACTSynthetic glucocorticoids are widely used for their anti-inflammatory and immunosuppressive actions. A possible unwanted effect of glucocorticoid treatment is suppression of the hypothalamic-pituitary-adrenal axis, which can lead to adrenal insufficiency. Factors affecting the risk of glucocorticoid induced adrenal insufficiency (GI-AI) include the duration of glucocorticoid therapy, mode of administration, glucocorticoid dose and potency, concomitant drugs that interfere with glucocorticoid metabolism, and individual susceptibility. Patients with exogenous glucocorticoid use may develop features of Cushing’s syndrome and, subsequently, glucocorticoid withdrawal syndrome when the treatment is tapered down. Symptoms of glucocorticoid withdrawal can overlap with those of the underlying disorder, as well as of GI-AI. A careful approach to the glucocorticoid taper and appropriate patient counseling are needed to assure a successful taper. Glucocorticoid therapy should not be completely stopped until recovery of adrenal function is achieved. In this review, we discuss the factors affecting the risk of GI-AI, propose a regimen for the glucocorticoid taper, and make suggestions for assessment of adrenal function recovery. We also describe current gaps in the management of patients with GI-AI and make suggestions for an approach to the glucocorticoid withdrawal syndrome, chronic management of glucocorticoid therapy, and education on GI-AI for patients and providers.

scholarly journals Management of endocrine disease: Glucocorticoid-induced adrenal insufficiency: Replace while we wait for evidence?

2021 ◽  
Author(s):  
Kristina Laugesen ◽  
Leonie H.a. Broersen ◽  
Simon Bøggild Hansen ◽  
Olaf M Dekkers ◽  
Henrik Toft Sørensen ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Randomized Trials ◽  
Current Evidence ◽  
Clinical Implications ◽  
High Dose ◽  
Biochemical Tests ◽  
Glucocorticoid Treatment ◽  
Prevalence Studies ◽  
Anti Inflammatory ◽  
Biochemical Testing

Glucocorticoids are, besides non-steroidal anti-inflammatory drugs, the most widely used anti-inflammatory medications. Prevalence studies indicate substantial use of both systemic and locally acting agents. A recognized adverse effect of glucocorticoid treatment is adrenal insufficiency, which is highly prevalent based on biochemical testing, but its clinical implications are poorly understood. Current evidence, including randomized trials and observational studies, indicates substantial variation among patients in both risk and course of glucocorticoid-induced adrenal insufficiency, but both are currently unpredictable. Oral and intra-articular formulations, as well as long-term and high-dose treatments, carry the highest risk of glucocorticoid-induced adrenal insufficiency defined by biochemical tests. However, no route of administration, treatment duration, or dose can be considered without risk. More research is needed to estimate the risk and temporal pattern of glucocorticoid-induced adrenal insufficiency, to investigate its clinical implications, and to identify predictors of risk and prognosis. Randomized trials are required to evaluate whether hydrocortisone replacement therapy mitigates risk and symptoms of glucocorticoid-induced adrenal insufficiency in patients discontinuing glucocorticoid treatment. This review aims to provide an overview of available evidence, pointing to knowledge gaps and unmet needs.

scholarly journals A Case of Unusual Initial Presentation of Panhypopituitarism From Metastatic Adenocarcinoma of Lungs

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A563-A564
Author(s):  
Malik Waseem Ahmad ◽  
Susannah Becker
Keyword(s):  
Lung Cancer ◽  
Pituitary Gland ◽  
Diabetes Insipidus ◽  
Adrenal Insufficiency ◽  
Metastatic Disease ◽  
Vision Loss ◽  
Peripheral Vision ◽  
High Dose ◽  
Metastatic Adenocarcinoma ◽  
Sellar Mass

Abstract Introduction: Metastatic disease of the pituitary gland account for about 1 to 2 percent of the sellar masses with suprasellar extension. Approximately 7 percent of the patients are symptomatic with varying symptoms based on the location and extent of metastases. We present you a case in which patient presented with features of Panhypopituitarism starting with severe hypothyroidism in a previously undiagnosed lung cancer. The Patient also suffered with some serious complications such as diabetes insipidus, adrenal insufficiency and complete vision loss. Clinical Case: A 60 y/o male with a past medical history of Hypertension, Chronic Obstructive Pulmonary Disease, smoking 2 packs of cigarettes per day for 40 years, second-degree heart block requiring a pacemaker, Chronic kidney disease 3b presented to the emergency department with complaints of left ankle pain, swelling, and syncope. The Patient was admitted for further workup and Endocrinology was consulted for concerns of hypothyroidism with severe myxedema and adrenal insufficiency. CT head was done, which showed sellar and suprasellar mass lesion measuring 1.8 x 2.2 x 3.0 cm. Finding were confirmed on MRI. Pertinent labs were PTH 57.7 pg/ml, TSH 0.33 uIU/ml, Free T4 0.4 ng/dl, ACTH 8 pg/ml, Cortisol 2.5 ug/dl, FSH 0.5 MIU/ml, LH 0.1 MIU/ml, testosterone 4 ng/dl, Prolactin 17.7 ng/ml, Insulin-like GFBP-3: 2.1 mg/L. The Patient was started on high dose Hydrocortisone, IV Levothyroxine T4 and desmopressin for Diabetes Insipidus. The Patient complained of peripheral vision loss. Neurosurgery partially resected the sellar mass through the transsphenoidal approach. Histopathology came back with Metastatic adenocarcinoma. Further clinical course was complicated by complete vision loss from increase in the sellar mass size after 8 weeks. Patient received radiation therapy but unfortunately there was no significant improvement in the vision. Conclusion: This case highlights some serious complication from metastatic disease of Pituitary gland from lung cancer. There is a need for continued annual screening for dysfunction of the hypothalamic-pituitary axis to monitor therapy. This case also highlights the importance of widespread screening of smokers in accordance with the standard lungs cancer screening recommendations. This can potentially prevent some of the serious complications of metastatic lung disease. Reference: Ross, D. Cooper, D. Mulder, J. (2020, September) Central Hypothyroidism. https://www.uptodate.com/contents/central-hypothyroidismSnyder, P. Cooper, D. Martin, K. (2020, September) Causes, presentation, and evaluation of sellar masses. https://www.uptodate.com/contents/causes-presentation-and-evaluation-of-sellar-masses

scholarly journals Adrenal Insufficiency Secondary to Septic Shock in a Male Patient with Iatrogenic Cushing’s Syndrome: 2 sides of the Same Coin?

2021 ◽  
Vol 14 ◽  
pp. 117955142110266
Author(s):  
Luca Foppiani
Keyword(s):  
Septic Shock ◽  
Adrenal Insufficiency ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Stressful Events ◽  
Glucocorticoid Therapy ◽  
Relative Adrenal Insufficiency ◽  
Glucocorticoid Treatment ◽  
Life Saving ◽  
Severe Infections

Cushing’s syndrome causes increased morbidity and mortality due to cardiovascular and infectious diseases. Exogenous Cushing’s syndrome can render the adrenal glands unable to cope with severe infections and may result in Addisonian crisis, which can be fatal if not properly diagnosed and treated. During hospitalization for disease exacerbation, a man on chronic glucocorticoid therapy for Crohn’s disease and Cushingoid features developed polymicrobial septic shock together with hypotension that was unresponsive to fluids. On suspicion of relative adrenal insufficiency (cortisol levels were “inadequately” normal), intravenous hydrocortisone was started; norepinephrine was also required to normalize blood pressure. Following clinical improvement, oral cortisone acetate was started. On discharge, he was instructed on how to manage stressful events by increasing oral glucocorticoid treatment or starting a parenteral formulation, if required. Chronic glucocorticoid therapy can cause severe side-effects; in addition, hypoadrenalism can occur in critical illnesses (eg, severe infections). Prompt recognition and proper therapy of this condition can be life-saving.

[18F]Fluoride PET Indicates Reduced Bone Formation in Severe Glucocorticoid-induced Osteoporosis

1998 ◽  
Vol 37 (02) ◽  
pp. 76-79 ◽  
Author(s):  
T. D. Kirchhoff ◽  
W. Burchert ◽  
J. v. d. Hoff ◽  
H. Zeidler ◽  
H. Hundeshagen ◽  
...  
Keyword(s):  
Bone Formation ◽  
Multiple Organ ◽  
High Dose ◽  
Multiple Fractures ◽  
Glucocorticoid Treatment ◽  
Organ Manifestations ◽  
Diagnostic Benefit ◽  
Sharp Syndrome ◽  
18F Fluoride

SummaryA 61-year-old female patient presenting with mixed connective tissue disease (Sharp syndrome), underwent a long-term high dose glucocorticoid treatment because of multiple organ manifestations. Under steroid therapy she developed severe osteoporosis resulting in multiple fractures. A dynamic [18F]fluoride PET study in this patient revealed reduced fluoride influx in non-fractured vertebrae. This finding corresponds to pathogenetic concepts which propose an inhibition of bone formation as major cause of glucocorticoid-induced osteoporosis. In the light of the presented case it seems to be promising to evaluate the diagnostic benefit of [18F]fluoride PET in osteoporosis.

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