scholarly journals Rituximab treatment for isolated IgG4-related hypophysitis in a teenage female

2018 ◽  
Vol 2018 ◽  
Author(s):  
Danielle R Bullock ◽  
Bradley S Miller ◽  
H Brent Clark ◽  
Patricia M Hobday
Keyword(s):  
Endocrine Function ◽  
List Type ◽  
Pituitary Mass ◽  
Related Disease ◽  
Pituitary Dysfunction ◽  
Igg4 Related Disease ◽  
Pituitary Disease ◽  
Appropriate Treatment ◽  
Organ Systems ◽  
Progression Of Disease

Summary IgG4-related hypophysitis is an important diagnostic consideration in patients with a pituitary mass or pituitary dysfunction and can initially present with headaches, visual field deficits and/or endocrine dysfunction. Isolated IgG4-related pituitary disease is rare, with most cases of IgG4-related disease involving additional organ systems. We report the case of a teenage female patient with isolated IgG4-related hypophysitis, diagnosed after initially presenting with headaches. Our patient had no presenting endocrinologic abnormalities. She was treated with surgical resection, prednisolone and rituximab with no further progression of disease and sustained normal endocrine function. This case, the youngest described patient with isolated IgG4-related hypophysitis and uniquely lacking endocrinologic abnormalities, adds to the limited reports of isolated pituitary disease. The use of rituximab for isolated pituitary disease has never been described. While IgG4-related hypophysitis has been increasingly recognized, substantial evidence concerning the appropriate treatment and follow-up of these patients is largely lacking. Learning points: IgG4-related hypophysitis most often occurs in the setting of additional organ involvement but can be an isolated finding. This diagnosis should therefore be considered in a patient presenting with pituitary abnormalities. Most patients with IgG4-related hypophysitis will have abnormal pituitary function, but normal functioning does not exclude this diagnosis. Corticosteroids have been the mainstay of therapy for IgG4-related disease, with other immunosuppressive regimens being reserved for refractory cases. Further research is needed to understand the effectiveness of corticosteroid-sparing regimens and whether there is utility in using these agents as first-line therapies.

scholarly journals Deterioration of pituitary function without relapse after steroid therapy for IgG4-related hypophysitis

2021 ◽  
Vol 2021 ◽  
Author(s):  
Nobuyuki Nishi ◽  
Ken Takeshima ◽  
Shuhei Morita ◽  
Hiroshi Iwakura ◽  
Masahiro Nishi ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Steroid Therapy ◽  
Continuous Monitoring ◽  
Laboratory Data ◽  
Pituitary Function ◽  
List Type ◽  
Related Disease ◽  
Pituitary Dysfunction ◽  
Igg4 Related Disease

Summary IgG4-related hypophysitis is an autoimmune hypophysitis associated with IgG4-related disease. Swelling of the pituitary gland is responsive to steroid therapy, but the prognosis of pituitary function after the treatment remains unclear. The present case implies that transiently improved pituitary function can re-worsen during long-term follow-up in IgG4-related hypophysitis. A 71-year-old male patient with IgG4-related hypophysitis visited a nearby hospital with malaise, anorexia, and polyuria. Pituitary dysfunction was suspected, so he was referred to our hospital for further examination. Imaging studies and laboratory data showed swelling of the pituitary gland and panhypopituitarism, which dramatically improved following steroid therapy. There was no evidence of relapsing IgG4-related disease during prednisolone tapering. Pituitary function was examined after 4 years under treatment with low-dose prednisolone; surprisingly, anterior pituitary function had worsened again. Our case suggests a need for continuous monitoring of pituitary function after steroid therapy for IgG4-related hypophysitis. This report illustrates the natural course of pituitary function in IgG4-related hypophysitis and may be informative when considering the introduction of steroid therapy. Learning points Steroid therapy is an effective first-line therapy for pituitary dysfunction and pituitary swelling in IgG4-related hypophysitis. Pituitary function can worsen again during follow-up, despite transient improvement after steroid therapy in IgG4-related hypophysitis. Continuous monitoring of pituitary function is necessary for IgG4-related hypophysitis, regardless of disease activity.

scholarly journals IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Naoshi Nishina ◽  
Yuko Kaneko ◽  
Masataka Kuwana ◽  
Hironari Hanaoka ◽  
Hideto Kameda ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Histopathological Examination ◽  
High Serum ◽  
Cell Infiltration ◽  
Pituitary Mass ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Plasma Cell Infiltration ◽  
Serum Igg4 ◽  
Systemic Lymphadenopathy

IgG4-related disease is a new disease group that affects multiple organs. It is characterized by high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells in the affected organ. Here, we describe an intriguing case that suggested that IgG4-related disease might present without IgG4 overexpression or infiltration, at least during a relapse. A 47-year-old man had been diagnosed with systemic lupus erythematosus 15 years. He was admitted due to a pituitary mass, systemic lymphadenopathy, and multiple nodules in the lungs and kidneys. The serum IgG4 level was normal and histopathological examination of the pituitary mass showed abundant lymphocyte and plasma cell infiltration with very few IgG4-positive cells. When we examined specimens preserved from 15 years ago, we found high serum IgG4 levels and IgG4-bearing plasma cell infiltration. This resulted in a diagnosis of IgG4-related disease, and we considered the current episode to be a relapse without IgG4 overexpression. This case indicated that, to clarify the pathogenesis of IgG4-related disease, current cases should repeat specimen evaluations over the course of IgG4-related disease to define diagnostic markers.

IgG4-related disease: a great mimicker of lung cancer

2021 ◽  
Vol 14 (2) ◽  
pp. e239976
Author(s):  
Natalie Louise Simon ◽  
Mostafa Negmeldin
Keyword(s):  
Lung Cancer ◽  
Inflammatory Infiltrate ◽  
Haematological Malignancy ◽  
Multiple Organ ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Ct Findings ◽  
Organ Systems ◽  
Pulmonary Manifestations ◽  
Storiform Fibrosis

IgG4-related disease is a rheumatological disorder, affecting multiple organ systems, and displaying dense lymphoplasmacytic inflammatory infiltrate and storiform fibrosis on histology. The pulmonary manifestations of IgG4-related disease are varied. Most commonly, bronchovascular consolidation and perilymphatic parenchymal thickening occur. IgG4-related disease can present as a solitary parenchymal mass and is often mistaken for a primary pulmonary or haematological malignancy. This report presents a case of IgG4-related disease in a patient with 6 months reported haemoptysis symptomatology and CT findings of perihilar lymphadenopathy, multiple pulmonary parenchymal nodules and a single parenchymal mass. Clinician cognisance of the histopathological presentation of IgG4-disease is important. It should be considered as a differential in patients for which investigations have ruled out malignancy, as it is responsive to glucocorticoids in the majority of cases.

scholarly journals MON-264 Pleotropic Clinical Presentation in Two Brazilians Patients with Confirmed IgG4-Related Hypophysitis

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Rafael de Oliveira ◽  
Pedro Gomes de Vasconcelos Silva ◽  
Daniel Bortolin Muller ◽  
Sheila Aparecida Coelho Siqueira ◽  
Rosely Antunes Patzina ◽  
...  
Keyword(s):  
Pituitary Gland ◽  
Hypogonadotropic Hypogonadism ◽  
Laboratory Evaluation ◽  
Pituitary Mass ◽  
Central Hypothyroidism ◽  
Azathioprine Therapy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Meningeal Inflammation ◽  
Serum Igg4

Abstract Background: Hypophysitis is a rare condition characterized by inflammation of the pituitary gland, usually resulting in hypopituitarism and pituitary enlargement with mass effect symptoms. IgG4-related hypophysitis can occur alone or as part of a multiorgan disease. Treatment with glucocorticoids is effective in 97% of the cases in reducing pituitary mass. Clinical cases: Case #1. A 56-yrs man with previous diagnosis of Mikulicz syndrome was referred to our service with fatigue and erectile dysfunction. Laboratory evaluation revealed hypogonadotropic hypogonadism, hyperprolactinemia (PRL=108 ng/mL) and central hypothyroidism. Sellar MRI depicted a pituitary mass with pituitary stalk thickening and a homogeneous uptake of gadolinium. During clinical follow-up, he also presented retroperitoneal fibrosis and IgG4-related disease was confirmed by serum IgG4 elevation and a pathological review of the previous salivary gland biopsy.Prednisone 80 mg/d treatment was initiated, with recovery of the thyrotrophic axis, reduction of PRL levels and significant reduction of the pituitary lesion. Due to maintenance of inflammatory activity and worsening of renal function, azathioprine therapy was associated, with subsequent inclusion of rituximab. Case #2. A 16-yrs boy was referred to our service presenting severe headache, bilateral visual deficit, right eyelid ptosis, hyposmia, polyuria and polydipsia. Cranial MRI depicted an extensive skull base mass involving pituitary gland, optic nerves, cavernous sinuses, olfactory bulb and clivus). Hormonal evaluation confirmed normoprolactinemia, hypogonadotropic hypogonadism and diabetes insipidus. Biopsy of the lesion revealed meningeal inflammation with immunohistochemistry suggesting IgG4-related sclerosing disease. No other organs were affected. An important lesion reduction and gonadotropic axis recovery occurred after 40 days of prednisone 60mg/d. After the drug withdrawal, methotrexate was introduced. However, after three years, headache and hyposmia recurred. A new MRI revealed increase of lesion and mycophenolate and rituximab were initiated, with clinical improvement without recurrences over time. Conclusion: Although a rare disease, IgG4-related disease should be included in the differential diagnosis amongst pituitary masses, with or without other affected organs. Immunosuppression with corticosteroids is the first treatment choice and other alternatives must be used in case of persistence of disease activity or relapse. These are very few Brazilian patients reported with IgG4 related disease. We described two cases with IgG4-related hypophysitis: one young patient, without involvement of other organs and another of middle age with systemic involvement, reinforcing the pleotropic clinical picture. Both required rituximab therapy due to disease progression.

IgG4-Related Appendiceal Disease: A First Case Report Fulfilling All Pathological Diagnostic Criteria and With Concomitant S100-Positive Dendritic/Schwann Cell Hyperplasia

2019 ◽  
Vol 27 (5) ◽  
pp. 541-546
Author(s):  
Dong Hyang Kwon ◽  
Bhaskar Kallakury ◽  
Pedro DeBrito ◽  
Norio Azumi
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cell ◽  
High Power Field ◽  
Surgical Morbidity ◽  
Cell Hyperplasia ◽  
Cell Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
First Case ◽  
Organ Systems

IgG4-related disease is a recent entity that has been described in a wide variety of organ systems. A 46-year-old female presented with acute appendicitis accompanied by a mass-forming lesion, raising a concern for neoplasm, and therefore, hemicolectomy was performed. The lesion revealed a dense lymphoplasmacytic infiltrate accompanied by storiform fibrosis and obliterative phlebitis. The IgG4/IgG plasma cell ratio was >50%, and the number of IgG4-positive plasma cells was >100/high-power field. In order to assess the IgG4/IgG plasma cell ratio, MUM1 was employed instead of IgG to successfully estimate the plasma cell concentration. There was also a concomitant hyperplasia of S100-positive cell, which could represent dendritic or Schwannian origin and possibly play a pathophysiologic role. The hyperplasia was significant by itself that it may mimic a mass-forming lesion. This newly described entity of the past decade deserves increased recognition due to clinical implication and surgical morbidity. This is the first case of IgG4-related disease in the appendix to our knowledge that fully satisfied all the pathological diagnostic criteria. We would like to also highlight our innovative approach of evaluating the IgG4/IgG plasma cell.

scholarly journals IgG4-related thyroiditis: a case report and review of literature

2014 ◽  
Vol 2014 ◽  
Author(s):  
Mahmud Abo Salook ◽  
Carlos Benbassat ◽  
Yulia Strenov ◽  
Amit Tirosh
Keyword(s):  
Hashimoto’S Thyroiditis ◽  
Systemic Disease ◽  
Hashimoto's Thyroiditis ◽  
List Type ◽  
Review Of Literature ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Aggressive Form ◽  
Learning Points ◽  
Very High

Summary A 55-year-old male, with a positive medical history for hypothyroidism, treated with stable doses for years was admitted with subacute thyroiditis and a feeling of pain and pressure in the neck. Laboratory tests showed decrease in TSH levels, elevated erythrocyte sedimentation rate, and very high antithyroid antibodies. Owing to enlarging goiter and exacerbation in the patient's complaints, he was operated with excision of a fibrotic and enlarged thyroid lobe. Elevated IgG4 plasma levels and high IgG4/IgG plasma cell ratio on immunohistochemistry led to the diagnosis of IgG4-mediated thyroiditis. We concluded that IgG4-thyroiditis and IgG4-related disease should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Learning points IgG4-related disease is a systemic disease that includes several syndromes; IgG4-related thyroiditis is one among them. IgG4-thyroiditis should be considered in all patients with an aggressive form of Hashimoto's thyroiditis. Patients with suspected IgG4-thyroiditis should have blood tested for IgG4/IgG ratio and appropriate immunohistochemical staining if possible.

scholarly journals Histiocytosis masquerading in the mesentery and pleura

2019 ◽  
Vol 12 (11) ◽  
pp. e232165
Author(s):  
Nina Dhillon ◽  
Brian Skinnider ◽  
Saman Darabian ◽  
Mollie Carruthers
Keyword(s):  
Peritoneal Carcinomatosis ◽  
Unknown Primary ◽  
Atypical Presentation ◽  
Diagnostic Dilemma ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Biopsy Specimens ◽  
Appropriate Treatment ◽  
Underlying Malignancy ◽  
Rosai Dorfman Disease

We present an atypical presentation of Rosai-Dorfman disease (RDD). Due to its overlap with IgG4-related disease (IgG4-RD), this case proved to be a diagnostic dilemma. Our case is an example of the importance of having a broad-based differential and, ultimately, an in-depth histopathological review. Our patient presented with a constellation of symptoms suggestive of an underlying malignancy. He was provisionally diagnosed with peritoneal carcinomatosis of an unknown primary. His initial presentation triggered a series of investigations, surgery and biopsies. Omental biopsy specimens were suggestive of IgG4-RD. Despite appropriate treatment for IgG4-RD, his disease progressed, specifically in the lungs. Pleural biopsies were then collected and assessed alongside the omental biopsies. On review and reassessment, the patient was formally diagnosed with RDD.

scholarly journals Pituitary abscess: a case report and review of the literature

2016 ◽  
Vol 2016 ◽  
Author(s):  
Apostolos K A Karagiannis ◽  
Fotini Dimitropoulou ◽  
Athanasios Papatheodorou ◽  
Stavroula Lyra ◽  
Andreas Seretis ◽  
...  
Keyword(s):  
Transsphenoidal Surgery ◽  
List Type ◽  
Definite Diagnosis ◽  
Review Of The Literature ◽  
Pituitary Abscess ◽  
Pituitary Mass ◽  
Inflammatory Infiltration ◽  
Pituitary Dysfunction ◽  
Anterior Pituitary Function ◽  
Learning Points

Summary Pituitary abscess is a rare life-threating entity that is usually misdiagnosed as a pituitary tumor with a definite diagnosis only made postoperatively. Over the last several decades, advances in healthcare have led to a significant decrease in morbidity and mortality due to pituitary abscess. We report a case of a 34-year-old woman who was admitted to our department for investigation of a pituitary mass and with symptoms of pituitary dysfunction, headaches and impaired vision. During her admission, she developed meningitis-like symptoms and was treated with antibiotics. She eventually underwent transsphenoidal surgery for excision of the pituitary mass. A significant amount of pus was evident intraoperatively; however, no pathogen was isolated. Six months later, the patient was well and had full recovery of the anterior pituitary function. Her menses returned, and she was only on treatment with desmopressin for diabetes insipidus that developed postoperatively. Learning points Pituitary abscess is a rare disease and the reported clinical features vary mimicking other pituitary lesions. The diagnosis of pituitary abscess is often very difficult to make and rarely included in the differential. The histological findings of acute inflammatory infiltration confirm the diagnosis of pituitary abscess. Medical and surgical treatment is usually recommended upon diagnosis of a pituitary abscess.

Serum IgG4 levels outperform IgG4/IgG RNA ratio in differential diagnosis of IgG4-related disease

2020 ◽  
Author(s):  
L Schulte ◽  
F Arnold ◽  
F Siegel ◽  
J Backhus ◽  
L Perkhofer ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4
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