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      Thickened Pituitary Stalk Associated with a Mass in the Sphenoidal Sinus: An Alarm to Suspect Hypophysitis by Immunoglobulin G4?

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          Introduction Hypophysitis is a chronic inflammation of the pituitary gland of complex and still incompletely defined pathogenesis. It belongs to the group of non-hormone-secreting sellar masses, sharing with them comparable clinical presentation and radiographic appearance.

          Objectives Describe the case of immunoglobulin G4 (IgG4)-related hypophysitis presenting as a mass in the sphenoid sinus.

          Resumed Report A 40-year-old Brazilian man had a diagnosis of central diabetes insipidus since 2001 associated with pituitary insufficiency. Pituitary magnetic resonance imaging revealed a centered pituitary stalk with focal nodular thickening and the presence of heterogeneous materials inside the sphenoid sinus. The patient was treated with testosterone replacement therapy. Laboratory results revealed increased IgG4 serum.

          Conclusion IgG4-related hypophysitis should be considered in patients with pituitary insufficiency associated with sellar mass and/or thickened pituitary stalk. IgG4 serum measurement for early diagnosis of IgG4-related hypophysitis should be performed.

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          Most cited references 16

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          A new clinicopathological entity of IgG4-related autoimmune disease.

          Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies. In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls. These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases.
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            Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

            Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team) and the other from lymph proliferation (Umehara team) supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC) for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP), compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease) and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.
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              IgG4-Related Disease

              Over the past decade and with increasing pace in the last few years, a “new” disease has emerged, gradually affecting a wide range of medical specialties and explaining a host of conditions previously regarded as separate entities. This newly recognized condition is IgG4-related disease (IgG4-RD), a potentially multiorgan disorder that is characterized by elevated serum IgG4 concentrations in the majority of cases. IgG4-RD was recognized in modern times in Japan through a series of seminal observations that occurred during the 1990s and the first few years of this century [1–5], but it is clear in reviewing the medical literature that IgG4-RD has been present and reported upon in various guises going back at least to the 1800s [6–11]. In addition to the frequent elevations of serum IgG4 concentrations, certain major pathologic hallmarks are generally present to one degree or another across all organ systems, providing the principal foundation for the belief that the disparate organ manifestations associated with this diagnosis are in fact part of the same systemic disease. These pathologic features include a lymphoplasmacytic infiltrate with a high percentage of plasma cells within the lesion staining for IgG4; a peculiar pattern of fibrosis known as “storiform” fibrosis; a tendency to affect veins in a manner that leads to obliterative phlebitis; and mild to moderate tissue eosinophilia [12]. IgG4-RD appears to sit at an intersection between different inflammatory pathways. Many but not all patients have substantial allergic or atopic histories, and early indications are that a “modified” Th2 response is critical to this condition [13]. Other patients also develop tumefactive lesions leading to misdiagnoses of cancer. Still others have clinical manifestations and serological findings that lead to erroneous classifications of their diagnoses as “connective tissue diseases.” The full links between the various inflammatory players in this symphony of inflammation remain to be fully elucidated. It is likely that a broader understanding of the ways in which B and T cells, fibroblasts, plasma cells, immune complexes, and other elements interact in IgG4-RD will provide important insights into the nature of its individual inflammatory constituents and the broader immune system. IgG4-RD is now recognized as a worldwide disease [14]. The international community convened in Boston in 2011 to compare notes, share experiences, and plan ways for moving ahead in understanding this condition. Building upon crucial earlier work in Japan, consensus papers pertaining to the nomenclature of this condition and to its pathological features have been published [12, 15]. Japanese investigators have also published diagnostic criteria for IgG4-RD [16]. In this special issue, we are pleased to present more than two dozen papers on IgG4-RD that address a number of facets of this condition: from its clinical manifestations to its radiologic features; from its pathology hallmarks to its serologic characteristics; and from its diagnostic challenges to early indications of treatment success. These papers capture the essence of IgG4-RD in 2012 and represent the current state-of-the-art against which future advances will be compared. John H. Stone John K. C. Chan Vikram Deshpande Kazuichi Okazaki Hisanori Umehara Yoh Zen

                Author and article information

                Int Arch Otorhinolaryngol
                Int Arch Otorhinolaryngol
                International Archives of Otorhinolaryngology
                Thieme Publicações Ltda (Rio de Janeiro, Brazil )
                05 March 2015
                July 2015
                : 19
                : 3
                : 273-276
                [1 ]Neurosurgical Unit, Universidade de São Paulo, São Paulo, SP, Brazil
                [2 ]Funcional Neurosurgery Unit, Universidade de São Paulo, São Paulo, SP, Brazil
                Author notes
                Address for correspondence Rafael Loch Batista, MD Neurosurgical Unit, USP Alameda Joauime Eugênio de Lima, 1058/102, Sao Paulo, Sao Paulo 01403002Brazil r-loch@ 123456uol.com.br rafael.loch@ 123456hc.fm.usp.br
                © Thieme Medical Publishers

                pituitary diseases, hypopituitarism, sphenoid sinus


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