The evaluation should include serum assays for hormones stimulating end organs in the hypothalamus–pituitary axis: luteinizing hormone and follicle stimulating hormone, adrenocorticotrophic hormone, thyroid stimulating hormone, growth hormone, prolactin, and electrolytes. Diabetes insipidus is common but may be masked. Abnormalities include isolated hypogonadism, central hypothyroidism or adrenocorticotropic hormone deficiency, or combined anterior pituitary hormone deficiencies . Hyperprolactinemia may occur due to the stalk defect.
Radiologic studies demonstrate a symmetrically enlarged pituitary gland with thickened stalk (Fig. 11). There may be absence of the normal precontrast T1 hyperintensity of the posterior pituitary gland, which can be associated with central diabetes insipidus . There is often involvement of the adjacent sphenoid sinus, meninges, or trigeminal nerve branches, which may extend into the orbit.
Although aortic disease with inflammation of the arterial wall is a well known complication IgG4-RD, involvement of the cervical and cerebral vessels is probably rare or at least usually subclinical. One patient had carotid and intracerebral inflammatory aneurysms in addition to retroperitoneal fibrosis, aortitis, and involvement of the iliac vessels . Another patient has been reported with carotid aneurysm evolving into an acute dissection . Vertebral dolichoectasia  and diffuse intracranial dilating vasculopathy with recurrent subarachnoid hemorrhage have been reported . We have seen one patient whose clinical picture closely mimicked giant cell arteritis, whose temporal artery biopsy revealed inflammation of the vessel wall with IgG4-RD pathology .
IgG4-RD generally responds well to treatment, but chronic or recurrent courses of therapy are required for many patients. It is desirable to initiate immunosuppression at a stage when the dominant histopathology is a lymphoplasmacytic infiltrate rather than an acellular fibrosis, as the latter is often treatment-refractory.
Most manifestations of IgG4-RD can be treated successfully with glucocorticoids, which are currently the first-line standard of care for this condition. On the contrary, responses to treatment with glucocorticoids are often incomplete, relapses are common, and substantial treatment-related morbidity may occur [23▪,53]. B-cell depletion with rituximab has good efficacy in IgG4-RD , and sheds light onto the role of plasma cells and their B-cell precursors in the pathogenesis of IgG4-RD . Radiotherapy may exceptionally be employed in refractory cases [23▪].
IgG4-RD is a complex and fascinating disorder that unifies diverse manifestations in multiple organ system with a shared histopathology. A variety of neuro-ophthalmic manifestations have been recognized. The diagnosis can be suspected based on a combination of a full clinical history, physical examination, and appropriate laboratory and radiologic tests, but pathologic confirmation of the diagnosis is desirable. When recognized and treated appropriately, the prognosis is usually good. Early institution of treatment to prevent chronic fibrosis or other irreversible tissue injury is important.
Papers of particular interest, published within the annual period of review, have been highlighted as:
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Retrospective pathologic case series that suggests that IgG4-RD and lymphoma can co-occur, but that lymphomas in IgG4-RD are more varied in location and type than previously suggested in Asian cohorts
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