IgG4-Related Disease Presenting as Hypertrophic Pachymeningitis

Abstract

IgG4-related disease (IgG4-RD) is a multi-organ, immune-mediated inflammatory condition of unknown etiology characterized by infiltration of tissues by IgG4 producing plasma cells. IgG4-related disease (IgG4-RD) can ideally affect any organs, but the involvement of the central nervous system (CNS) is a rare entity. We present a case of a 67-year-old male who presented with diplopia with imaging showing hypertrophic pachymeningitis (HPM) and was diagnosed with IgG4-RD of the CNS based on histopathology report.

Neuro-Ophthalmologic Igg4-Related disease

“…Meningeal involvement may be contiguous with disease in the orbit or pituitary gland and these patients may show elevations of cerebrospinal fluid IgG4 level, indicating intrathecal production of IgG4. On MRI, the dura may show smooth thickening with homogeneous diffuse linear enhancement (as in the case presented), or focal nodular thickening, mimicking a meningioma, and may result in compression of surrounding structures, especially cranial nerves [15].…”

Case report of a patient with Erdheim-Chester disease presenting with neuro-endocrine symptoms and negative for BRAF mutation

“…CNS-IgG4-RD [10] Central nervous system and systemic involvement (e.g., pancreas, salivary glands, kidneys, lungs) Periorbital pseudotumor, pituitary inflammation, diffuse intracranial dural/dural thickening or masses.…”

The “great imitator”: IgG4‐related disease of the oral cavity. Two case reports and scoping review

“…12 Apart from the best-known manifestations of IgG4-RD in the major salivary glands, lacrimal glands and thyroid (i.e., Riedel's thyroiditis), there is an increasing number of reports describing the involvement of extraglandular tissues, such as extraorbital muscles, orbital nerves, meninges, sinonasal tract, trigeminal nerve branches, skin, ears, and larynx. [13][14][15][16][17][18][19] The oral cavity is not among the commonly reported locations in the head and neck area, and its involvement is often neglected in most reviews dealing with this topic, thus organ-specific features have not yet been identified in the international diagnostic criteria for IgG4-RD. 20 For these reasons, it is of the utmost importance that oral medicine physicians, head and neck surgeons, and oral and maxillofacial pathologists be familiar with the clinicopathological manifestations of IgG4-RD in the oral cavity, in order to avoid misdiagnosis and extensive surgical resections that may impair the patient's quality of life.…”

“…This is the case of Mikulicz's disease (MD), which is characterized by bilateral enlargement of lacrimal and salivary glands, 11 or chronic sclerosing sialadenitis (Küttner's tumor, KT), which usually presents as a firm swelling of the submandibular gland 12 . Apart from the best‐known manifestations of IgG4‐RD in the major salivary glands, lacrimal glands and thyroid (i.e., Riedel's thyroiditis), there is an increasing number of reports describing the involvement of extraglandular tissues, such as extraorbital muscles, orbital nerves, meninges, sinonasal tract, trigeminal nerve branches, skin, ears, and larynx 13–19 . The oral cavity is not among the commonly reported locations in the head and neck area, and its involvement is often neglected in most reviews dealing with this topic, thus organ‐specific features have not yet been identified in the international diagnostic criteria for IgG4‐RD 20 …”