IgG4-related disease presenting with an epidural inflammatory pseudotumor: a case report

Abstract

BackgroundInflammatory pseudotumor is a rare clinical condition that can be related to immunoglobulin G4 disease. Only a few cases of spinal inflammatory pseudotumors have been reported in the literature and an association with immunoglobulin G4 disease was not conclusive in any of them. We describe what we believe to be the first biopsy-proven case of an epidural inflammatory pseudotumor related to immunoglobulin G4 disease.Case presentationA 57-year-old Caucasian woman presented to our hospital with severe paraparesis, gait disturbance, and sensory loss secondary to a relapsing epidural mass. Examination of a biopsy specimen revealed a lymphoplasmacytic infiltration with fibrosis and an immunoglobulin G4-positive plasma cell ratio of over 50 %, which are compatible with a diagnosis of immunoglobulin G4-related inflammatory pseudotumor. Our patient was successfully treated with systemic and epidural administration of glucocorticoids.ConclusionImmunoglobulin G4-related disease is an emerging clinical condition in which central nervous system involvement is still uncommon. We describe the case of a patient with an epidural mass with medullar compression, which was proved to be an immunoglobulin G4-related epidural inflammatory pseudotumor. Our findings suggest a new manifestation of immunoglobulin G4-related disease. This disorder should be considered in the differential diagnosis of spinal tumors as a potentially treatable condition with glucocorticoids.

Immunoglobulin G4–related epidural inflammatory pseudotumor presenting with pulmonary complications and spinal cord compression: case report

“…IPT of the epidural space is very rare and, to our knowledge, there have only been 9 previously reported cases of epidural IPT in the literature. 5,6,9,10,12,16,17,19 A search of PubMed was completed for case reports and case series involving this disease. Furthermore, only one of these cases had a suspected diagnosis of IgG4-RD.…”

“…Furthermore, only one of these cases had a suspected diagnosis of IgG4-RD. 5 Ferreira et al described the case of a patient who presented with severe paraparesis and sensory loss and was admitted to a hospital in Lisbon, Portugal, with biopsy-confirmed IgG4related IPT from T-10 to T-12. 5 This patient also required decompression laminectomy and complete resection of the mass.…”

“…5 Ferreira et al described the case of a patient who presented with severe paraparesis and sensory loss and was admitted to a hospital in Lisbon, Portugal, with biopsy-confirmed IgG4related IPT from T-10 to T-12. 5 This patient also required decompression laminectomy and complete resection of the mass. However, the patient experienced recurrence of the epidural mass on 2 separate occasions and required 2 subsequent surgeries.…”

“…22,23 A total of 9 cases of IPT involving the epidural space have been reported in the literature. 5,6,9,10,12,17,19 However, only one of these IPT cases had a confirmed diagnosis of IgG4-RD. 5 We present a rare case of a patient with IgG4-related epidural IPT with extension through the thoracic vertebrae into the right lung.…”

“…5,6,9,10,12,17,19 However, only one of these IPT cases had a confirmed diagnosis of IgG4-RD. 5 We present a rare case of a patient with IgG4-related epidural IPT with extension through the thoracic vertebrae into the right lung. This IgG4-related IPT required resection of the pulmonary mass, emergency decompression of the spinal cord, and administration of high doses of glucocorticosteroids.…”