IgG4-Related Sclerosing Disease Causing Spinal Cord Compression: The First Reported Case in Literature

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.

Successful treatment of IgG4‐related hypertrophic pachymeningitis with induction rituximab and dexamethasone followed by maintenance rituximab

“…IgG4RD is a rare polyclonal lymphoproliferative disorder, 1 which commonly involves the pancreas, lungs, thyroid gland, lymph nodes, extra pancreatic bile ducts, retroperitoneum, aorta, and lacrimal and salivary glands. 6,7 Intracranial involvement is rare and most commonly involves hypertrophic pachymeningitis and hypophysitis. 8 In a Japanese survey, the prevalence of hypertrophic pachymeningitis was about 1 case per 1 000 000 with 8.8% of the cases having IgG4RD as the underlying etiology.…”

Immunoglobulin G4-Related Disease Masquerading an Epidural Spinal Cord Tumor

“…[1][2][3][4][5][6] Other possible findings are an increased serum IgG4 levels, an IgG4/IgG proportion greater than 40%, and IgG4+ plasma cells infiltration of organs. [1][2][3][4][5][6] However, the sensitivity and specificity of serum IgG4 in the diagnosis of IgG4-RD are uncertain. 1 Steroid administration is the mainstay treatment for IgG4-RD and leads to a dramatic clinical response in most cases.…”

“…IgG4-RD is a rare, potentially underdiagnosed cause of pseudotumor-like lesions. [1][2][3][4][5][6] The condition is defined by fibrosis induced by IgG4+ plasma cells, which may ultimately contribute to end-organ failure. 2 IgG4-RD manifests predominately in the pituitary gland, brain parenchyma, meninges, peripheral nerves, and rarely less often epidural tissue in the central nervous system.…”

“…2 IgG4-RD manifests predominately in the pituitary gland, brain parenchyma, meninges, peripheral nerves, and rarely less often epidural tissue in the central nervous system. [2][3][4][5][6] IgG4-related epidural IPT may present with varied symptoms depending upon how severe the compression is and on the segment of the spinal cord affected. 3,5 In the case discussed here, the patient stated back pain that eventually led to paraparesis and bilateral leg hypoesthesia.…”

“…1,2 It usually involves the pancreas, hepatobiliary tract, lungs, salivary gland, orbit, and lymph nodes. [1][2][3][4] Spinal epidural tissue involvement is extremely rare. 1,[3][4][5][6] This report aims to present a rare case occurrence of IgG4-related epidural inflammatory pseudotumor (IPT), causing compression on the spinal cord in a young male with a relevant literature review.…”