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

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 thegallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe anIgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused byIgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed denselymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response tothe administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case inliterature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response ofIgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longestreported period of follow-up reported in the literature to date