Introduction: Immunoglobulin (Ig)G4-related disease belongs to a rare compilation of conditions in rheumatology and may involve a multitude of organs. Amidst the central nervous system (CNS) presentation, involvement of spinal cord is rarer still. Case Report: A 50-year-old male came with chief complaints of tingling sensation over both soles since 2 months associated with lower back ache and gait disturbance in the form of spastic gait. X-rays of spine were suggestive of a growth at the level of D10–D12 compressing the spinal cord with no focal sclerotic/lytic lesions, and MRI dorsolumbar spine showed dural tail sign. The patient underwent excision of dural mass, and histopathology revealed majority of plasma cells positive for IgG4. Case 2: A 65-year-old female came with complaints of cough, shortness of breath, and fever on and off since 2 months. No history of hemoptysis, purulent sputum, weight loss. On examination: There were bilateral rhonchi in the left upper zone. MRI spine showed focal erosion with soft-tissue thickening at the right paravertebral region extending from D5-9. The patient underwent surgery (fusion D6-8 and ostectomy D7 posterior rib resection D7 right side) with the right pleural biopsy and transpendicular intracorporal biopsy D7. Histopathology was compatible with findings of IgG4 disease. Discussion: IgG4 tumors presenting in CNS itself is rare and rarer in spinal cord. Histopathological examination is central to diagnose and prognosticate the disease further as IgG4-related disease manifestations may recur without proper treatment. Keywords: IgG4, spinal mass, central nervous system, pleura.
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