Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is a rare condition with symptoms often mimicking malignancy, infection, or other autoimmune diseases. This case report describes the unique case of a 62-year-old male initially diagnosed with IgG4-SC, followed by subsequent diagnosis of cholangiocarcinoma. Biliary tract cancer in the setting of IgG4 related disease has been previously described; however, this patient course is novel as it encompasses the spectrum of challenges in IgG4-SC management, including diagnostic uncertainty, risk of infection with immunosuppressive agents, and development of malignancy diagnosed shortly following IgG4-SC diagnosis. We review the literature of management, outcomes, and malignancy risk and furthermore, highlight a promising recent therapy in treatment of IgG4 related disease, inebilizumab.

STUDY DESIGN: This is a case series. PURPOSE: We wanted to identify variations in the practice patterns among neurosurgeons and orthopedic surgeons for the management of spinal disorders. OVERVIEW OF LITERATURE: Spinal disorders are common in the clinical practice of both neurosurgeons and orthopedic surgeons. It has been observed that despite the availability of various guidelines, there is lack of consensus among surgeons about the management of various disorders. METHODS: A questionnaire was distributed, either directly or via e-mail, to the both the neurosurgeons and orthopedic surgeons who worked at 5 tertiary care centers within a single region of Korea. The surgeons were working either in private practice or in academic institutions. The details of the questionnaire included demographic details and the specialty (orthopedic/neurosurgeon). The surgeons were classified according to the level of experience as up to 5 years, 6-10 years and > 10 years. Questions were asked about the approach to lumbar discectomy (fragmentectomy or aggressive disc removal), using steroids for treating discitis, the fusion preference for spondylolisthesis, the role of an orthosis after fusion, the preferred surgical approach for spinal stenosis, the operative approach for spinal trauma (early within 72 hours or late > 72 hours) and the role of surgery in complete spinal cord injury. The data was analyzed using SPSS ver 16. p-values < 0.05 were considered to be significant. RESULTS: Of the 30 surgeons who completed the questionnaire, 20 were neurosurgeons and 10 were orthopedic surgeons. Statistically significant differences were observed for the management of spinal stenosis, spondylolisthesis, using an orthosis after fusion, the type of lumbar discectomy and the value of surgical intervention after complete spinal cord injury. CONCLUSIONS: Our results suggest that there continues to exist a statistically significant lack of consensus among neurosurgeons and orthopedic spine surgeons when considering using an orthosis after fusion, the type of discectomy and the value of intervention after complete spinal injury.
Consensus ; Discitis ; Diskectomy ; Electronic Mail ; Korea ; Orthopedics ; Orthotic Devices ; Private Practice ; Spinal Cord ; Spinal Cord Injuries ; Spinal Injuries ; Spinal Stenosis ; Spine ; Spondylolisthesis ; Steroids ; Tertiary Care Centers ; Surveys and Questionnaires