DIAGNOSIS THROUGH OBFUSCATION: NEUROSARCOIDOSIS PRESENTING IN A PATIENT WITH RECENTLY DIAGNOSED PULMONARY SARCOIDOSIS

Authors
Category Primary study
JournalChest
Year 2022
SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Sarcoidosis is an immune-mediated disorder presenting with granulomas that can affect many organ systems. Neurosarcoidosis (NS) has clinical manifestations in up to 10% of patients and has a variable presentation which presents a diagnostic challenge (1). Here, we present a case of a patient whose NS diagnosis was obscured by chronic immunosuppressive therapy for pulmonary sarcoidosis. CASE PRESENTATION: A 57-year-old white male initially presented to rheumatology for work up of his recently diagnosed bilateral anterior uveitis. Chest imagining revealed hilar lymphadenopathy which raised concerns for pulmonary sarcoidosis. He was started on high dose steroids which were eventually transitioned to CellCept and was then referred to Pulmonary Clinic. Hilar lymph node sampling was performed, and pathology showed both caseating and non-caseating granulomas. Infectious work-up was negative and he was diagnosed with Pulmonary sarcoidosis. Shortly after beginning CellCept, the patient began getting daily headaches with fever. Due to concerns of an adverse reaction, his medication was switched to methotrexate. Further workup was performed after the patient reported fevers at home. Imaging at the time was inconclusive and lumbar puncture showed low glucose, elevated protein, and increased WBCs with 80% lymphocytes. The patient mentioned episodes of diffuse joint pain and paresthesia in his feet which progressed to involve his bilateral lower extremities. A diagnosis of probable NS was made based on his persistent neurological symptoms, negative infectious work up and CSF findings. DISCUSSION: While sarcoidosis is primarily diagnosed from its pulmonary involvement, 10% of patients are reported to have neurological involvement (1). When present, NS occurs within the first 2 years after diagnosis of systemic sarcoidosis (1). The most common symptoms of NS are peripheral and cranial neuropathy (3). Definitive diagnosis of NS requires biopsy of the nervous system. Probable diagnosis requires pathologic confirmation of systemic granulomatous disease as well as MRI, CSF, and/or EMG findings consistent with NS (1,3). Diagnosis can be obscured by side effects from medications, such as Cellcept (up to 50%), or secondary infections related to immunosuppression (2). Once diagnosed, systemic glucocorticoids are first line therapy (1). CONCLUSIONS: Neurosarcoidosis is an uncommon manifestation of systemic sarcoidosis. Consideration of medication side effects and infectious etiologies should be evaluated prior to diagnosis. Reference #1: Bradshaw MJ, Pawate S, Koth LL, Cho TA, Gelfand JM. Neurosarcoidosis. Neurol - Neuroimmunol Neuroinflammation. 2021;8(6):e1084. doi:10.1212/NXI.0000000000001084 Reference #2: CELLCEPT Prescribing Information. https://www.gene.com/download/pdf/cellcept_prescribing.pdf Reference #3: Stern BJ, Royal W III, Gelfand JM, et al. Definition and Consensus Diagnostic Criteria for Neurosarcoidosis: From the Neurosarcoidosis Consortium Consensus Group. JAMA Neurol. 2018;75(12):1546-1553. doi:10.1001/jamaneurol.2018.2295 DISCLOSURES: No relevant relationships by Chase Baxter No relevant relationships by Zachary Holliday No relevant relationships by Kevin Hooshmand
Epistemonikos ID: e6cc664549c04c380e9882a0b366dffe4758ddad
First added on: Feb 05, 2024