Duke Department of Medicine, Division of Rheumatology and Immunology, Duke University, 40 Duke Medicine Cir Clinic 1J, Durham, NC, 27710, USA.
Duke Department of Neurology, Duke University, 40 Duke Medicine Cir Clinic 1L, Durham, NC, 27701, USA.
Curr Allergy Asthma Rep. 2023 Jul;23(7):399-410. doi: 10.1007/s11882-023-01092-z. Epub 2023 May 31.
Neurosarcoidosis is a rare manifestation of sarcoidosis that is challenging to diagnose. Biopsy confirmation of granulomas is not sufficient, as other granulomatous diseases can present similarly. This review is intended to guide the clinician in identifying key conditions to exclude prior to concluding a diagnosis of neurosarcoidosis.
Although new biomarkers are being studied, there are no reliable tests for neurosarcoidosis. Advances in serum testing and imaging have improved the diagnosis for key mimics of neurosarcoidosis in certain clinical scenarios, but biopsy remains an important method of differentiation. Key mimics of neurosarcoidosis in all cases include infections (tuberculosis, fungal), autoimmune disease (vasculitis, IgG4-related disease), and lymphoma. As neurosarcoidosis can affect any part of the nervous system, patients should have a unique differential diagnosis tailored to their clinical presentation. Although biopsy can assist with excluding mimics, diagnosis is ultimately clinical.
神经结节病是结节病的一种罕见表现,其诊断具有挑战性。肉芽肿的活检确认是不够的,因为其他肉芽肿性疾病也可能表现相似。本综述旨在指导临床医生在得出神经结节病的诊断之前,确定需要排除的关键疾病。
尽管正在研究新的生物标志物,但目前还没有针对神经结节病的可靠检测方法。血清检测和影像学的进展提高了某些临床情况下神经结节病关键类似疾病的诊断水平,但活检仍然是一种重要的鉴别方法。在所有情况下,神经结节病的关键类似疾病包括感染(结核、真菌)、自身免疫性疾病(血管炎、IgG4 相关疾病)和淋巴瘤。由于神经结节病可影响神经系统的任何部位,因此患者应根据其临床表现进行独特的鉴别诊断。虽然活检有助于排除类似疾病,但诊断最终还是要靠临床。