Neurosarcoidosis: an update on diagnosis and therapy

References

• Ungprasert P, Carmona EM, Utz JP, et al., Epidemiology of sarcoidosis 1946-2013: a population-based study. Mayo Clin Proc. 2016;l91(2): 183–188.
   Google Scholar
• Judson MA, Boan AF, Lackland DT. The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29(2):119–127.
   PubMed Web of Science ®Google Scholar
• Baughman RP, Teirstein AS, Judson MA, et al. Case control etiologic study of sarcoidosis (ACCESS) research group. clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001;164(10):1885–1889.
   PubMed Web of Science ®Google Scholar
• Fritz D, van de Beek D, Brouwer MC. Clinical features, treatment and outcome in neurosarcoidosis: systematic review and meta-analysis. BMC Neurol. 2016;16(1):220.
   PubMedGoogle Scholar
• Morimoto T, Azuma A, Abe S, et al. Epidemiology of sarcoidosis in Japan. Eur Respir J. 2008;31:372–9.
   PubMed Web of Science ®Google Scholar
• Iwai K, Tachibana T, Takemura T, et al. Pathological studies on sarcoidosis autopsy. Acta Pathol Jpn. 1993;43(7–8):372–376.
   PubMedGoogle Scholar
• Ricker W, Clark M. Sarcoidosis; a clinicopathological review of 300 cases, including 22 autopsies. Am J Clin Pathol. 1949;19(8):725–749.
   PubMed Web of Science ®Google Scholar
• Culver DA, Ribeiro Neto ML, Moss BP, et al. Neurosarcoidosis. Semin Respir Care Med. 2017;38:1–15.
   PubMed Web of Science ®Google Scholar
• Allen RKA, Sellars RE, Sandstrom PA. A prospective study of 32 patients with neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2003;20(2):118–125.
   PubMed Web of Science ®Google Scholar
• Ferriby D, de Seze J, Stojkovic T, et al., Long-term follow-up of neurosarcoidosis. Neurology. 2001;57(5): 927–979.
   PubMed Web of Science ®Google Scholar
• Joseph FG, Scolding NJ. Neurosarcoidosis: a study of 30 new cases. J Neurol Neurosurg Psychiatry. 2009;80(3):297–304.
   PubMed Web of Science ®Google Scholar
• Stern BJ, Krumholz A, Johns C, et al. Sarcoidosis and its neurological manifestations. Arch Neurol. 1985;42(9):909–917.
   PubMedGoogle Scholar
• Voortman M, Hendriks CMR, Elfferich MDP, et al. The Burden of sarcoidosis symptoms from a patient perspective. Lung. 2019;197(2):155–161.
   PubMed Web of Science ®Google Scholar
• Carlson ML, White JR, Espahbodi M, et al. Cranial base manifestation of neurosarcoidosis: a review of 305 patients. Otol Neurontol. 2014; 36(1) 156–166.
   Web of Science ®Google Scholar
• Pawate S, Moses H, Sriram S. Presentations and outcomes of neurosarcoidosis: a study of 54 cases. Q J Med. 2009;102(7):449–460.
   Google Scholar
• Dua A, Manadan A. Heerfordt’s syndrome, or uveoparotid fever. N Engl J Med. 2013;369(5):458.
   PubMed Web of Science ®Google Scholar
• Ungprasert P, Crowson CS, Matteson EL. Clinical characteristics of parotid gland sarcoidosis: a population-based study. JAMA Otolaryngol Head Neck Surg. 2016;142(5):503–504.
   PubMed Web of Science ®Google Scholar
• Lower EE, Broderick JP, Brott TG, et al. Diagnosis and management of neurological sarcoidosis. Arch Int Med. 1997;157(16):1864–1868.
   PubMedGoogle Scholar
• Ungprasert P, Matteson EL. Neurosarcoidosis. Rheumatol Dis Clin N Am. 2017;46(4):593–597.
   Google Scholar
• Christoforidis GA, Spickler EM, Recio MV, et al. MR of CNS sarcoidosis: correlation of imaging features to clinical symptoms and response to treatment. AJNR Am J Neuroradiol. 1999;20(4):655–669.
   PubMed Web of Science ®Google Scholar
• Stern BJ, Royal W, Gelfand JM, et al., Definition and consensus diagnostic criteria for neurosarcoidosis: from the Neurosarcoidosis Consortium Consensus Group. JAMA Neurol. 2018;75(12): 1546–53.8.
   PubMed Web of Science ®Google Scholar
• Kane K. Deafness in sarcoidosis. J Laryngol Otol. 1976;90(6):531–537.
   PubMed Web of Science ®Google Scholar
• Oksanen V, Salmi T. Visual and auditory evoked potentials in the early diagnosis and follow-up of neurosarcoidosis. Acta Neurol Scand. 1986;74(1):38–42.
   PubMed Web of Science ®Google Scholar
• Stern BJ, Aksamit A, Clifford D, et al. Neurologic presentation of sarcoidosis. Neurol Clin. 2010;28(1):185–198.
   PubMed Web of Science ®Google Scholar
• Marangoni S, Argentiero V, Tavolato B. Neurosarcoidosis. Clinical description of 7 cases with proposal for a new diagnostic strategy. J Neurol. 2006;253(4):488–495.
   PubMed Web of Science ®Google Scholar
• Nowak DA, and Widenka DC . Neurosarcoidosis.: a review of its intracranial manifestation. J Neurol. 2001;248(5):363–372.
   PubMed Web of Science ®Google Scholar
• J B Jr, Strickland MC, William JP, et al. Computed tomography changes in neurosarcoidosis clearing with steroid treatment. J Comput Assist Tomogr. 1979;3(3):398–399.
   PubMed Web of Science ®Google Scholar
• Ho SU, Berenberg RA, Kim KS, et al. Sarcoid encephalopathy with diffuse inflammation and focal hydrocephalus shown by sequential CT. Neurology. 1979;29(8):1161–1165.
   PubMed Web of Science ®Google Scholar
• Herring AB, Urich H. Sarcoidosis of the central nervous system. J Neurol Sci. 1969;9(3):405–422.
   PubMed Web of Science ®Google Scholar
• Meyer JS, Foley JM, Campagna-Pinto D. Granulomatous angiitis of the meninges in sarcoidosis. Arch Neurol. 1953;69(5):587–600.
   Google Scholar
• Cohen-Aubert F, Galanaud D, Grabli D, et al. Spinal cord sarcoidosis. Clinical and laboratory profile and outcome of 31 patients in a case-control study. Medicine (Baltimore). 2010;89(2):133–140.
   PubMed Web of Science ®Google Scholar
• Bradley DA, Lower EE, Baughman RP. Diagnosis and management of spinal sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23(1):58–65.
   PubMed Web of Science ®Google Scholar
• Sohn M, Culver DA, Judson MA, et al. Spinal cord neurosarcoidosis. Am J Med Sci. 2014;347(3):195–198.
   PubMed Web of Science ®Google Scholar
• Durel CA, Marignier R, Maucort-Boulch D, et al. Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients. J Neurol. 2016;263(5):981–990.
   PubMed Web of Science ®Google Scholar
• Hoitsma E, Faber CG, Drent M, et al. Neurosarcoidosis: a clinical dilemma. Lancet Neurol. 2004;3(7):397–407.
   PubMed Web of Science ®Google Scholar
• Koffman B, Junck L, Elias SB, et al. Polyradiculopathy in sarcoidosis. Muscle Nerve. 1999;22(5):608–613.
   PubMed Web of Science ®Google Scholar
• Nemni R, Galassi G, Cohen M, et al. Symmetric sarcoid polyneuropathy: analysis of a sural nerve biopsy. Neurology. 1981;31(10):1217–1222.
   PubMed Web of Science ®Google Scholar
• Said G, Lacroix C, Plante-Bordeneuve V, et al. Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: a clinicopathological study of 11 patients. Brain. 2002;125(2):264–275.
   PubMedGoogle Scholar
• Bakkers M, Merkies IS, Lauria G, et al. Intraepidermal nerve fiber density and its application in sarcoidosis. Neurology. 2009;73(14):1141–1148.
   Web of Science ®Google Scholar
• Tavee JO, Karwa K, Ahmed Z, et al. Sarcoidosis-associated small fiber neuropathy in a large cohort: clinical aspects and response to IVIG and anti-TNF alpha treatment. Respir Med. 2017;126:135–138.
   PubMed Web of Science ®Google Scholar
• Costabel U, Hunninghake GW. ATS/ERS/WASOG statement on sarcoidosis. Sarcoidosis statement committee. American thoracic society. European respiratory society. World association for sarcoidosis and other granulomatous disorders. Eur Respir J. 1999;14(4):735–737.
   PubMedGoogle Scholar
• Judson MA, Costabel U, Drent M, et al. The WASOG sarcoidosis organ assessment instrument: an update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(1):19–27.
   PubMed Web of Science ®Google Scholar
• Schupp JC, Freitag-Wolf S, Bargagli E, et al. Phenotypes of organ involvement in sarcoidosis. Eur Respir J. 2018;25(1):1700991.
   Google Scholar
• Smith JK, Matheus MG, Castillo M. Imaging manifestations of neurosarcoidosis. AJR Am J Roentgenol. 2004;182(2):289–295.
   PubMed Web of Science ®Google Scholar
• Dumas JL, Valeyre D, Berlin C, et al. Central nervous system sarcoidosis: follow-up at MRI during steroid therapy. Radiology. 2000;214(2):411–420.
   PubMed Web of Science ®Google Scholar
• Shah R, Robertson GH, Cure JK. Correlation of MR imaging findings and clinical manifestations in neurosarcoidosis. AJNR Am J Neuroradiol. 2009;30(5):953–961.
   PubMed Web of Science ®Google Scholar
• Hashmi M, Kyristsis AP. Diagnosis and treatment of intramedullary spinal cord sarcoidosis. J Neurol. 1998;245(3):178–185.
   PubMed Web of Science ®Google Scholar
• Petereit HF, Reske D, Tumani H, et al., Soluble CSF interleukin 2 receptor as indicator of neurosarcoidosis. J Neurol. 2010;257(11): 1855–1863.
   PubMed Web of Science ®Google Scholar
• Tahmoush AJ, Albert J, Connor WW. CSF-ACE activity in probable CNS neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(3):191–197.
   PubMed Web of Science ®Google Scholar
• Dale JC, O’Brien JF. Determination of angiotensin-converting enzyme levels in cerebrospinal fluid is not useful test for the diagnosis of neurosarcoidosis. Mayo Clin Proc. 1999;74(5):535.
   PubMed Web of Science ®Google Scholar
• Burns TM, Dyck PJ, Aksamit AJ, et al. The natural history and long-term outcome of 57 limb sarcoidosis neuropathy cases. J Neurol Sci. 2006;244(1–2):77–87.
   PubMed Web of Science ®Google Scholar
• Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021;58(6):2004079.
   PubMed Web of Science ®Google Scholar
• Scott TF, Yandora K, Valeri A, et al. Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients. Arch Neurol. 2007;64(5):691–696.
   PubMedGoogle Scholar
• Culver DA, Dahan A, Bajorunas D, et al. Cibinetide improves corneal nerve fiber abundance in patients with sarcoidosis-associated small nerve fiber loss and neuropathic pain. Invest Opthalmol Vis Sci. 2017;58(6):BIO52–BIO60.
   PubMed Web of Science ®Google Scholar
• Adamec I, Ozretic D, Zadro I, et al. Progressive meningoencephalitis due to neurosarcoidosis. Clin Neurol Neurosurg. 2013;115(6):793–795.
   PubMed Web of Science ®Google Scholar
• Lally E, Murchison AP, Moster ML. Compressive optic neuropathy from neurosarcoidosis. Ophthalmic Plast Reconstr Surg. 2015;31:e79.
   PubMed Web of Science ®Google Scholar
• Tamme T, Liebur E, Kulla A. Sarcoidosis (Heerfordt syndrome): a case report. Stoxmatologija. 2007;9:61–64.
   PubMedGoogle Scholar
• Romao VC, Lima A, Bernardes M, et al. Three decades of low-dose methotrexate in rheumatoid arthritis: can we predict toxicity? Immunol Res. 2014;60(2–3):289–310.
   PubMed Web of Science ®Google Scholar
• Lucas CJ, Dimmitt SB, Martin JH. Optimising low-dose methotrexate for rheumatoid arthritis-A review. Br J Clin Pharmacol. 2019;85(10):2228–2234.
   PubMed Web of Science ®Google Scholar
• Androdias G, Maillet D, Mariginier R, et al. Mycophenolate mofetil may be effective in CNS sarcoidosis but not in sarcoid myopathy. Neurology. 2011;76(13):1168–1172.
   PubMed Web of Science ®Google Scholar
• Agbogu BN, Stern BJ, Sewell C, et al. Therapeutic considerations in patients with refractory neurosarcoidosis. Arch Neurol. 1995;52(9):875–879.
   PubMedGoogle Scholar
• Arun T, Palace J. Effect of immunotherapies and clinical outcomes in neurosarcoidosis: a retrospective cohort study. J Neurol. 2021;268(7):2466–2472.
   PubMed Web of Science ®Google Scholar
• Sharma OP. Effectiveness of chloroquine and hydroxychloroquine in treating selected patients with sarcoidosis with neurological involvement. Arch Neurol. 1998;55(9):1248–1254.
   PubMedGoogle Scholar
• Amber KT, Bloom R, Mrowietz U, et al. TNF-alpha: a treatment target or causes of sarcoidosis? J Eur Acad Dermatol Venereol. 2015;29(11):2104–2111.
   PubMed Web of Science ®Google Scholar
• Baughman RP, Drent M, Kavuru K, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006;174(7):795–802.
   PubMed Web of Science ®Google Scholar
• Rossman MD, Newman LS, Baughman RP, et al. A double-blinded, randomized, placebo-controlled trial of infliximab in subjects with active pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23(3):201–208.
   PubMed Web of Science ®Google Scholar
• Judson MA, Baughman RP, Costable U, et al., Efficacy of infliximab in extra-pulmonary sarcoidosis: results from a randomised trial. Eur Respir J. 2008;31(6): 1189–1196.
   PubMed Web of Science ®Google Scholar
• Toth C, Martin L, Morrish W, et al. Dramatic MRI improvement with refractory neurosarcoidosis treated with infliximab. Acta Neurol Scand. 2007;116(4):259–262.
   PubMed Web of Science ®Google Scholar
• Aubart FC, Bouvry D, Galanaud D, et al. Long-term outcomes of refractory neuro-sarcoidosis treated with infliximab. J Neurol. 2017;264(5):891–897.
   PubMed Web of Science ®Google Scholar
• Hilezian F, Maarouf A, Boutiere C, et al. TNF-alpha inhibitors used as steroid-sparing maintenance monotherapy in parenchymal CNS sarcoidosis. J Neurol Neurosurg Psychiatry. 2021;92(8):890–896.
   PubMed Web of Science ®Google Scholar
• Panselinas E, Rodgers JK, Judson MA. Clinical outcomes in sarcoidosis after cessation of infliximab treatment. Respirology. 2009;14(4):522–528.
   PubMed Web of Science ®Google Scholar
• Sandborn WJ, Hanauer SB. Infliximab in treatment of Crohn’s disease: a user’s guide for clinicians. Am J Gastroenterol. 2002;97(12):2962–2972.
   PubMed Web of Science ®Google Scholar
• Marnane M, Lynch T, Scott J, et al. Steroid-unresponsive neurosarcoidosis successfully treated with Adalimumab. J Neurol. 2009;256(1):139–140.
   PubMed Web of Science ®Google Scholar
• Bomprezzi R, Pati S, Chansakul C, et al. A case of neurosarcoidosis successfully treated with rituximab. Neurology. 2010;75(6):568–570.
   PubMed Web of Science ®Google Scholar
• Sharp M, Donnelly SC, Moller DR. Tocilizumab in sarcoidosis patients failing steroid sparing therapies and anti-TNF agents. Respir Med. 2019;1:100004.
   Google Scholar
• Friedman MA, Le B, Stevens J, et al. Tofacitinib as a steroid-sparing therapy in pulmonary sarcoidosis, an open-label prospective proof-of-concept study. Lung. 2021;199(2):147–153.
   PubMed Web of Science ®Google Scholar
• Doty JD, Mazur JE, Judson MA. Treatment of corticosteroid-resistant neurosarcoidosis with short course cyclophosphamide regimen. Chest. 2003;124(5):2023–2026.
   PubMed Web of Science ®Google Scholar
• Baughman RP, Barney JB, O’Hare L, et al. A retrospective pilot study examining the use of Acthar gel in sarcoidosis patients. Respir Med. 2016;110:66–72.
   PubMed Web of Science ®Google Scholar