The cerebrospinal fluid <scp>CD</scp>4/<scp>CD</scp>8 ratio and interleukin‐6 and ‐10 levels in neurosarcoidosis: a multicenter, pragmatic, comparative study

Chazal, Thibaud; Costopoulos, Myrto; Maillart, Élisabeth; Fleury, C; Psimaras, Dimitri; Legendre, Paul; Chambrun, Marc Pineton de; Haroche, Julien; Lubetzki, Catherine; Amoura, Zahir; Legarff-Tavernier, M; Aubart, F. Cohen

doi:10.1111/ene.13975

Cited by 33 publications

(28 citation statements)

References 26 publications

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“…However, its sensitivity is low, ranging from 0 to 66.7% [156,164]. The CD4/CD8 ratio in CSF is higher in neurosarcoidosis than in multiple sclerosis and other neurological inflammatory disorders and may be helpful for the differential diagnosis [165]. Hypoglycorachia (up to 14%) and oligoclonal bands (up to 42%) are also biological features associated with neurosarcoidosis [156].…”

Section: Neurosarcoidosismentioning

confidence: 99%

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sève

Pachéco

Durupt

et al. 2021

Cells

224

144

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Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and renal dysfunction, affect less than 10% of patients. The diagnosis is not standardized but is based on three major criteria: a compatible clinical and/or radiological presentation, the histological evidence of non-necrotizing granulomatous inflammation in one or more tissues and the exclusion of alternative causes of granulomatous disease. Certain clinical features are considered to be highly specific of the disease (e.g., Löfgren’s syndrome, lupus pernio, Heerfordt’s syndrome) and do not require histological confirmation. New diagnostic guidelines were recently published. Specific clinical criteria have been developed for the diagnosis of cardiac, neurological and ocular sarcoidosis. This article focuses on the clinical presentation and the common differentials that need to be considered when appropriate.

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Section: Neurosarcoidosismentioning

confidence: 99%

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sève

Pachéco

Durupt

et al. 2021

Cells

224

144

View full text Add to dashboard Cite

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“…Moreover, an IL-6 level >50 pg/mL predicted progression and shorter relapse-free survival than those with an IL-6 level <50 pg/mL. 66 As part of CSF analysis, IL-6 and CD4/CD8 may increase diagnostic sensitivity and specificity for NS.…”

Section: Diagnostic Approach Neurosarcoidosis Manifestationsmentioning

confidence: 96%

The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment

Voortman

Stern

Saketkoo

et al. 2020

Semin Respir Crit Care Med

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Neurosarcoidosis (NS) is an often severe, destructive manifestation with a likely under-reported prevalence of 5 to 15% of sarcoidosis cases, and in its active phase demands timely treatment intervention. Clinical signs and symptoms of NS are variable and wide-ranging, depending on anatomical involvement. Cranial nerve dysfunction, cerebrospinal parenchymal disease, aseptic meningitis, and leptomeningeal disease are the most commonly recognized manifestations. However, non-organ-specific potentially neurologically driven symptoms, such as fatigue, cognitive dysfunction, and small fiber neuropathy, appear frequently.Heterogeneous clinical presentations and absence of any single conclusive test or biomarker render NS, and sarcoidosis itself, a challenging definitive diagnosis. Clinical suspicion of NS warrants a thorough systemic and neurologic evaluation hopefully resulting in supportive extraneural physical exam and/or tissue findings. Treatment targets the severity of the manifestation, with careful discernment of whether NS reflects active potentially reversible inflammatory granulomatous disease versus inactive postinflammatory damage whereby functional impairment is unlikely to be pharmacologically responsive. Non-organ-specific symptoms are poorly understood, challenging in deciphering reversibility and often identified too late to respond to conventional immunosuppressive/pharmacological treatment. Physical therapy, coping strategies, and stress reduction may benefit patients with all disease activity levels of NS.This publication provides an approach to screening, diagnosis, disease activity discernment, and pharmacological as well as nonpharmacological treatment interventions to reduce disability and protect health-related quality of life in NS.

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“…Another study reported increased IL-6 levels in the cerebrospinal fluid (CSF) of patients with neurosarcoidosis as compared to patients with multiple sclerosis or other inflammatory disorders. CSF concentration of IL-6 > 50 pg/mL was associated with a higher risk of relapse or progression of neurosarcoidosis (25). Genetic variations in the genes encoding IL-6 were preferentially upregulated in patients with severe and progressive sarcoidosis thus giving further evidence of the pathogenic role of this proinflammatory cytokine (26,27).…”

Section: Interleukin-6 Blockingmentioning

confidence: 98%

Emerging Molecular Targets for the Treatment of Refractory Sarcoidosis

et al. 2020

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Sarcoidosis is a multisystem granulomatous disease of unknown origin that has variable clinical course and can affect nearly any organ. It has a chronic course in about 25% of patients. Corticosteroids (CS) are the cornerstone of therapy but their long-term use is associated with cumulative toxicity. Commonly used CS-sparing agents include methotrexate, cyclophosphamide, azathioprine, and mycophenolate mofetil. Twenty to forty percentage of sarcoidosis patients are refractory to these therapies or develop severe adverse events. Therefore, additional and targeted CS-sparing agents are needed for chronic sarcoidosis. Macrophage activation, interferon response, and formation of the granuloma are mainly mediated by T helper-1 responses. Different pro-inflammatory cytokines such as interleukin (IL)-8, IL-12, IL-6, and tumor necrosis factor-alpha (TNF-α) have been shown to be highly expressed in sarcoidosis-affected tissues. As a result of increased production of these cytokines, Janus kinase-signal transducer and activator of transcription (JAK-STAT) signaling is constitutively active in sarcoidosis. Several studies of biological agents that target TNF-α have reported their efficacy and appear today as a second line option in refractory sarcoidosis. Some case series report a positive effect of tocilizumab an anti-IL-6 monoclonal antibody in this setting. More recently, JAK inhibition appears as a new promising strategy. This review highlights key advances on the management of chronic refractory sarcoidosis. Novel therapeutic strategies and treatment agents to manage the disease are described.

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The cerebrospinal fluid CD4/CD8 ratio and interleukin‐6 and ‐10 levels in neurosarcoidosis: a multicenter, pragmatic, comparative study

Cited by 33 publications

References 26 publications

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

The Burden of Neurosarcoidosis: Essential Approaches to Early Diagnosis and Treatment

Emerging Molecular Targets for the Treatment of Refractory Sarcoidosis

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