Treatment of neurosarcoidosis

Bitoun, Samuel; Bouvry, Diane; Borie, R.; Mahévas, Mathieu; Sacré, Karim; Haroche, Julien; Psimaras, Dimitri; Pottier, Corinne; Mathian, Alexis; Hié, M.; Boutin, D.; Papo, Thomas; Godeau, Bertrand; Valeyre, Dominique; Nunès, Hilario; Amoura, Zahir; Aubart, F. Cohen

doi:10.1212/wnl.0000000000003431

Cited by 81 publications

(21 citation statements)

References 8 publications

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“… 22 Common steroid-sparing therapies in clinical practice include methotrexate, azathioprine, mycophenolate mofetil, leflunomide, hydroxychloroquine, and, increasingly, infliximab. 25 Retrospective analyses suggest that mycophenolate mofetil may be less effective than methotrexate (at least for preventing “relapse”), but such studies risk confounding by indication. 25 , 26 In retrospective analyses, infliximab, a TNFα inhibitor, is associated with favorable outcomes, including some cases refractory to other therapies.…”

Section: Discussionmentioning

confidence: 99%

“… 25 Retrospective analyses suggest that mycophenolate mofetil may be less effective than methotrexate (at least for preventing “relapse”), but such studies risk confounding by indication. 25 , 26 In retrospective analyses, infliximab, a TNFα inhibitor, is associated with favorable outcomes, including some cases refractory to other therapies. 8 …”

Section: Discussionmentioning

confidence: 99%

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Romeo

Lisak

Meltzer³

et al. 2018

Neurol Neuroimmunol Neuroinflamm

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A young man with numbness in arms and legs

Romeo

Lisak

Meltzer³

et al. 2018

Neurol Neuroimmunol Neuroinflamm

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“…48,49 In cardiac sarcoidosis, left ventricular ejection fraction improvement and suppression or lowering of 18-fluorodeoxyglucose uptake in positron emission tomography help to assess treatment efficacy. 50,51 In neurosarcoidosis trials, the ePOST or extrapulmonary physician organ severity tool is the most commonly used efficacy assessment tool 52,53 even if clinical improvement, resolution of gadolinium uptake on magnetic resonance imaging, and improvement in cerebrospinal fluid inflammation are also used and may represent easier ways to ensure correct evaluation of treatment efficacy. 54,55 In the case of renal sarcoidosis, improvement of serum creatinine levels, and the tapering of CS are the most commonly used criteria for assessing treatment efficacy.…”

Section: What Are Sarcoidosis First-line Treatments?mentioning

confidence: 99%

“…80,81 Data obtained in neurologic sarcoidosis with CNS involvement do not suggest any advantage of MMF over MTX. 52 Benefits of MMF therapy rely on rapid onset of action and limited toxicity in hematological and infectious complications. 6 The usual dose range varies from 2 to 3 g a day in maintenance therapy (Table 3).…”

Section: Mycophenolate Mofetilmentioning

confidence: 99%

<p>Refractory Sarcoidosis: A Review</p>

Jammal

Jamilloux

Gerfaud‐Valentin

et al. 2020

TCRM

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Sarcoidosis is a multi-system disease of unknown etiology characterized by granuloma formation in various organs (especially lung and mediastinohilar lymph nodes). In more than half of patients, the disease resolves spontaneously. When indicated, it usually responds to corticosteroids, the first-line treatment, but some patients may not respond or tolerate them. An absence of treatment response is rare and urges for verifying the absence of a diagnosis error, the good adherence of the treatment, the presence of active lesions susceptible to respond since fibrotic lesions are irreversible. That is when second-line treatments, immunosuppressants (methotrexate, leflunomide, azathioprine, mycophenolate mofetil, hydroxychloroquine), should be considered. Methotrexate is the only first-line immunosuppressant validated by a randomized controlled trial. Refractory sarcoidosis is not yet a well-defined condition, but it remains a real challenge for the physicians. Herein, we considered refractory sarcoidosis as a disease in which second-line treatments are not sufficient to achieve satisfying disease control or satisfying corticosteroids tapering. Tumor necrosis alpha inhibitors, third-line treatments, have been validated through randomized controlled trials. There are currently no guidelines or recommendations regarding refractory sarcoidosis. Moreover, criteria defining non-response to treatment need to be clearly specified. The delay to achieve response to organ involvement and drugs also should be defined. In the past ten years, the efficacy of several immunosuppressants beforehand used in other autoimmune or inflammatory diseases was reported in refractory cases series. Among them, anti-CD20 antibodies (rituximab), repository corticotrophin injection, and anti-JAK therapy anti-interleukin-6 receptor monoclonal antibody (tocilizumab) were the main reported. Unfortunately, no clinical trial is available to validate their use in the case of sarcoidosis. Currently, other immunosuppressants such as JAK inhibitors are on trial to assess their efficacy in sarcoidosis. In this review, we propose to summarize the state of the art regarding the use of immunosuppressants and their management in the case of refractory or multidrugresistant sarcoidosis.

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“…In a single center retrospective study (n=37), the treatment had no demonstrable effect on pulmonary function trends in patients failing or intolerant of methotrexate and prednisone 160. A French multicenter review of neurosarcoidosis outcomes in 40 patients suggested higher relapse rates with mycophenolate than with methotrexate, although the treatment strategies were not balanced between the two groups 161. Despite the paucity of robust evidence, no randomized trial has confidently demonstrated the inferiority of mycophenolate compared with other second line agents.…”

Section: Treatment Optionsmentioning

confidence: 99%

New advances in the management of pulmonary sarcoidosis

Culver

Judson

2019

BMJ

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Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.

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Treatment of neurosarcoidosis

Cited by 81 publications

References 8 publications

A young man with numbness in arms and legs

A young man with numbness in arms and legs

<p>Refractory Sarcoidosis: A Review</p>

New advances in the management of pulmonary sarcoidosis

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