Sarcoidosis-associated small fiber neuropathy in a large cohort: Clinical aspects and response to IVIG and anti-TNF alpha treatment

Tavee, Jinny; Karwa, Karen; Ahmed, Zubair; Thompson, Nicolas R.; Parambil, Joseph G.; Culver, Daniel A.

doi:10.1016/j.rmed.2017.03.011

Cited by 134 publications

(102 citation statements)

References 8 publications

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“…In a retrospective review of 115 patients, the SFN treatment response rates were 76%, 67%, and 71% for treatment with intravenous immunoglobulins, anti-TNF-α, and combination therapy with both, respectively 75. By contrast, in the same trial patients treated with methotrexate or corticosteroids showed no improvement or even worsening of symptoms.…”

Section: Current Disease Specific Treatmentsmentioning

confidence: 98%

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Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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Sensory polyneuropathies, which are caused by dysfunction of peripheral sensory nerve fibers, are a heterogeneous group of disorders that range from the common diabetic neuropathy to the rare sensory neuronopathies. The presenting symptoms, acuity, time course, severity, and subsequent morbidity vary and depend on the type of fiber that is affected and the underlying cause. Damage to small thinly myelinated and unmyelinated nerve fibers results in neuropathic pain, whereas damage to large myelinated sensory afferents results in proprioceptive deficits and ataxia. The causes of these disorders are diverse and include metabolic, toxic, infectious, inflammatory, autoimmune, and genetic conditions. Idiopathic sensory polyneuropathies are common although they should be considered a diagnosis of exclusion. The diagnostic evaluation involves electrophysiologic testing including nerve conduction studies, histopathologic analysis of nerve tissue, serum studies, and sometimes autonomic testing and cerebrospinal fluid analysis. The treatment of these diseases depends on the underlying cause and may include immunotherapy, mitigation of risk factors, symptomatic treatment, and gene therapy, such as the recently developed RNA interference and antisense oligonucleotide therapies for transthyretin familial amyloid polyneuropathy. Many of these disorders have no directed treatment, in which case management remains symptomatic and supportive. More research is needed into the underlying pathophysiology of nerve damage in these polyneuropathies to guide advances in treatment.

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Section: Current Disease Specific Treatmentsmentioning

confidence: 98%

“…Unlike pulmonary sarcoidosis, which preferentially affects African-Americans, SFN seems to affects mainly white people 75. Most patients will have a non-length dependent pattern of numbness, pain, and paresthesia.…”

Section: Differential Diagnosis Of Small Fiber Neuropathiesmentioning

confidence: 99%

Diagnosis and management of sensory polyneuropathy

Gwathmey

Pearson

2019

BMJ

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“…Several case series reported the efficacy of intravenous immunoglobulins (IVIg) in SFN. 157,158 IVIg were also reported to be effective in other manifestations such as sarcoid myopathy 159 or neuropathy. 160…”

Section: Adjunctive Therapiesmentioning

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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“…It can occur in up to 40% of patients with sarcoidosis [16]. Patients can present with pain or dysautonomia, and can be mistaken for fibromyalgia.…”

Section: Organ Involvementmentioning

confidence: 99%

“…Infliximab results in improvement in skin lesions, pulmonary function and inflammatory markers, including PET scan and neurological improvement in neurosarcoidosis patients [36,37]. IVIG has also been used as therapy in refractory neurosarcoidosis [16].…”

Section: Organ Involvementmentioning

confidence: 99%

Sarcoidosis: The Organs Involved. A Primer for Primary Care Physicians

Rissmiller¹

2017

Int Arch Intern Med

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Sarcoidosis is a granulomatous disease of unknown etiology that can affect any organ. Management of sarcoidosis patients with the potential for multi-organ involvement can be complex and require the expertise of multiple specialties. The goal of this review is to discuss clinical, imaging and epidemiologic features of organ involvement in sarcoidosis, treatment strategies, and which patients are appropriate for referral to a clinic dedicated to the management of patients with sarcoidosis.

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Sarcoidosis-associated small fiber neuropathy in a large cohort: Clinical aspects and response to IVIG and anti-TNF alpha treatment

Cited by 134 publications

References 8 publications

Diagnosis and management of sensory polyneuropathy

Diagnosis and management of sensory polyneuropathy

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

Sarcoidosis: The Organs Involved. A Primer for Primary Care Physicians

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