2009
DOI: 10.3109/s10165-009-0180-2
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Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome

Abstract: Neurological involvement occurs in approximately 20% of patients with primary Sjögren's syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy. Small fiber neuropathy (SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjögren's syndrome who developed SFN an… Show more

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Cited by 49 publications
(4 citation statements)
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“…Most of the selected articles for the review were used skin biopsy for the definitive diagnosis and/or genetic tests [54,57] (Table 1). The skin biopsy was used alone [32,48,62] or in the most cases together with other diagnostic procedure, as nerve conduction examinations [33,41,56] or scales to assess the severity of the neuropathic symptoms (Table 1). Quantitative sensory testing (QST) with vibratory (VDT), cold (CDT), and heat-pain (HP) detection threshold testing were added for the specific diagnosis [30,40,61].…”
Section: Diagnostic Examinationmentioning
confidence: 99%
“…Most of the selected articles for the review were used skin biopsy for the definitive diagnosis and/or genetic tests [54,57] (Table 1). The skin biopsy was used alone [32,48,62] or in the most cases together with other diagnostic procedure, as nerve conduction examinations [33,41,56] or scales to assess the severity of the neuropathic symptoms (Table 1). Quantitative sensory testing (QST) with vibratory (VDT), cold (CDT), and heat-pain (HP) detection threshold testing were added for the specific diagnosis [30,40,61].…”
Section: Diagnostic Examinationmentioning
confidence: 99%
“…A small uncontrolled trial of intravenous immunoglobulins in Sjögren’s syndrome found a reduction in neuropathic pain in SFN,250 but in other series the response to corticosteroids has been poor 150251252. A small prospective, phase III clinical trial testing the benefit of intravenous immunoglobulins in patients with painful large fiber sensory polyneuropathy will soon be enrolling and could potentially inform treatment choices in patients with SFN that is associated with Sjögren’s syndrome (Clinical trials identifier NCT03700138).…”
Section: Emerging Disease Specific Treatmentsmentioning
confidence: 99%
“…Many different treatments were experienced. Opioid analgesics [8,21,24] or nonopioid analgesic [22,26], corticosteroids [8,10,33,34,37,44], intravenous immunoglobulin (IVIG) alone [8,12,15,23,27,31,37,39,46,47] or in combination with other specific drugs, such as azathioprine [29], anti-epileptic drugs [4,11,13,16,18,28,32], immunotherapy [14,19,37], hormone therapy [7,43]. Less used are the following therapeutic strategies, in used for specific disorders, such as ARA290, an erythropoietin derivate for sarcoidosis SFN [45], recombinant human nerve growth factor for diabetic SFN [5], propranolol for SFN related to aquagenic pruritus [9], plasma exchange therapy for complex regional pain syndrome [6], enzyme replacement therapy for Fabry related SFN [17,35], botulinum toxin type A for keloid [38].…”
Section: Pharmacologic and Rehabilitation Therapymentioning
confidence: 99%
“…The benefits of intravenous immunoglobulin (IVIG) were reported for neuropathic pain in Sjogren [8,27]. In 8 studies, the efficacy and safety of IVIg are evaluated in patients with different features of SFN [12,15,23,27,31,37,39,46,47]. In contrast, IVIG had disappointing results, according to Pereira et al [33] and Yuki et al [44].…”
Section: The Pharmacological Approachesmentioning
confidence: 99%