Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome

Wakasugi, Daisuke; Kato, Takashi; Gono, Takahisa; Ito, Eiichi; Nodera, Hiroyuki; Kawaguchi, Yasushi; Yamanaka, Hisashi; Hara, Masako

doi:10.1007/s10165-009-0180-2

Cited by 39 publications

(39 citation statements)

References 14 publications

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“…In general, the response to corticosteroids is poor 35,40,60,61 ; further studies are needed to assess the efficacy of immunosuppressant agents, particularly the response to Rituximab, which is a monoclonal antibody directed against CD20 antigen on B-cells. In 5 Sjögren patients treated with IvIG for a small-fiber neuropathy involving the torso as well as the proximal extremities, Morozumi et al reported improvement in pain as assessed by a visual analogue scale.…”

Section: Immunomodulatory or Immunosuppressive Therapy For Smallfibermentioning

confidence: 98%

Peripheral Nervous System Manifestations of Sjögren Syndrome

Birnbaum

2010

The Neurologist

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Sjögren syndrome is among the most common autoimmune diseases affecting adults in the United States, and is frequently regarded as an immune-mediated exocrinopathy exclusively causing dry eyes and dry mouth. However, as a systemic rheumatic disease, there can be various "extraglandular" complications. The eclectic permutation of peripheral nervous system (PNS) syndromes which occur in Sjögren patients are among the most common and severe extraglandular complications. This review article highlights the evaluation, differential diagnosis, immunopathogenic mechanisms, and potential treatment options of these PNS complications encountered by neurologists. The sensory neuropathies constitute the most frequent PNS complication. Sjögren patients can suffer from severe neuropathic pain, with small-fiber neuropathy causing lancinating or burning pain which can disproportionately affect the proximal torso or extremities, and the face (ie, in a "non-length-dependent distribution"). The technique of skin biopsy, assessing for the intraepidermal nerve fiber density of unmyelinated nerves, provides a useful technique for neurologists to diagnose small-fiber neuropathies, especially when there is such a non-length-dependent distribution. Other diagnostic techniques (ie, electromyography/nerve-conduction studies, evoked potentials, nerve and muscle biopsy) may be useful in specific subtypes of neuropathies. A rational approach to treatment requires a careful appraisal of the clinical subtype of the neuropathy, as well as a familiarity with such discriminating immunopathogenic mechanisms. The application of the traditional armamentarium used for neuropathic pain can be especially challenging. Sjögren patients can suffer from debilitating fatigue, sicca symptoms, and autonomic findings; as such manifestations can be complications of various neuropathic agents, neurologists should understand how to minimize such iatrogenic complications. Therefore, this article will empower neurologists to more effectively collaborate with rheumatologists, in the diagnosis and treatment of Sjögren patients with PNS complications.

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Section: Immunomodulatory or Immunosuppressive Therapy For Smallfibermentioning

confidence: 98%

Peripheral Nervous System Manifestations of Sjögren Syndrome

Birnbaum

2010

The Neurologist

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“…Several case reports and case series describe the efficacy of intravenous (IV) IgG in patients with peripheral neuropathy [31, 32•, 33– 36]. Both ataxic and painful forms of Sjögren’s-related peripheral neuropathy have benefited.…”

Section: Recent Developments In Sjögren’s Syndrome–associated Systemimentioning

confidence: 99%

Vasculitis in Sjögren’s Syndrome

Scofield

2011

Curr Rheumatol Rep

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Sjögren’s syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren’s syndrome patients also have disease in other organs. One of the most common extraglandular manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological examination, is common. Although half of those individuals with subcutaneous vasculitis have only a single episode, skin vasculitic involvement is associated with more severe disease. Necrotizing vasculitis of medium-sized vessels resembling polyarteritis nodosa can occur in Sjögren’s syndrome patients. Experience in therapy for vasculitis is limited, but intravenous IgG may be effective. Recent data support a relationship between neuromyelitis optica (Devic disease) and Sjögren’s syndrome. Sjögren’s syndrome patients with optic neuritis or transverse myelitis have anti–aquaporin-4, which are characteristic of Devic disease. Devic disease patients have salivary lymphocytic infiltration similar to that found among Sjögren’s syndrome patients.

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“…Other therapeutic options that have shown benefit are azathioprine, methotrexate, cyclosporine, tumor necrosis factor antagonists, IVIG, and plasmapheresis. 2,4,9,21,22 Finally, rituximab shows promise as a recent therapeutic breakthrough, but its overall benefit is yet to be substantiated. 23 …”

Section: Discussionmentioning

confidence: 97%