This medical condition has either been superseded or has become inactive
| Specific Conditions |
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| Indication for Ig Use |
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| Level of Evidence | Insufficient data (Category 4a) |
| Description and Diagnostic Criteria | The diagnosis of primary Sjögren’s syndrome should be consistent with the criteria of the American-European Study group (Tzioufas et al, 2007). Intravenous immunoglobulin (IVIg) may be indicated in patients with some neuropathy subtypes associated with Sjögren’s syndrome (excluding those caused by necrotising vasculitis) where other treatments have been ineffective. |
| Justification for Evidence Category |
Sjögren’s syndrome associated neuropathy comprises a heterogeneous group of neuropathies. There is a very low level of evidence for IVIg use in this condition, with conflicting reports of efficacy. Improvement has been reported in small case series of patients with some forms of sensory neuropathy (Pereira 2016, Rist 2011, Yamashita 2013). Conflicting reports exist for patients with ataxic sensory neuronopathy, with one study reporting benefit (Takahashi 2003) but most studies (Rist 2011, Pereira 2016) and clinical experience suggesting it is ineffective for this patient group. As there may be a subset of patients who benefit from Ig therapy for this condition the Specialist Working Groups (Immunology and Neurology) of the Immunoglobulin Governance Program have recommended to retain this condition within the Criteria, but only where demonstrable benefit is shown. Where no demonstrable benefit can be identified, patients will not be eligible to remain on Ig therapy under these criteria. |
| Diagnosis Requirements |
A diagnosis must be made by a Neurologist. |
| Qualifying Criteria for Ig Therapy |
Severe, primary Sjögren’s syndrome associated neuropathy that is unresponsive to corticosteroid and immunosuppressant therapy
Use this indication for new patients and those that have never trialled off from Ig therapy. Please use the indication Relapse of Sjögren’s syndrome associated neuropathy within six months of trial off Ig therapy for responding patients who have relapsed after weaning from Ig therapy.
AND
AND
Review by a neurologist is required within four months of treatment to determine whether the patient has responded. Continuing six monthly reviews may be undertaken by neurologists, rheumatologists or immunologists. After a year of therapy a trial of Ig weaning should be attempted with a view to cessation within six months unless there is a contraindication to doing so. Documentation of clinical effectiveness is necessary for continuation of IVIg therapy. Relapse of Sjögren’s syndrome associated neuropathy within six months of trial off Ig therapy
This indication should be used for responding Sjögren’s syndrome patients who have relapsed within six months of commencement of a trial off immunoglobulin therapy. For those who have not received Ig therapy previously please use the indication Severe, primary Sjögren’s syndrome associated neuropathy that is unresponsive to corticosteroid and immunosuppressant therapy
AND
Review by a neurologist is required within four months of treatment to determine whether the patient has responded. Thereafter the continuing six monthly reviews may be undertaken by a neurologist, rheumatologist or immunologist. After a year of therapy a trial of Ig weaning should be attempted with a view to cessation within six months unless there is a contraindication to doing so. Documentation of clinical efficacy is necessary for continuation of IVIg therapy. Once a patient has relapsed when trialled off Ig treatment, a second line immunomodulatory agent should be strongly considered as additional therapy. |
| Exclusion Criteria |
Sjögren’s syndrome associated vasculitic neuropathy |
| Review Criteria for Assessing the Effectiveness of Ig Use |
Severe, primary Sjögren’s syndrome associated neuropathy that is unresponsive to corticosteroid and immunosuppressant therapy
Review by a neurologist is required within four months (induction plus three maintenance cycles) of treatment to determine whether the patient has responded. If there is no benefit after this treatment, IVIg therapy should be abandoned. In responding patients, continuing six monthly reviews may be undertaken by neurologists, rheumatologists or immunologists.
After a year of therapy a trial of Ig weaning should be attempted with a view to cessation within six months unless there is a contraindication to doing so. Documentation of clinical effectiveness is necessary for continuation of IVIg therapy. Clinical effectiveness of Ig therapy can be assessed by: On review of the initial authorisation period
On review of a continuing authorisation period
AND
Relapse of Sjögren’s syndrome associated neuropathy within six months of trial off Ig therapy
Review by a neurologist is required within four months (induction plus three maintenance cycles) of treatment to determine whether the patient has responded. If there is no benefit after this treatment, IVIg therapy should be abandoned. In responding patients, continuing six monthly reviews may be undertaken by neurologists, rheumatologists or immunologists.
After a year of therapy a trial of Ig weaning should be attempted with a view to cessation within six months unless there is a contraindication to doing so. Documentation of clinical effectiveness is necessary for continuation of IVIg therapy. Once a patient has relapsed when trialled off Ig treatment, a second line immunomodulatory agent should be strongly considered as additional therapy. Clinical effectiveness of Ig therapy may be assessed by: On review of the initial authorisation period
On review of a continuing authorisation period
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| Dose |
Severe, primary Sjögren’s syndrome associated neuropathy that is unresponsive to corticosteroid and immunosuppressant therapy
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. Relapse of Sjögren’s syndrome associated neuropathy within six months of trial off Ig therapy
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. |
| Bibliography |
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| Bonita, R & Beaglehole R 1988, ‘Recovery of motor function after stroke’ Stroke, vol. 19, no. 12, pp. 1497-500. https://www.ncbi.nlm.nih.gov/pubmed/3201508 Brito-Zeron, P, Akasbi, M, Bosch, X, et al 2013, ‘Classification and characterisation of peripheral neuropathies in 102 patients with primary Sjögren's syndrome’, Clinical and Experimental Rheumatology, vol. 31, no. 1, pp. 103-10. http://www.clinexprheumatol.org/abstract.asp?a=5938 Danieli, MG, Pettinari, L, Morariu, R, et al 2012, ‘Intravenous immunoglobulin and mycophenolate mofetil for long-standing sensory neuropathy in Sjögren’s syndrome’, Case reports in immunology, Volume 2012, Article ID 186320, 3 pages. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4207592/ Molina, JA, Benito-Leon, J, Bermejo, F, et al 1996, ‘Intravenous immunoglobulin therapy in sensory neuropathy associated with Sjögren’s syndrome’, Journal of neurology, neurosurgery, and psychiatry, vol. 60, no. 6, pp.699. http://jnnp.bmj.com/content/60/6/699.1.short Morozumi, S, Kawagahira Y, Iijima M, et al 2009, ‘Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren's syndrome’, Journal of the Neurological Sciences, vol. 279, pp. 57-61.https://www.ncbi.nlm.nih.gov/pubmed/19168191 Pereira, PR, Viala, K, Maisonobe, T,et al 2016, ‘Sjögren Sensory Neuropathy – Long-term outcome and treatment Response in a series of 13 cases’, Medicine (Baltimore), vol. 95, no. 19, pp. e3632. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902517/ Rankin J 1957, ‘Cerebral vascular accidents in patients over the age of 60’, Scottish medical journal, vol. 2, no. 4, pp. 127-36. Rist, S, Sellam, J, Hachulla, E, et al 2011, ‘Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren’s syndrome: A national, multicentric retrospective study’, Arthritis care & research, vol. 63, no. 9, pp. 1339-44. https://www.ncbi.nlm.nih.gov/pubmed/21584943 Stroke Society of Australia, ‘The Modified Rankin Scale’, Last Updated 2010. http://www.strokesociety.com.au/index.php?option=com_content&view=article&id=292:modified-rankin-scale-astn&catid=40:astn Takahashi, Y, Takata, T, Hoshino, M, et al 2003, ‘Benefit of IVIg for long-standing ataxic sensory neuronopathy with Sjögren’s syndrome’, Neurology, vol. 60, no. 3, pp. 503-5. https://www.ncbi.nlm.nih.gov/pubmed/12578938 Tzioufas, AG & Voulgarelis, M 2007, ‘Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias’, Best Practice & Research Clinical Rheumatology, vol. 21, issue. 6, pp. 989-1010. http://www.bprclinrheum.com/article/S1521-6942(07)00105-2/fulltext Wakasugi, D, Kato, T, Gono, T, et al 2009, ‘Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren's syndrome’, Modern rheumatology, vol. 19, no. 4, pp. 437-40. https://www.ncbi.nlm.nih.gov/pubmed/19458906 Smith, AJ, Jackson, MW, Wang, F, et al 2005, ‘Neutralisation of muscarinic receptor autoantibodies by intravenous immunoglobulin in Sjögren’s syndrome’, Human Immunology, vol. 66, no. 4, pp. 411–416. https://www.sciencedirect.com/science/article/abs/pii/S0198885905000339 Van Swieten, JC, Koudstaal, PJ, Visser, MC, et al 1988, ‘Interobserver agreement for the assessment of handicap in stroke patients, Stroke, vol. 19, no. 5, pp. 604-607. https://www.ncbi.nlm.nih.gov/pubmed/3363593 Yamashita, H, Eri, T, Ueda, Y, et al 2013, ‘Diagnosis and treatment of primary Sjögren syndrome-associated peripheral neuropathy: a six-case series’, Modern Rheumatology, vol. 23, no. 5, pp. 925-33. http://link.springer.com/article/10.1007/s10165-012-0767-x |