ITEM CRITERIA v2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING GROUP RATIONALE FOR ADDITIONS/CLARIFICATIONS Condition name revised to more appropriately Sjögren’s syndrome Sjogren’s syndrome associated neuropathy Condition reflect the specific diagnosis where evidence Name suggests some benefit of Ig therapy and is consistent with use in exceptional circumstances. Specialty Immunology Neurology The specialty is more appropriately Neurology given that the indication for Ig therapy will only be for Sjogren’s syndrome associated neuopathy and not other manifestations of Sjogren’s disease Category Exceptional circumstances only Exceptional circumstances only Unchanged Relevant specific conditions are defined to Specific Sjögren’s syndrome Small fibre neuropathy support data analysis Conditions Autonomic neuropathy Sensory neuropathy/neuronopathy Level of Insufficient data (Category 4a) Insufficient data (Category 4a) Unchanged Evidence Sjogren’s syndrome associated neuropathy Justification This section has been developed following comprises a heterogeneous group of neuropathies. for Evidence consultation of recent literature and by SWG There is a very low level of evidence for IVIg use in Category consensus. It is proposed to formally consult this condition, with conflicting reports of efficacy. with Australian and New Zealand Association Improvement has been reported in small case of Neurologists, ASCIA and the Australian series of patients with some forms of sensory Rheumatology Association regarding the neuropathy (Pereira 2016, Rist 2011, Yamashita proposed criteria during the Public 2013). Conflicting reports exist for patients with Consultation period. ataxic sensory neuronopathy, with one study reporting benefit (Takahashi 2003) but most
ITEM CRITERIA v2.1 PROPOSED REVISIONS TO THE CRITERIA SPECIALIST WORKING GROUP RATIONALE FOR ADDITIONS/CLARIFICATIONS Condition Name Sjögren’s syndrome Sjogren’s syndrome associated neuropathy Condition name revised to more appropriately reflect the specific diagnosis where evidence suggests some benefit of Ig therapy and is consistent with use in exceptional circumstances. Specialty Immunology Neurology The specialty is more appropriately Neurology given that the indication for Ig therapy will only be for Sjogren’s syndrome associated neuopathy and not other manifestations of Sjogren’s disease Category Exceptional circumstances only Exceptional circumstances only Unchanged Specific Conditions Sjögren’s syndrome Small fibre neuropathy Autonomic neuropathy Sensory neuropathy/neuronopathy Relevant specific conditions are defined to support data analysis Level of Evidence Insufficient data (Category 4a) Insufficient data (Category 4a) Unchanged Justification for Evidence Category Sjogren’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 This section has been developed following consultation of recent literature and by SWG consensus. It is proposed to formally consult with Australian and New Zealand Association of Neurologists, ASCIA and the Australian Rheumatology Association regarding the proposed criteria during the Public Consultation period.