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各位委員您好, 感謝委員於7月16日參與第二次系統性紅斑狼瘡(systemic lupus erythematosus,SLE)共識小組會議,第二次會議中未能如期討論以及提及需要新增的statements,已製成線上投票問卷,再協請各委員們於8月22日(週二)前完成:
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Statement 22: Hydroxychloroquine is recommended for all patients with SLE unless contraindicated. The maintenance dose should not exceed 5 mg/kg per day of real bodyweight, but individualized based on the risk of flare and retinal toxicity. In the absence of risk factors for retinal toxicity, ophthalmological screening (visual fields examination and/or spectral domain-optical coherence tomography) should be performed at baseline, after 5 years, and yearly thereafter.
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Statement 27: Due to the potential adverse effects of therapies, immunosuppressive treatment should not be administered or altered based solely on serological activity.
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Statement 28: For severe neuropsychiatric manifestations of SLE with an inflammatory origin, the recommended first-line treatment involves a combination of moderate to high-dose glucocorticoids along with immunosuppressive agents like cyclophosphamide.
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Statement 30: For neuropsychiatric SLE that is thromboembolic with antiphospholipid antibodies, anti-coagulation is required. Vitamin K antagonist is preferred to direct-acting oral anticoagulants.
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Statement 31: Neuropsychiatric SLE requires prompt diagnosis and management. Neuroimaging, cerebrospinal fluid study, autoantibodies, and considerations of clinical syndromes, timing in the course of SLE, patient age and concomitant SLE disease activity, with rigorous exclusion of alternative causes, help in differentiation of SLE and non-SLE related neuropsychiatric manifestations. The therapeutic options include symptomatic, antithrombotic and immunosuppressive agents (including pulses of intravenous methylprednisolone), tailored by the predominant putative pathogenic pathway and the type, severity and activity of the neuropsychiatric manifestations.
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Statement 33: Therapeutic plasmapheresis or plasma exchange may be considered for thrombotic thrombocytopenic purpura, catastrophic antiphospholipid syndrome, diffuse alveolar hemorrhage, refractory or severe neuropsychiatric SLE, or life-threatening hematological manifestations.
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Statement 34: Acute treatment of severe lupus thrombocytopenia includes high-dose GC (including pulses of intravenous methylprednisolone) and/or intravenous immunoglobulin G. Immunosuppressive/GC-sparing agents such as azathioprine, cyclosporine, or mycophenolate can be used as maintenance therapy. Refractory cases can be treated with rituximab, cyclophosphamide, or thrombopoietin receptor agonists.
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Additional statement:
Pregnancy: Women with SLE can safely become pregnant with pregnancy planning and preconception assessment. Patients will need to be maintained on medications that are compatible with pregnancy and should continue these medications in pregnancy to prevent lupus flare.
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Additional statement:
Pulmonary hypertension (1): In SLE patients who present with Raynaud’s phenomenon, pleuritis, high disease activity, thrombocytopenia, acute/chronic cutaneous lupus, systemic hypertension, and ILD, as well as for those with anti-U1-RNP, anti-SSA, anti-SSB, and anti-cardiolipin antibodies, early screening for PAH should be considered.
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Additional statement:
Pulmonary hypertension (2): Clinical management of SLE-PAH should follow a risk-directed approach using a simplified four-strata risk-assessment tool aim to achieving and/or maintaining a low-risk status. PAH can be managed with pulmonary vasodilator therapy in combination with immunosuppressant for the treatment of SLE.
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