Kaplan Allen P
Allergy Asthma Proc. 2018 May 1;39(3):184-190. doi: 10.2500/aap.2018.39.4121.
Chronic Spontaneous Urticaria (CSU) is an endogenous disorder that is strongly associated with autoimmunity, particularly with immunoglobulin G (IgG) antibody to the alpha subunit of the IgE receptor seen in 35-40% of patients. Basophils and cutaneous mast cells can be activated and lead to a late-phase-like perivascular infiltration about small venules and hive formation.
Review of current literature.
Antibody to thyroid antigens are seen in 25% of patients; a small fraction of these may be clinically hypothyroid (Hashimoto's Thyroiditis). Forty percent of patients have angioedema, but not laryngeal edema. Therapy typically begins with second-generation antihistamines (H1 receptor blockers) up to four times a day. The failure rate is substantial, and estimates vary from 25% to 50%. The drug of choice for antihistamine resistant cases is omalizumab, at 300 mg/month, which is effective in 70% of patients. H-2-antagonists and leucotriene antagonists are no longer recommended because the literature does not support additional efficacy beyond blockage of H-1 receptors. For patients unresponsive to antihistamines and omalizumab, cyclosporine is recommended next. This is similarly effective in 65-70% of patients; however, assessment of blood pressure and renal function need to be followed every 4-6 weeks. Corticosteroid should not be employed chronically; however, a brief course of 3-10 days can be used acutely for severe exacerbations. Other agents, such as dapsone, sulfasalazine, or hydroxychloroquin, can be tried when the aforementioned medications fail, but the results are unpredictable because they have not been shown to have efficacy beyond the placebo effect (25-30%), and have not been studied in patients for whom the aforementioned approach i.e. antihistamines, omalizumab, and cyclosporine has failed.
High dose antihistamines, omalizumab and cyclosporine (in that order) are effective and recommended for therapy of CUS, an inflammatory skin disorder associated with autoimmunity in 45% of patients.
慢性自发性荨麻疹（CSU）是一种内源性疾病，与自身免疫密切相关，尤其是与35% - 40%患者体内出现的针对IgE受体α亚基的免疫球蛋白G（IgG）抗体有关。嗜碱性粒细胞和皮肤肥大细胞可被激活，导致小静脉周围出现类似迟发性的血管周围浸润和荨麻疹形成。
回顾当前文献。
25%的患者体内可检测到甲状腺抗原抗体；其中一小部分患者可能临床上表现为甲状腺功能减退（桥本甲状腺炎）。40%的患者有血管性水肿，但无喉部水肿。治疗通常从第二代抗组胺药（H1受体阻滞剂）开始，每天服用多达4次。失败率很高，估计在25%至50%之间。抗组胺药耐药病例的首选药物是奥马珠单抗，每月300毫克，70%的患者使用该药有效。不再推荐使用H2拮抗剂和白三烯拮抗剂，因为文献表明除了阻断H1受体外，它们并无额外疗效。对于对抗组胺药和奥马珠单抗无反应的患者，接下来推荐使用环孢素。该药在65% - 70%的患者中同样有效；然而，需要每4 - 6周监测血压和肾功能。不应长期使用皮质类固醇；不过，对于严重发作，可短期（3 - 10天）急性使用。当上述药物无效时，可尝试使用其他药物，如氨苯砜、柳氮磺胺吡啶或羟氯喹，但结果不可预测，因为尚未证明它们除了安慰剂效应（25% - 30%）外还有疗效，且未在上述方法（即抗组胺药、奥马珠单抗和环孢素）治疗失败的患者中进行研究。
高剂量抗组胺药、奥马珠单抗和环孢素（按此顺序）对CSU有效且推荐用于治疗，CSU是一种45%患者与自身免疫相关的炎症性皮肤病。
