Systemic therapy with conventional and novel immunomodulatory agents for ocular inflammatory disease

Ocular inflammatory disease is the third leading cause of blindness in the United States. In addition to the conventional immunomodulatory agents, which include antimetabolites, alkylating agents, and antibiotics such as cyclosporine, many of which have been used in the treatment of this disease for decades, several new treatment modalities have emerged within the past 10 years. We review in detail the characteristics, safety, and efficacy of the conventional immunomodulators, the more novel agents such as the biologics, and investigational drugs that appear promising in the treatment of ocular inflammatory disease.     

大疱性类天疱疮患者血清与疱液中巨噬细胞游走抑制因子的检测

目前认为大疱性类天疱疮(BP)的发病不仅与自身抗体的产生有关,而且与细胞因子网络的紊乱密切相关.巨噬细胞游走抑制因子(macrophage migration inhibitory factor,MIF)作为一种前炎症因子,在细胞因子网络平衡中发挥一定作用.本研究采用ELISA方法检测BP患者活动期血清、疱液中MIF的含量和缓解期血清中MIF的含量,以探讨MIF在BP发病机制中的作用及其临床意义.     

大疱性表皮坏死松解型药疹患者的护理

目的总结大疱性表皮坏死松解型药疹患者的护理要点。方法主要护理措施包括对患者进行保护性隔离,做好皮肤粘膜护理和导管护理;严密观察病情、治疗效果、并发症;保持呼吸道通畅,促进排痰,防止肺部感染;提供充足的热量,为组织修复提供足够的原料。结果对病人关心、体贴,耐心细致做好心理护理及家属的解释教育工作,使大疱性表皮坏死松解型药疹患者度过难关。结论加强医疗科的监测护理将更有助于重症患者的康复。     

131例神经系统疾病患者血清中抗BP180和抗BP230抗体的检测

目的：检测神经系统疾病患者血清中抗BP180、抗BP230和抗基底膜带抗体的阳性率。方法：收集神经系统疾病患者和正常对照血清,采用酶联免疫吸附试验（ELISA）和间接免疫荧光（IIF）检测两组血清中抗BPl80NC16A抗体和BP230抗体水平。结果：共收集到131例神经系统疾病患者血清(脑卒中109例,脑肿瘤17例,其他神经系统疾病19例)和131例正常对照血清。病例组中抗BP180NC16A1阳性率为1.45%,低于对照组的3.05%,差异具有统计学差异（P = 0.009）,病例组中抗BP230抗体阳性率5.34%与对照组（2.29%）比较,差异无统计学意义。IIF检测抗基底膜带抗体结果均为阴性。结论：抗BP180NC16A抗体在神经系统疾病患者中有较高的阳性率,可能与神经系统疾病患者合并大疱性类天疱疮相关。     

Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity

BACKGROUND: Mucous membrane pemphigoid (MMP), a chronic autoimmune subepithelial blistering disease, is associated with circulating IgG and/or IgA autoantibodies against several basement membrane zone antigens. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. OBJECTIVES: To analyse serum autoantibody profile and HLA class II alleles in MMP patients and to correlate this with the clinical presentation of disease. METHODS: Well-defined subgroups consisting of 124 patients with MMP were examined for IgG and IgA reactivity with immunoblotting using human epidermal, dermal and placental amnion proteins. The results were further analysed on the basis of detailed clinical (sites of involvement and disease severity) and immunopathological criteria (immunofluorescence study and HLA class II alleles). RESULTS: Immunoblot assay revealed that the majority of MMP patients had IgG (93 of 124, 75%) and/or IgA autoantibodies (63 of 124, 51%) to BP180 (including its soluble ectodomains, 120-kDa LAD-1 and 97-kDa LABD97 antigens). Other antigens targeted predominantly by IgG autoantibodies included: BP230 in 34 (27%), beta4 integrin in 26 (21%), and laminin 5 in three (2%). All the BP230+ sera and 23 (88%) beta4 integrin+ sera also reacted with at least one of the BP180 antigens. Over 85% of patients with reactivity to beta4 integrin had ocular involvement. In most cases of MMP, more severe clinical features were associated with antibody reactivity to multiple basement membrane zone antigens, as well as reactivity to multiple BP180 component antigens. Dual BP180/LAD-1 reactivity with IgG and IgA was associated with a more severe phenotype. In addition, the subset-dependent autoantibody reactivity correlated well with specific HLA class II alleles, DQB1*0301, DRB1*04 and DRB1*11. CONCLUSIONS: Our results confirmed that BP180 is a major autoantigen targeted by the sera of patients with MMP. The disease-prevalent HLA class II alleles and humoral autoimmune response against the particular subsets of antigenic epitope(s) within BP180 ectodomain may contribute to the clinicopathological significance and disease severity of MMP.     

Serum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgaris

Background BAFF [B‐cell activating factor belonging to the tumour necrosis factor (TNF) family] is a member of the TNF superfamily that regulates B‐lymphocyte proliferation and survival. It has been demonstrated that increased levels of soluble BAFF are associated with systemic autoimmunity in patients with systemic lupus erythematosus, rheumatoid arthritis and Sjögren's syndrome, and in animal models of spontaneous autoimmune diseases. However, the significance of circulating BAFF in autoimmune bullous diseases is unknown. Objectives To examine whether BAFF levels are elevated in the autoimmune blistering diseases pemphigus vulgaris (PV) and bullous pemphigoid (BP). Methods We examined sera obtained from 21 patients with PV, 39 patients with BP and 22 healthy donors. We performed enzyme‐linked immunosorbent assays for soluble BAFF and each disease‐specific antibody: antidesmoglein‐3 antibody for PV and anti‐BP180 antibody for BP. Results Significant elevations of serum BAFF levels were found in the patients with BP, but not with PV. There was apparently no significant association between the serum BAFF levels and titres of anti‐BP180 antibodies in the patients with BP. However, serum BAFF levels tended to be more elevated in patients with a shorter disease duration. There was a tendency that BAFF levels increased before the anti‐BP180 antibody levels increased at the onset of BP and quickly decreased in response to treatment. Conclusions BAFF may be a useful marker for early activation of an autoimmune diathesis and may play a critical role in triggering activation of self‐antigen‐driven autoreactive B cells in BP.