Vantelon J M, Godeau B, André C, Bierling P
Laboratoire d'Immunologie Plaquettaire, Hôpital Henri Mondor AP-HP, Créteil, France.
Thromb Haemost. 2000 Jan;83(1):42-5.
In an attempt to evaluate the frequency of autoimmune markers in autoimmune thrombocytopenic purpura (AITP) and to determine if autoimmune markers in patients with isolated AITP were associated with particular disease manifestations, we analyzed records of 122 consecutive adults with AITP. Twenty-nine patients (24%) had significant titers of one or several autoimmune markers at AITP onset. Among them, 16 (13%) had antinuclear antibodies. The presence of autoimmune markers did not correlate with presenting feature, response to treatment or long-term outcome of AITP. Six patients (5%) developed seven autoimmune diseases during follow-up, comprising systemic lupus erythematosus, an antiphospholipid syndrome, autoimmune haemolytic anemia (n = 2), Grave's disease, Hashimoto's disease and primary biliary cirrhosis. At AITP onset, three of these patients had isolated biological markers of the autoimmune disease they later developed. The annual average incidence rate of autoimmune diseases was 1% per patient-year in the entire group and 0.4% in the group of patients with no autoimmune markers at AITP onset. This low rate is probably due to careful assessment at diagnosis for concomitant overt autoimmune disease. We recommend extensive screening for autoimmune markers at AITP onset, and careful follow-up of patients with autoimmune markers. Routine screening for autoimmune markers during AITP follow-up is not necessary for patients with no autoimmune markers at AITP onset. Systemic lupus erythematosus (SLE) and other autoimmune disorders can complicate autoimmune thrombocytopenic purpura (AITP) or be diagnosed concomitantly with otherwise unremarkable AITP (1, 2). However, the frequency and prognostic value of isolated autoimmune markers (i.e. not associated with an autoimmune disorder), particularly antinuclear antibodies (ANA) at AITP onset or during follow-up is controversial (3-8). For example, the committee organized by George et al. (9) to write guideline on the diagnosis and treatment of AITP stated that the search for ANA and lupus anticoagulant were of "uncertain appropriateness at diagnosis and during follow-up". In an attempt to help practicians to make decisions, we analyzed the frequency of autoimmune markers and autoimmune disorders at onset and during the follow-up in 122 adults with AITP and no overt autoimmune disease at diagnosis. These consecutive patients were followed by the same physician for a mean period of 6 years, and had routine screening tests for autoimmune markers and disorders at onset, before steroid therapy, and regularly during follow-up.
为了评估自身免疫性血小板减少性紫癜（AITP）中自身免疫标志物的出现频率，并确定孤立性AITP患者的自身免疫标志物是否与特定疾病表现相关，我们分析了122例连续性成年AITP患者的记录。29例患者（24%）在AITP发病时有一种或几种自身免疫标志物的滴度显著升高。其中，16例（13%）有抗核抗体。自身免疫标志物的存在与AITP的就诊特征、治疗反应或长期预后无关。6例患者（5%）在随访期间发生了7种自身免疫性疾病，包括系统性红斑狼疮、抗磷脂综合征、自身免疫性溶血性贫血（2例）、格雷夫斯病、桥本甲状腺炎和原发性胆汁性肝硬化。在AITP发病时，这些患者中有3例具有他们后来所患自身免疫性疾病的孤立生物学标志物。整个组自身免疫性疾病的年平均发病率为每位患者每年1%，AITP发病时无自身免疫标志物的患者组为0.4%。这个低发病率可能是由于诊断时对伴发的明显自身免疫性疾病进行了仔细评估。我们建议在AITP发病时广泛筛查自身免疫标志物，并对有自身免疫标志物的患者进行仔细随访。对于AITP发病时无自身免疫标志物的患者，在AITP随访期间进行自身免疫标志物的常规筛查没有必要。系统性红斑狼疮（SLE）和其他自身免疫性疾病可使自身免疫性血小板减少性紫癜（AITP）复杂化，或在其他方面不明显的AITP患者中同时被诊断出来（1,2）。然而，孤立性自身免疫标志物（即与自身免疫性疾病无关）的出现频率和预后价值，特别是AITP发病时或随访期间的抗核抗体（ANA），存在争议（3 - 8）。例如，由乔治等人组织的委员会（9）撰写的关于AITP诊断和治疗的指南指出，寻找ANA和狼疮抗凝物在“诊断时和随访期间的适用性不确定”。为了帮助临床医生做出决策，我们分析了122例成年AITP患者发病时和随访期间自身免疫标志物和自身免疫性疾病的出现频率，这些患者在诊断时无明显自身免疫性疾病。这些连续性患者由同一位医生随访平均6年，并在发病时、类固醇治疗前以及随访期间定期进行自身免疫标志物和疾病的常规筛查。
