Senécal J L, Chartier S, Rothfield N
Division of Rheumatology, Hôpital Notre-Dame, Montréal, Québec, Canada.
J Rheumatol. 1995 May;22(5):868-75.
To define the clinical manifestations, autoantibody associations, optimal treatment, and prognosis of hypergammaglobulinemic purpura associated with systemic autoimmune rheumatic diseases.
Of 303 consecutive patients with systemic autoimmune rheumatic diseases evaluated over 5 years, 17 French Canadian patients with hypergammaglobulinemic purpura with systemic lupus erythematosus (SLE) (n = 12) or another systemic autoimmune rheumatic disease (n = 5) were identified and followed prospectively. Mild secondary Sjörgren's syndrome developed in 9 (53%) patients.
Sixteen (94.1%) patients were women. Attacks of hypergammaglobulinemic purpura occurred in the pretibial (76.5%) or perimalleolar (70.5%) areas or the dorsal aspect of the feet (52.9%). Triggering factors included walking, prolonged standing, and alcohol intake. The mean duration of attacks was 6.1 days. Systemic manifestations consistent with a flare of the underlying systemic autoimmune rheumatic diseases accompanied hypergammaglobulinemic purpura attacks in 15 (88%) patients. Arthralgias (n = 13, 86.6%), arthritis (n = 9, 69.2%), and periarthritis were characterstically localized adjacent to the purpura. Anti-Ro antibodies were expressed in all (100%) patients with hypergammaglobulinemic purpura with SLE, but in only 11 (28.9%) of 38 consecutive patients with SLE without hypergammaglobulinemic purpura (P < 0.000001, odds ratio 84, 95% confidence interval 4.6, 1525). The positive predictive values for hypergammaglobulinemic purpura in SLE were: anti-Ro plus anti-La 73%, anti-La 57%, and anti-Ro 52%. The negative predictive value of anti-Ro was 100%. Although 11 (92%) patients with SLE with anti-Ro expressed anti-52 kDa Ro [4 (36.3%) of whom also expressed anti-60 kDa Ro], this frequency was not greater than in anti-Ro positive patients with SLE without hypergammaglobulinemic purpura. The effects of indomethacin or hydroxychloroquine were assessed over 6 months in 8 patients with recurrent incapacitating hypergammaglobulinemic purpura. Complete (n = 4) or partial (n = 4) remission of hypergammaglobulinemic purpura occurred. In 5 additional patients with severe hypergammaglobulinemic purpura, attacks stopped with prednisone 25 to 60 mg daily. The mean duration of hypergammaglobulinemic purpura followup was 5.4 years (range 1-6 years). At last followup, hypergammaglobulinemic purpura had resolved in 11 (64.7%) patients despite persistently abnormal serology.
In the absence of anti-Ro antibodies, a presumptive diagnosis of hypergammaglobulinemic purpura secondary to SLE should be questioned. Prednisone should be used only in severe hypergammaglobulinemic purpura. Indomethacin and hydroxychloroquine are of value in the treatment of milder hypergammaglobulinemic purpura.
明确与系统性自身免疫性风湿性疾病相关的高球蛋白血症性紫癜的临床表现、自身抗体关联、最佳治疗方法及预后。
在5年期间评估的303例连续性系统性自身免疫性风湿性疾病患者中，确定了17例法裔加拿大患者，他们患有高球蛋白血症性紫癜合并系统性红斑狼疮（SLE）（n = 12）或其他系统性自身免疫性风湿性疾病（n = 5），并进行前瞻性随访。9例（53%）患者出现轻度继发性干燥综合征。
16例（94.1%）患者为女性。高球蛋白血症性紫癜发作部位为胫前（76.5%）、踝周（70.5%）或足背（52.9%）。诱发因素包括行走、长时间站立和饮酒。发作的平均持续时间为6.1天。15例（88%）患者高球蛋白血症性紫癜发作时伴有与潜在系统性自身免疫性风湿性疾病病情活动相符的全身表现。关节痛（n = 13，86.6%）、关节炎（n = 9，69.2%）和关节周围炎的特征是局限于紫癜附近。抗Ro抗体在所有（100%）合并SLE的高球蛋白血症性紫癜患者中均有表达，但在38例连续性无高球蛋白血症性紫癜的SLE患者中仅11例（28.9%）表达（P < 0.000001，比值比84，95%置信区间4.6，1525）。SLE中高球蛋白血症性紫癜的阳性预测值为：抗Ro加抗La 73%，抗La 57%，抗Ro 52%。抗Ro的阴性预测值为100%。尽管11例（92%）抗Ro阳性的SLE患者表达抗52 kDa Ro [其中4例（36.3%）也表达抗60 kDa Ro]，但该频率并不高于无高球蛋白血症性紫癜的抗Ro阳性SLE患者。对8例复发性致残性高球蛋白血症性紫癜患者在6个月内评估了吲哚美辛或羟氯喹的疗效。高球蛋白血症性紫癜出现完全缓解（n = 4）或部分缓解（n = 4）。另外5例严重高球蛋白血症性紫癜患者，每日服用25至60 mg泼尼松后发作停止。高球蛋白血症性紫癜的平均随访时间为5.4年（范围1 - 6年）。在最后一次随访时，尽管血清学检查仍异常，但11例（64.7%）患者的高球蛋白血症性紫癜已消退。
若无抗Ro抗体，应质疑继发于SLE的高球蛋白血症性紫癜的初步诊断。泼尼松仅应用于严重的高球蛋白血症性紫癜。吲哚美辛和羟氯喹在治疗较轻的高球蛋白血症性紫癜方面有价值。
