Ramos-Casals Manuel, Robles Angel, Brito-Zerón Pilar, Nardi Norma, Nicolás José M, Forns Xavier, Plaza Joan, Yagüe Jordi, Sánchez-Tapias Jose M, Font Josep
Department of Autoimmune Diseases, School of Medicine, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain.
Semin Arthritis Rheum. 2006 Dec;36(3):189-96. doi: 10.1016/j.semarthrit.2006.08.005. Epub 2006 Sep 22.
To analyze the etiology, clinical presentation, and outcomes of patients with life-threatening cryoglobulinemic vasculitis.
We studied 209 consecutive patients with cryoglobulinemic vasculitis. A potentially life-threatening cryoglobulinemia was considered as the development of renal failure, vasculitic abdominal involvement, pulmonary hemorrhage, or central nervous system involvement.
Twenty-nine (14%) patients had life-threatening cryoglobulinemic vasculitis. There were 17 women and 12 men, with a mean age of 57 years. In 17 (59%) patients, life-threatening cryoglobulinemia was the initial clinical feature of the disease. The 29 patients had a total of 33 life-threatening episodes, which included renal failure due to cryoglobulinemic glomerulonephritis (n = 18), intestinal vasculitis (n = 8), pulmonary hemorrhage (n = 4), and central nervous system involvement (n = 3). In comparison with a control group of age-sex-matched patients with milder cryoglobulinemic vasculitis, those with severe cryoglobulinemic vasculitis had a higher frequency of fever (28% versus 7%, P = 0.017), type II cryoglobulins (100% versus 59%, P = 0.008), low C3 levels (55% versus 20%, P = 0.001), and a higher mean value of cryocrit (11.4% versus 3.3%, P = 0.004). Nineteen (66%) of the 29 patients with life-threatening involvement died, with the mortality rate reaching 100% in patients with intestinal ischemia and pulmonary hemorrhage.
Life-threatening cryoglobulinemic vasculitis was observed in 14% of our patients, with almost two-thirds of episodes occurring at the onset of the disease. Fever, high cryocrit levels, and low C3 levels were associated with this severe presentation. Two-thirds of the patients died, with mortality for pulmonary hemorrhage and intestinal ischemia reaching 100%.
分析危及生命的冷球蛋白血症性血管炎患者的病因、临床表现及预后。
我们研究了209例连续性冷球蛋白血症性血管炎患者。潜在危及生命的冷球蛋白血症被定义为出现肾衰竭、血管炎性腹部受累、肺出血或中枢神经系统受累。
29例（14%）患者患有危及生命的冷球蛋白血症性血管炎。其中女性17例，男性12例，平均年龄57岁。17例（59%）患者中，危及生命的冷球蛋白血症是该疾病的初始临床特征。这29例患者共发生33次危及生命的发作，包括冷球蛋白血症性肾小球肾炎导致的肾衰竭（n = 18）、肠道血管炎（n = 8）、肺出血（n = 4）和中枢神经系统受累（n = 3）。与年龄和性别匹配的轻度冷球蛋白血症性血管炎对照组相比，严重冷球蛋白血症性血管炎患者发热频率更高（28% 对7%，P = 0.017）、Ⅱ型冷球蛋白比例更高（100% 对59%，P = 0.008）、C3水平更低（55% 对20%，P = 0.001），冷沉淀比容平均值更高（11.4% 对3.3%，P = 正确答案：0.004）。29例危及生命受累患者中有19例（66%）死亡，肠道缺血和肺出血患者的死亡率达100%。
我们的患者中有14%发生危及生命的冷球蛋白血症性血管炎，近三分之二的发作发生在疾病初发时。发热、高冷沉淀比容水平和低C3水平与这种严重表现相关。三分之二的患者死亡，肺出血和肠道缺血的死亡率达100%。
