Ohta Yui, Tsuchiya Takaaki, Oka Masatoshi, Tachibana Moriaki, Kondo Yoshitaka, Fukushima Kaoruko, Matsuno Shiho, Yamanaka Noriko, Suzuki Noriyuki, Komatsu Akiko, Rokutan Hirofumi, Yumura Wako, Arai Tomio, Ishigami Akihito, Itabashi Mitsuyo, Takei Takashi
Department of Nephrology and Dialysis, Tokyo Metropolitan Institute for Geriatrics and Gerontology, 35-2 Sakae-Cho, Itabashi, Tokyo, 173-0015, Japan.
Tokyo Metropolitan Institute for Geriatrics and Gerontology, 35-2 Sakae-Cho, Itabashi, Tokyo, 173-0015, Japan.
CEN Case Rep. 2025 Jan 6. doi: 10.1007/s13730-024-00963-2.
Type I and mixed cryoglobulinemic vasculitis differ in pathophysiology, clinical presentation, and therapeutic response. We report a case of refractory cryoglobulinemic vasculitis diagnosed following ischemic non-obstructive coronary artery disease (INOCA). The patient presented with dyspnea, as well as abdominal pain due to ischemic enteritis, purpura, and renal failure requiring dialysis. Despite the patient's IgG λ-type monoclonal gammopathy of undetermined significance (MGUS) and negative hepatitis C virus, the presence of rheumatoid factor (RF) activity and the possibility of IgM involvement were suggested by cryoglobulin analysis and strong glomerular IgM deposition. The condition was diagnosed as mixed cryoglobulinemia, and various immunomodulatory treatments, including methylprednisolone, rituximab and plasmapheresis, were administered without achieving cryoglobulin negativity. However, treatment with bortezomib and dexamethasone ultimately led to cryoglobulin negativity and clinical improvement although the patient was not weaned off dialysis, resulting in remission of the cryoglobulinemic vasculitis. This case suggests that bortezomib, a proteasome inhibitor, may be a promising treatment for refractory cryoglobulinemic vasculitis.
I型和混合型冷球蛋白血症性血管炎在病理生理学、临床表现和治疗反应方面存在差异。我们报告一例在诊断为缺血性非阻塞性冠状动脉疾病（INOCA）后确诊的难治性冷球蛋白血症性血管炎病例。该患者出现呼吸困难，以及因缺血性肠炎导致的腹痛、紫癜和需要透析的肾衰竭。尽管患者存在意义未明的IgG λ型单克隆丙种球蛋白病（MGUS）且丙型肝炎病毒检测阴性，但冷球蛋白分析提示存在类风湿因子（RF）活性以及IgM参与的可能性，并且肾小球有强烈的IgM沉积。该疾病被诊断为混合型冷球蛋白血症，给予了包括甲泼尼龙、利妥昔单抗和血浆置换在内的各种免疫调节治疗，但未实现冷球蛋白转阴。然而，使用硼替佐米和地塞米松治疗最终导致冷球蛋白转阴且临床症状改善，尽管患者未停止透析，冷球蛋白血症性血管炎得以缓解。该病例提示，蛋白酶体抑制剂硼替佐米可能是难治性冷球蛋白血症性血管炎的一种有前景的治疗方法。
