Ding Yan, Li Chaoran, Huang Wensheng, Zhu Linzhong, Wang Lifang, Ma Doudou, Zhang Juan, Shi Lianjie
Department of Rheumatology and Immunology, Peking University Shougang Hospital, Beijing 100144, China.
Department of Gastrointestinal Surgey, Peking University Shougang Hospital, Beijing 100144, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2024 Dec 18;56(6):1101-1105. doi: 10.19723/j.issn.1671-167X.2024.06.025.
Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain. The patient had elevated serum creatinine levels, prompting further investigation through immunofixation electrophoresis and bone marrow aspiration biopsy. Immunofixation electrophoresis showed IgA-λ-type monoclonal immunoglobulin, while the bone marrow aspiration biopsy suggested plasmacytosis. Kidney biopsy indicated membranous hyperplastic glomerulonephritis, light and heavy chain deposition, IgA-λ. The patient was diagnosed with monoclonal gammopathy of renal significance. In light of the elevated serum creatinine, the patient was treated with chemotherapy regimen (bortezomib +cyclophosphamide +dexamethasone). After chemotherapy, there was no significant improvement in the patient's renal function. Subsequently, the patient experienced abdominal pain, skin purpura, joint pain and severe gastrointestinal bleeding. Gastroenteroscopy did not find the exact bleeding position. Angiography revealed hyperplasia of left jejunal artery. Surgical operation found that the bleeding site was located between the jejunum and ileum, where scattered hemorrhagic spots and multiple ulcers were present on the surface of the small intestine, with the deepest ulcers reaching the serosal layer. And the damaged intestine was removed during the operation. Intestinal pathology showed multiple intestinal submucosal arteritis, rusulting in intestinal wall necrosis and multiple ulcers. Considering intestinal lesions as gastrointestinal involvement of IgA vasculitis, methylprednisolone was used continually after the operation, and the patient's condition was improved. However, after half a year, the patient suffered a severe respiratory infection and experienced a recurrence of serious gastrointestinal bleeding. It was considered that the infection triggered the activity of IgA vasculitis, accompanied by gastrointestinal involvement. Finally, the patient died from gastrointestinal bleeding. The present case represented a patient with monoclonal gammopathy of renal significance and IgA vasculitis, prominently presenting with renal insufficiency and severe gastrointestinal bleeding, making the diagnosis and treatment process complex. Patients with IgA monoclonal gammopathy who presented with abdominal pain, purpura, and arthralgia should be vigilant for the possibility of concomitant IgA vasculitis. The treatment of cases with IgA vasculitis combined with monoclonal gammopathy of renal significance was rather challenging. Plasma cell targeting therapy might be an effective regimen for IgA vasculitis with monoclonal gammopathy. However, patients with poor renal response to the treatment indicated poor prognosis.
意义未明的单克隆丙种球蛋白病合并肾损害被称为具有肾意义的单克隆丙种球蛋白病。关于意义未明的单克隆丙种球蛋白病患者合并IgA血管炎的报道较少。在此，我们报告一例具有肾意义的单克隆丙种球蛋白病患者，其有IgA血管炎的表现，包括紫癜、胃肠道出血和关节疼痛。患者血清肌酐水平升高，促使通过免疫固定电泳和骨髓穿刺活检进行进一步检查。免疫固定电泳显示IgA-λ型单克隆免疫球蛋白，而骨髓穿刺活检提示浆细胞增多。肾活检显示膜性增生性肾小球肾炎、轻链和重链沉积、IgA-λ。该患者被诊断为具有肾意义的单克隆丙种球蛋白病。鉴于血清肌酐升高，患者接受化疗方案（硼替佐米+环磷酰胺+地塞米松）治疗。化疗后，患者肾功能无明显改善。随后，患者出现腹痛、皮肤紫癜、关节疼痛和严重的胃肠道出血。胃镜检查未发现确切出血部位。血管造影显示左空肠动脉增生。手术发现出血部位位于空肠和回肠之间，小肠表面有散在出血点和多个溃疡，最深的溃疡达浆膜层。术中切除受损肠段。肠道病理显示多处肠黏膜下动脉炎，导致肠壁坏死和多个溃疡。考虑肠道病变为IgA血管炎的胃肠道受累，术后继续使用甲泼尼龙，患者病情好转。然而，半年后，患者发生严重呼吸道感染，并再次出现严重的胃肠道出血。认为感染触发了IgA血管炎的活动，并伴有胃肠道受累。最终，患者死于胃肠道出血。本病例代表了一名具有肾意义的单克隆丙种球蛋白病和IgA血管炎患者，突出表现为肾功能不全和严重胃肠道出血，使得诊断和治疗过程复杂。出现腹痛、紫癜和关节痛的IgA单克隆丙种球蛋白病患者应警惕合并IgA血管炎的可能性。IgA血管炎合并具有肾意义的单克隆丙种球蛋白病病例的治疗颇具挑战性。浆细胞靶向治疗可能是治疗合并单克隆丙种球蛋白病的IgA血管炎的有效方案。然而，对治疗肾反应不佳的患者预后不良。
