Milito Cinzia, Cinetto Francesco, Megna Valentina, Spadaro Giuseppe, Quinti Isabella, Liberatore Mauro
Department of Molecular Medicine, Sapienza University of Rome, 00185 Rome, Italy.
Department of Medicine-DIMED, University of Padova, 35122 Padova, Italy. Internal Medicine I, Ca' Foncello Hospital, Treviso, Italy.
J Clin Med. 2020 Mar 30;9(4):949. doi: 10.3390/jcm9040949.
Enteropathy may be the first presentation of immunodeficiency or it may occur during the course of the disease and in association with malabsorption in patients affected by primary antibody diseases. For these patients, immunoglobulin G (IgG) replacement therapy prevents infectious and non-infectious complications. Nonetheless some patients cannot achieve optimal IgG trough levels, even when treated with high Ig doses in absence of protein-losing syndromes. We investigated seven patients affected by common variable immunodeficiencies (CVIDs) and treated with high Ig doses (600-800 mg/kg/month) showing low IgG trough level. Patients underwent abdominal scintigraphy with human polyclonal immunoglobulin G labeled with 99mTc and with white blood cells labeled by 111 Indium-oxinate to investigate asymptomatic bowel inflammation. A concentration of labeled leukocytes in abdominal segments greater than that observed with human polyclonal immunoglobulin G was evident only in one patient. In five patients a slight concentration of both radiopharmaceuticals was reported, due to mild intestinal inflammatory response. These data might be related to mild increase of capillary permeability in the absence of inflammation leukocyte mediated. This study discloses a new cause of IgG-accelerated catabolism due to inflammatory bowel conditions without diarrhea in CVID patients.
肠病可能是免疫缺陷的首发表现，也可能在疾病过程中出现，并与原发性抗体疾病患者的吸收不良相关。对于这些患者，免疫球蛋白G（IgG）替代疗法可预防感染性和非感染性并发症。尽管如此，一些患者即使在没有蛋白质丢失综合征的情况下接受高剂量Ig治疗，也无法达到最佳的IgG谷值水平。我们调查了7例患有常见可变免疫缺陷（CVID）且接受高剂量Ig（600 - 800mg/kg/月）治疗但IgG谷值水平较低的患者。患者接受了用99mTc标记的人多克隆免疫球蛋白G和用111铟-奥昔萘酸标记的白细胞进行的腹部闪烁扫描，以研究无症状性肠道炎症。仅在1例患者中观察到腹部节段中标记白细胞的浓度高于用人多克隆免疫球蛋白G观察到的浓度。在5例患者中，由于轻度肠道炎症反应，报告了两种放射性药物均有轻微浓度。这些数据可能与在无炎症白细胞介导的情况下毛细血管通透性的轻度增加有关。这项研究揭示了CVID患者中由于无腹泻的炎症性肠病导致IgG加速分解代谢的一个新原因。
