Krasselt Marco, Hoyer Bimba
Medizinische Klinik III - Endokrinologie, Nephrologie und Rheumatologie, Bereich Rheumatologie, Universitätsklinikum Leipzig AöR, Liebigstr. 20, 04103, Leipzig, Deutschland.
Rheumatologisches Zentrum (GBA) Leipzig, Leipzig, Deutschland.
Inn Med (Heidelb). 2025 Apr 1. doi: 10.1007/s00108-025-01891-8.
Immunoglobulin G4-related disease (IgG4-RD) is a rare and clinically diverse entity with an estimated incidence of 0.8-1.4 per 100,000 person-years. Typical sites of manifestation include the head and neck area, the hepato-pancreato-biliary system, and the retroperitoneum. Perhaps the best-known variant is retroperitoneal fibrosis, formerly known as Ormond's disease. Biopsy and histological examination are of great diagnostic importance, as relying solely on serum IgG4 is problematic and not sufficiently specific. Therapeutically, in addition to the initial administration of glucocorticoids, treatment with B cell-directed antibodies, particularly rituximab, has become clinically established. Initial studies demonstrate the effectiveness of other therapies targeting B cells. Although the disease has received more clinical attention in recent years, it is still necessary to further increase awareness of IgG4-RD to enable optimal treatment for patients.
免疫球蛋白G4相关疾病（IgG4-RD）是一种罕见且临床表现多样的疾病，估计发病率为每10万人年0.8-1.4例。典型的表现部位包括头颈部区域、肝胰胆系统和腹膜后。也许最广为人知的变体是腹膜后纤维化，以前称为奥蒙德病。活检和组织学检查具有重要的诊断意义，因为仅依靠血清IgG4存在问题且特异性不足。在治疗方面，除了最初给予糖皮质激素外，使用B细胞导向抗体，特别是利妥昔单抗进行治疗已在临床上确立。初步研究证明了其他针对B细胞疗法的有效性。尽管近年来该疾病受到了更多的临床关注，但仍有必要进一步提高对IgG4-RD的认识，以便为患者提供最佳治疗。
