Spandorfer Robert, Ahmad Madiha, Khosroshahi Arezou
From the Hospital for Special Surgery, Division of Rheumatology, New York, NY.
Division of Rheumatology, Atlanta VA Medical Center.
J Clin Rheumatol. 2023 Jan 1;29(1):23-28. doi: 10.1097/RHU.0000000000001895. Epub 2022 Jul 30.
INTRODUCTION/OBJECTIVES: Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous fibroinflammatory condition. The 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria for IgG4-RD were published to provide unified classification criteria in clinical research. The purpose of this study was to characterize demographics, disease manifestations, and treatments of patients with IgG4-RD and assess performance of the Classification Criteria in a heterogeneous cohort with a large population of Black patients.
This was a medical records review of all patients referred to a specialized IgG4-RD clinic. Demographics, serology, histopathology, disease manifestations, and treatment information were collected and analyzed. An ACR/EULAR Classification Criteria score for IgG4-RD was calculated to compare performance in definite diagnosis, probable diagnosis, and mimicker groups.
A total of 198 patients were evaluated. Eighty-five (43%) were mimickers. Of the remaining 114, 58 (51%) were classified as definite, and 56 (49%) as probable cases by treating clinicians. Pancreatitis was the most common presentation (37%) among 28 different organ manifestations of IgG4-RD. In patients with definite clinical diagnosis of IgG4-RD, 84% met the IgG4-RD Classification Criteria (i.e., score ≥20) with mean score of 29. Only 9% of the probable cases met this threshold with an average score of 8. None of the mimickers met the Classification Criteria.
This study highlights the broad spectrum of IgG4-RD and validates the use of the ACR/EULAR Classification Criteria for IgG4-RD, including a large proportion of Black patients. As shown in this study, IgG4-RD is a heterogeneous disease and continues to represent a diagnostic challenge to clinicians.
引言/目的：免疫球蛋白G4相关疾病（IgG4-RD）是一种异质性纤维炎症性疾病。2019年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）发布了IgG4-RD分类标准，以便在临床研究中提供统一的分类标准。本研究的目的是描述IgG4-RD患者的人口统计学特征、疾病表现和治疗情况，并评估该分类标准在包含大量黑人患者的异质性队列中的表现。
这是一项对所有转诊至专门的IgG4-RD诊所的患者的病历回顾。收集并分析了人口统计学、血清学、组织病理学、疾病表现和治疗信息。计算IgG4-RD的ACR/EULAR分类标准评分，以比较其在确诊、可能诊断和疑似组中的表现。
共评估了198例患者。其中85例（43%）为疑似患者。在其余114例中，经治疗的临床医生将58例（51%）分类为确诊，56例（49%）分类为可能病例。胰腺炎是IgG4-RD的28种不同器官表现中最常见的表现（37%）。在临床确诊为IgG4-RD的患者中，84%符合IgG4-RD分类标准（即评分≥20），平均评分为29分。只有9%的可能病例达到该阈值，平均评分为8分。没有疑似患者符合分类标准。
本研究突出了IgG4-RD的广泛范围，并验证了ACR/EULAR IgG4-RD分类标准的应用，该队列包括很大比例的黑人患者。如本研究所示，IgG4-RD是一种异质性疾病，对临床医生来说仍然是一个诊断挑战。
