Session Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect many organs. It frequently causes retroperitoneal/periarterial lesions, which are referred to as IgG4-related retroperitoneal fibrosis/periarteritis (RF/P). However, clinical and laboratory features of this disease have not been well clarified. This study aimed to clarify the clinical and laboratory features of IgG4-related RF/P. <span”>
Methods: We retrospectively evaluated clinical features including subjective symptoms, laboratory data, and imaging findings at diagnosis in 99 patients (pts) diagnosed with IgG4-related RF/P by experienced physicians. The diagnosis of this disease was made principally on the basis of the presence of consistent retroperitoneal/periarterial radiological findings, the fulfillment of the comprehensive diagnostic criteria or each set of organ-specific diagnostic criteria, and exclusion of other diseases. <span”>
Results: Eighty-four pts were men, and 15 were women (average age 67.4 years). At diagnosis, 42.4% of pts presented subjective symptoms including pain (23.2%), fever (8.1%), and edema (4.1%). Allergic predisposition was found in 37.4% of pts. Hydronephrosis was detected in 21.2%. Current or past smoking was present in 65.4%.<span”>IgG4-related other organ involvement was detected in 69.7%, and the average number of involved other organs was 1.9 (range: 0-8). Serologically, the average serum IgG4 level was 851 mg/dL (range: 59-3610), and 91.8% of pts had serum IgG4 > 135 mg/dL. Hypocomplementemia was observed in 24.4%. An elevated serum C-reactive protein (CRP) level (> 1 mg/dL) was found in 23.6%. The affected aorta/artery comprised mainly 8 thoracic aortas, 67 abdominal aortas, 50 iliac arteries, 8 mesenteric arteries, and 9 coronary arteries. Luminal dilatation of the affected lesions at diagnosis was observed in 26.3% of pts. On the other hand, 24.3% had retroperitoneal, periureteral, or renal pelvic lesions.<span”> Compared with pts without biopsy of the retroperitoneal/periarterial lesions, pts with it showed significantly lower serum IgG4 levels (643 vs 953 mg/dL, P=0.011) and IgG4/IgG ratio (23.9 vs 31.1 %, P=0.022), higher serum IgE (1,533 vs 543 IU/mL, P=0.020) and CRP levels (1.65 vs 0.56 mg/dL, P=0.025), lower incidence of other organ involvement (30.3 vs 86.4 %, P<0.001) and allergy (21.2 vs 45.5 %, P=0.027), and higher incidence of luminal dilatation (42.4 vs 18.2 %, P=0.015), pain (36.4 vs 16.7 %, P=0.043), and current smoking (58.8 vs 26.6 %, P=0.020). Multivariate logistic regression analysis indicated that the presence of other organ involvement [Odds ratio (OR); 0.011, P<0.001], serum CRP elevation (OR; 3.81, P=0.002), and the presence of iliac artery lesion (OR; 0.049, P=0.008) had an independent influence on the performance of biopsies of the retroperitoneal/periarterial lesions.
Conclusion: The present study clarified the clinical and laboratory features of IgG4-related RF/P, and suggested the possibility that biopsy of the retroperitoneal/periarterial lesions was avoidable in pts with other organ involvement.
To cite this abstract in AMA style:Mizushima I, Kasashima S, Yamamoto M, Saeki T, Yamada K, Inoue D, Kasashima F, Matsumoto Y, Amiya E, Notohara K, Sato Y, Zen Y, Kawa S, Kawano M, Ishizaka N. Clinical and Laboratory Features of IgG4-Related Retroperitoneal Fibrosis/Periarteritis in Japan: Retrospective Multicenter Study of 99 Cases [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/clinical-and-laboratory-features-of-igg4-related-retroperitoneal-fibrosisperiarteritis-in-japan-retrospective-multicenter-study-of-99-cases/. Accessed October 20, 2020.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-laboratory-features-of-igg4-related-retroperitoneal-fibrosisperiarteritis-in-japan-retrospective-multicenter-study-of-99-cases/