<span”>

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.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-and-laboratory-features-of-igg4-related-retroperitoneal-fibrosisperiarteritis-in-japan-retrospective-multicenter-study-of-99-cases/