Clinical Features of 10 Patients with IgG4-related Retroperitoneal Fibrosis

Abstract

Objective To elucidate the clinical characteristics of IgG4-related retroperitoneal fibrosis (RF). Methods IgG4-related RF was diagnosed when all of the following three criteria were fulfilled: retroperitoneal soft tissue masses surrounding the aorta and/or adjacent tissues, elevation of the serum IgG4 levels, and abundant infiltration of IgG4-positive plasma cells in at least one organ or site. Ten patients were diagnosed as having IgG4-related RF. Results The mean age at diagnosis was 70.1 years, and the male-to-female ratio was 1:0.6. Only two patients had initial symptoms predominantly related to RF (back pain and edema of the lower extremities), while the remaining eight patients reported initial symptoms due to associated diseases. On laboratory examination, a severe inflammatory reaction was observed in one patient. Elevation of the levels of serum IgG and IgE, eosinophilia and positivity of antinuclear antibodies were detected in seven, five, two and seven patients, respectively. The retroperitoneal masses were detected primarily in the left renal hilus in four patients, in the periaortic region in five patients and in both regions in one patient. Hydronephrosis was present in five patients. The histological diagnosis was confirmed in the retroperitoneal masses (resection, n=1 biopsy, n=2) and extraretroperitoneal lesions (n=7). Twenty-four other IgG4-related diseases were found to be associated with IgG4-related RF in nine patients (autoimmune pancreatitis (n=2), sialadenitis (n=4), dacryoadenitis (n= 5), lymphadenopathy (n=9), pulmonary pseudotumor (n=1) and pituitary pseudotumor (n=1)). Seven patients underwent steroid therapy, all of whom responded well and showed no instances relapse. Conclusion IgG4-related RF has several clinical characteristic features. Our diagnostic criteria may be helpful in obtaining a correct diagnosis.

IgG4‐related retroperitoneal fibrosis: a newly characterized disease

“…The average age was 66.4 years old (range 52–79). The male‐to‐female ratio was 3.3 : 1.0 (40 males and 12 females) …”

IgG4‐related disease

“…Soft tissue masses mostly surround the abdominal aorta, iliac arteries, ureters and renal hilum, and may result in ureteral obstruction and hydronephrosis (Fig. b) . In addition to periaortic involvement, lymphoplasmacytic aortitis has been reported in IgG4‐RD .…”

“…3b). 101,102 In addition to periaortic involvement, lymphoplasmacytic aortitis has been reported in IgG4-RD. [103][104][105][106] Aneurysm formation can occur with a risk of rupture and should be prevented by anti-inflammatory therapy.…”

Immunoglobulin G4-Related Retroperitoneal Fibrosis Treated with Hochuekkito, a Kampo Medicine, following Steroid Treatment

“…Accordingly, multifocal fibrosclerosis is now considered an IgG4-related disease [3]. Some individual cases and several case series of RF without obvious manifestations of AIP have been reported [9, 10]. In the present case, only the retroperitoneal space was observed to be affected, and no other sclerosing lesions were identified.…”

“…Other retroperitoneal masses such as malignant lymphomas and metastatic tumors may also be present. With regard to the histological criterion, histological findings from sites other than the retroperitoneal mass can be used, as performing biopsies of such masses is usually difficult [10]. Although our patient's mass was confined to the retroperitoneal space, we were able to biopsy the mass via minimal laparotomy and confirm the histological criterion.…”