Abstract
Objective Our aim was to group cases of idiopathic retroperitoneal
fibrosis (IRPF) into those associated with immunoglobulin (Ig) G4-related
disease (IgG4-RD) (IgG4-RPF) and those not associated with IgG4-RD
(non-IgG4-RPF) and to compare the clinical presentation, laboratory findings,
imaging, and treatment methods.
Methods A total of 46 patients were included in the study. According to
the assessment by two experienced rheumatologists and the IgG4-RPF
classification criteria published by the American College of
Rheumatology/European League Against Rheumatism (ACR/EULAR) in 2019, 11 patients
who were admitted as IgG4-RPF were included in the IgG4-RPF group and 35
patients who were not admitted as IgG4-RPF were included in the non-IgG4-RPF
group. The clinical and demographic characteristics, affected sites, laboratory
results and treatments were retrospectively evaluated and compared.
Results The mean age of our patients was 50.69 (±11.33) in the
non-IgG4-RPF group and 55.36 (±7.80) in the IgG4-RPF group and was similar in
both groups (p=0.340). The male gender dominated in both groups. Although there
was no significant difference between the groups in terms of acute renal failure
(p=0.074), this was more frequent in the IgG4-RPF group (81.82%). An increased
IgG4 concentration was found in about half (n=6, 54.55%) of the patients in the
IgG4-RPF group, while this was present in only 5.71% (n=2) of the patients in
the non-IgG4-RPF group (p=0.001). The most commonly used diagnostic imaging
modality was computed tomography. Biopsy was performed in almost all patients
(n=10, 90.91%) in the IgG4-RPF group and 11 (31.43%) in the non-IgG4-RPF group
(p<0.001). Drug treatment alone and drug treatment in combination with
interventional treatment were the most commonly used treatment modalities in the
non-IgG4-RPF group (n=12, 34.29% for both). In the IgG4-RPF group, the
combination of medical and interventional treatment was the most commonly used
treatment modality (n=5, 45.45%). No significant difference was found between
the two groups in the assessment of treatment success (p>0.05).
Conclusion IRPF can present with similar clinical and imaging findings in
IgG4-RPF and non-IgG4-RPF groups. Since a similar treatment success can be
achieved in both groups with similar therapeutic agents, we believe that a
biopsy is not necessary in patients with an uncertain diagnosis of IRPF.
Keywords
biopsy - computed tomography - idiopathic retroperitoneal fibrosis - immunoglobulin G4-related disease - medical therapy
Schlüsselwörter
biopsie - computer tomographie - idiopathische retroperitoneale fibrose - immunglobulin G4-bedingte erkrankung - medizinische therapie
