Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People's Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.

Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach.

Retroperitoneal fibrosis (RPF) is an uncommon disease characterized by a fibrous reaction that takes place in the peri-aortic retroperitoneum and often entraps the ureters causing obstructive uropathy. RPF is idiopathic in the majority of cases, but can also be secondary to malignancies, infections, drugs, radiotherapy, and rare histiocytic disorders such as Erdheim-Chester disease. Idiopathic RPF is an immune-mediated disease, which can either be isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibro-inflammatory disorder recently renamed as IgG4-related disease. The differential diagnosis between idiopathic, IgG4-related and secondary RPF is crucial, essentially because the therapeutic approaches - especially of idiopathic vs. secondary RPF - can be dramatically different. This review focuses on the clinical, laboratory and imaging features of the different RPF forms, and also provides an overview of the available treatment options.

Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation.

Retroperitoneal fibrosis (RPF) is an inflammatory disorder that affects the infrarenal great vessels and surrounding structures. Although first described in 1948 by Ormond, much of the information currently available on this disease is conflicting and results from multiple definitions used by different investigators. We conducted the current study to describe the clinical, laboratory, and radiographic presentation of RPF from a single center using data collected in a prospective fashion.Data on all patients who were referred to our RPF clinic were prospectively collected. We obtained information on presenting signs and symptoms, and on the presence or absence of "traditional risk factors," comorbidities, and family histories. All patients underwent the same battery of laboratory testing, including complete blood count, renal function profile, erythrocyte sedimentation rate (ESR), thyroid-stimulating hormone, and antinuclear antibodies (ANA). All patients had contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) of the abdomen and pelvis. The extent of disease was classified based on the anatomic location of the soft-tissue density.Forty-eight patients (26 male and 22 female) met the criteria for RPF and were included in the study. The mean age was 54.25 years. Few patients had traditional risk factors for RPF. Pain and weight loss were the most common presenting symptoms. The mean hemoglobin was 11.6 g/dL. The mean ESR was 40.5 mm/h. Nine patients had positive ANA, all with negative antibodies to dsDNA. CT or MRI revealed the presence of a periaortic soft-tissue density in all (100%) patients; 69% had pericaval involvement, 62% had obstruction of 1 or both ureters, and 35% of patients had involvement of the renal artery or renal vein.In the current report we present the demographics and laboratory and radiographic presentation of a homogenous group of patients with RPF. We attempt to solidify a proper scheme of classification for the disease, and suggest an anatomic classification system that may be used for future studies.

Ormond's disease or secondary retroperitoneal fibrosis? An overview of retroperitoneal fibrosis.

Retroperitoneal fibrosis represents a rare inflammatory disease. About two thirds of all cases seem to be idiopathic (= Ormond's disease). The remaining one third is secondary and may be ascribed to infections, trauma, radiation therapy, malignant diseases, and the use of certain drugs. Up to 15 % of patients have additional fibrotic processes outside the retroperitoneum. The clinical symptoms of retroperitoneal fibrosis are non-specific. In sonography retroperitoneal fibrosis appears as a retroperitoneal hypoechoic mass which can involve the ureters and thus cause hydronephrosis. Intravenous urography and MR urography can demonstrate the typical triad of medial deviation and extrinsic compression of the ureters and hydronephrosis. CT and MRI are the modalities of choice for the diagnosis and follow-up of this disease. The lesion typically begins at the level of the fourth or fifth lumbar vertebra and appears as a plaque, encasing the aorta and the inferior vena cava and often enveloping and medially displacing the ureters. In unenhanced CT, retroperitoneal fibrosis appears as a mass that is isodense with muscle. When using MRI, the mass is hypointense in T 1-weighted images and of variable intensity in T 2-weighted images according to its stage: it may be hyperintense in early stages, while the tissue may have a low signal in late stages. After the administration of contrast media, enhancement is greatest in the early inflammatory phase and minimal in the late fibrotic phase. Dynamic gadolinium enhancement can be useful for assessing disease activity, monitoring response to treatment, and detecting relapse. To differentiate retroperitoneal masses, diffusion-weighted MRI may provide useful information.

[Retroperitoneal fibrosis, M. Ormond, periaoartitis, ...?]. / Retroperitoneale Fibrose, Morbus Ormond, chronische Periaortitis, ...?

Retroperitoneal fibrosis or Morbus Ormond is a rare disease characterized by inflammatory fibrosis of the retroperitoneal space and the abdominal aorta often including the common iliac arteries. The abdominal aorta can be enlarged leading to a classification of retroperitoneal fibrosis with or without aneurysm of the abdominal aorta. The classic form of retroperitoneal fibrosis usually presents without aneurysm of the abdominal aorta. Clinical presentation is not specific and includes abdominal, flank or back pain. Mechanisms, initiating factors and pathophysiology of the disease have remained unclear to date, however there is evidence that autoimmune mechanisms are involved. The most relevant complication of retroperitoneal fibrosis is the involvement of the ureter leading to obstructive nephropathy and requiring relief from obstruction. Because of the low frequency of the disease associated with a great variety of presentation no randomized controlled trials are available. Drug therapy is limited to steroids alone or in combination with immunosuppressive drugs such as azathioprine or even cyclophosphamide. The response to medical therapy is variable from patient to patient. Clinical endpoints are general symptoms, sedimentation rate, and imaging studies with abdomen CT or MRI.

Fibrosis retroperitoneal: comunicación de 4 casos / Retroperitoneal fibrosis: a report of 4 cases

Presentamos cuatro casos de fibrosis retroperitoneal (FRP) cuyo diagnóstico fue realizado durante los últimos tres años. Los métodos de elección en la actualidad son la tomografía computada (TC) y la resonancia magnética (RM). La TC muestra una masa isodensa que engloba la aorta y la vena cava inferior así como la compresión ureteral, (utilizando contraste endovenoso). La RM muestra una placa fibrosa hipointensa en T1, que rodea los grandes vasos. El tratamiento de la FRP primaria o idiopática se basa en resolver la obstrucción ureteral, reestablecer la función renal y prevenir infecciones. En la FRP secundaria, el tratamiento está focalizado a la resolución de la patología asociada (AU)

The spectrum of chronic periaortitis.

A review of the histology of 440 sections of atherosclerotic aortas and arteries showed that 85% contained advanced atherosclerotic plaques. Of these, 92% showed some degree of adventitial inflammation with subclinical chronic periaortitis in 49%. A review of 20 cases of clinical chronic periaortitis, which included 12 cases of inflammatory aneurysm and 8 cases of idiopathic retroperitoneal fibrosis, showed that there were no significant differences between them apart from an increase in aortic diameter in the former. The term chronic periaortitis is appropriate for the spectrum of subclinical and clinical forms of chronic adventitial inflammation associated with advanced atherosclerosis and medial thinning.