Chronic periaortitis: a fibro-inflammatory disorder

doi:10.1016/j.berh.2008.12.002

Best Practice & Research Clinical Rheumatology

Volume 23, Issue 3, June 2009, Pages 339-353

https://doi.org/10.1016/j.berh.2008.12.002 Get rights and content

Chronic periaortitis includes a spectrum of rare conditions characterized by fibro-inflammatory tissue surrounding the abdominal aorta. Although it has been considered a localized inflammatory disease secondary to atherosclerosis, several clinico-laboratory findings suggest a systemic autoimmune origin; additionally, it may involve the thoracic aorta and the origin of its major branches, with a pattern similar to that of the large-vessel vasculitides. Its pathogenesis is still unclear. Computed tomography and magnetic resonance imaging are the modalities of choice for the diagnosis, whereas fluorodeoxyglucose/positron emission tomography emerges as a sensitive imaging modality to assess the inflammatory activity of the periaortic tissue. The treatment of chronic periaortitis is largely empirical, since no randomized trials have been carried out. Corticosteroids, immunosuppressants and endoscopic or surgical procedures must be appropriately combined for the correct management of chronic periaortitis patients.

Section snippets

Nomenclature

The confusion about the nomenclature of CP is due to the heterogeneity of the diseases it embraces and to its rarity, which so far has not allowed the development of diagnostic or classification criteria.

CP may develop around an undilated or a dilated aorta. In the former case, the definition of IRF is commonly used; in the latter, clinicians used to distinguish between perianeurysmal retroperitoneal fibrosis and IAAAs on the basis of the presence or absence, respectively, of encasement of

Epidemiology, genetic and environmental determinants

Epidemiology of CP is not well known; the only available epidemiological data concern IRF and IAAAs. A study performed in Finland in 2004 showed that IRF has an annual incidence of 0.1 per 100,000 people and a prevalence of 1.38 cases per 100,000 inhabitants [47]. No data are available about the incidence of secondary retroperitoneal fibrosis [4]. Epidemiological data regarding IAAAs show that they represent 4–10% of all abdominal aortic aneurysms *[48], [49], [50].

CP frequently develops in

Clinical manifestations

The onset of CP is often characterized by non-specific signs and symptoms. Lumbar, abdominal or flank pain is present in about 80% of the patients; it has been described as insidious, persistent and dull, unmodified by movement or rest. During the initial phases patients may find relief using non-steroidal anti-inflammatory drugs (NSAIDs), but the beneficial effect of these agents is transient *[2], [54]. If the ureters are involved, the pain may be acute and colic-like. In addition to pain,

Laboratory findings

There are no specific laboratory tests for the diagnosis of CP. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are usually high and are often used to monitor the clinical course of the disease *[2], [60], [62], [63]. Renal dysfunction is related to the severity of ureteral involvement; although very rarely an underlying glomerulonephritis may be present, thus proteinuria and urinary sediment should always be assessed [64]. Microscopic or macroscopic haematuria can be

Pathogenesis

The pathogenesis of CP is still unclear. The most popular theory, proposed by Parums and Mitchinson, suggests that it is due to a local inflammatory reaction against an antigen localized in the atherosclerotic plaques of the abdominal aorta [1], [3]. The precise target antigen has not been identified, but these investigators hypothesized that it is a modified lipid such as oxidized low-density lipoproteins (LDLs) or ceroid [1], [3], [67], [68]. Since an intact media constitutes an

Pathology

The classical macroscopic appearance of CP is that of a greyish mass infiltrating the retroperitoneal tissue surrounding the abdominal aorta and the iliac arteries and usually extending from the origin of the renal arteries to the pelvic brim. Perivascular involvement of the thoracic aorta is not uncommon, while rarely atypical localizations such as peri-duodenal, peri-pancreatic and pelvic sites have also been found [1], [5], *[32].

Microscopic examination reveals a sclerotic tissue and an

Imaging and differential diagnosis

Imaging studies are essential for the diagnosis of CP. Retroperitoneal biopsy is performed only when the retroperitoneal tissue has atypical localizations or when there are clinical and radiological signs of an underlying malignancy/infection; moreover, multiple biopsies must be performed if ureterolysis is carried out to relieve ureteral obstruction. We also recommend performing retroperitoneal biopsy if local clinical experience with CP is limited.

Computed tomography (CT) and magnetic

Treatment

Both medical and surgical approaches have a role in the management of CP patients, but no established therapeutic algorithm exists. The first goal of treatment is to relieve CP-related obstructive complications, primarily obstructive uropathy. However, treatment also needs to address other issues, such as inducing regression of the retroperitoneal mass, switching off the inflammatory response and the systemic symptoms it causes, and preventing relapses.

If CP has a typical clinical presentation

Summary

Chronic periaortitis includes idiopathic retroperitoneal fibrosis (non-aneurysmal form) and inflammatory abdominal aortic aneurysms (aneurysmal form); it may be isolated or associated with systemic inflammatory or autoimmune diseases, or diffuse sclerosing lesions. Secondary forms of retroperitoneal fibrosis must be ruled out. Additionally, for diagnostic and therapeutic purposes it must be distinguished from aortitis, which usually lacks the periaortic fibro-inflammatory reaction.

Chronic

Acknowledgements

The authors gratefully acknowledge Dr Domenico Corradi for kindly providing the histological pictures, and Professor Carlo Buzio for his continuous clinical and scientific support.

References (95)

A. Vaglio et al.
Retroperitoneal fibrosis
Lancet
(2006)
A. Vaglio et al.
Evidence of autoimmunity in chronic periaortitis: a prospective study
Am J Med
(2003)
R.M. Serra et al.
Perianeurysmal retroperitoneal fibrosis. An unusual cause of renal failure
Am J Med
(1980)
N. Sakalihasan et al.
Abdominal aortic aneurysm
Lancet
(2005)
A. Vaglio et al.
Retroperitoneal fibrosis: evolving concepts
Rheum Dis Clin North Am
(2007)
Y. Matsumoto et al.
A case of multiple immunoglobulin G4-related periarteritis: a tumorous lesion of the coronary artery and abdominal aortic aneurysm
Hum Pathol
(2008)
D. Corradi et al.
Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis
Kidney Int
(2007)
C. Salvarani et al.
Polymyalgia rheumatica and giant-cell arteritis
Lancet
(2008)
R. Virmani et al.
Sudden cardiac death
Cardiovasc Pathol
(2001)
B.T. Muller et al.
Mycotic aneurysms of the thoracic and abdominal aorta and iliac arteries: experience with anatomic and extra-anatomic repair in 33 cases
J Vasc Surg
(2001)

L.D. Kerr et al.

Occult active giant cell aortitis necessitating surgical repair

J Thorac Cardiovasc Surg

(2000)

T. Uibu et al.

Asbestos exposure as a risk factor for retroperitoneal fibrosis

Lancet

(2004)

K. Yusuf et al.

Inflammatory abdominal aortic aneurysm: predictors of long-term outcome in a case-control study

Surgery

(2007)

U. von Fritschen et al.

Inflammatory abdominal aortic aneurysm: A postoperative course of retroperitoneal fibrosis

J Vasc Surg

(1999)

O.F. Miller et al.

Presentation of idiopathic retroperitoneal fibrosis in the pediatric population

J Pediatr Surg

(2003)

G.S. Gilkeson et al.

Retroperitoneal fibrosis. A true connective tissue disease

Rheum Dis Clin North Am

(1996)

E.J. Doolin et al.

Familial retroperitoneal fibrosis

J Pediatr Surg

(1987)

P.G. Duffy et al.

Idiopathic retroperitoneal fibrosis in twins

J Urol

(1984)

T.E. Rasmussen et al.

Genetic risk factors in inflammatory abdominal aortic aneurysms: polymorphic residue 70 in the HLA-DR B1 gene as a key genetic element

J Vasc Surg

(1997)

A.H. Kardar et al.

Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration

J Urol

(2002)

E.F. van Bommel et al.

Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone

Am J Kidney Dis

(2007)

R. Marcolongo et al.

Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases

Am J Med

(2004)

D.V. Parums et al.

The localisation of immunoglobulin in chronic periaortitis

Atherosclerosis

(1986)

D.A. Breems et al.

The role of advanced atherosclerosis in idiopathic retroperitoneal fibrosis. Analysis of nine cases

Neth J Med

(2000)

A. Vaglio et al.

Autoimmune aspects of chronic periaortitis

Autoimmun Rev

(2006)

S. Tanaka et al.

Detection of active cytomegalovirus infection in inflammatory aortic aneurysms with RNA polymerase chain reaction

J Vasc Surg

(1994)

T. Tang et al.

Inflammatory abdominal aortic aneurysms

Eur J Vasc Endovasc Surg

(2005)

J.J. Kottra et al.

Retroperitoneal fibrosis

Radiol Clin North Am

(1996)

A.K. Srinivasan et al.

Comparison of laparoscopic with open approach for ureterolysis in patients with retroperitoneal fibrosis

J Urol

(2008)

M.J. Mitchinson

The pathology of idiopathic retroperitoneal fibrosis

J Clin Pathol

(1970)

D.V. Parums

The spectrum of chronic periaortitis

Histopathology

(1990)

L. Koep et al.

The clinical significance of retroperitoneal fibrosis

Surgery

(1977)

D. Hughes et al.

Idiopathic retroperitoneal fibrosis is a macrophage-rich process. Implications for its pathogenesis and treatment

Am J Surg Pathol

(1993)

P. Greco et al.

Tuberculosis as a trigger of retroperitoneal fibrosis

Clin Infect Dis

(2005)

C. Salvarani et al.

Positron emission tomography (PET): evaluation of chronic periaortitis

Arthritis Rheum

(2005)

N. Pipitone et al.

Images in cardiovascular medicine. Chronic periaortitis

Circulation

(2008)

H. Okada et al.

Retroperitoneal fibrosis and systemic lupus erythematosus

Nephrol Dial Transplant

(1999)

T.C. Tsai et al.

Retroperitoneal fibrosis and juvenile rheumatoid arthritis

Pediatr Nephrol

(1996)

A. Vaglio et al.

Peripheral inflammatory arthritis in patients with chronic periaortitis: report of five cases and review of the literature

Rheumatology (Oxford)

(2008)

R.S. de et al.

Periaortitis heralding Wegener's granulomatosis

J Rheumatol

(2002)

M.L. Hautekeete et al.

Retroperitoneal fibrosis after surgery for aortic aneurysm in a patient with periarteritis nodosa: successful treatment with corticosteroids

J Intern Med

(1990)

H. Ohara et al.

Systemic extrapancreatic lesions associated with autoimmune pancreatitis

J Gastroenterol

(2007)

A. Vaglio et al.

Chronic periaortitis: a spectrum of diseases

Curr Opin Rheumatol

(2005)

T. Kamisawa et al.

IgG4-related sclerosing disease

World J Gastroenterol

(2008)

T. Kamisawa et al.

Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease

J Gastroenterol

(2006)

H. Hamano et al.

Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis

Lancet

(2002)

T. Kamisawa

Is it time to reconsider autoimmune pancreatitis?

J Gastroenterol

(2006)

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In the last years, the usefulness of ¹⁸F-fluodeoxyglucose positron emission tomography/computed tomography (¹⁸F-FDG PET/CT) and ¹⁸F-fluodeoxyglucose positron emission tomography/magnetic resonance (¹⁸F-FDG PET/MR) for the evaluation of retroperitoneal fibrosis (RPF) has developed with encouraging evidences. These imaging methods are giving promising results in the initial evaluation of disease activity and extension, especially for the evaluation of RPF in IgG4-related disease (IgG4-RD). PET imaging is also particularly useful for the assessment of residual disease after medical therapy and for the follow-up. In general, patients with positive PET scan, at first evaluation or during subsequent evaluation, have higher levels of acute-phase reactant. Moreover, compared to other imaging modalities (CT or MR), ¹⁸F-FDG PET has the capacity to evaluate disease metabolic activity. Otherwise, PET/CT may be superior to conventional anatomical imaging and to acute-phase reactant for the evaluation RPF in revealing active inflammation in the prediction of treatment response and prognosis. Retroperitoneal malignancies are a frequently tends to enter in the differential diagnosis with RPF and moderate evidences are underlying the capacity of PET imaging to discriminate between these two entities. Moreover, hybrid functional imaging such as PET, for the evaluation of RFP, seems to be particularly comfortable, with moderate radiation exposure.
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Aortic involvement is relatively common in the context of IgG4-related disease (IgG4-RD). It includes IgG4-aortitis, and IgG4-(chronic) periaortitis (IgG4-CP). The latter overlaps with IgG4-retroperitoneal fibrosis (IgG4-RPF). Aortic wall thickening which characterizes these entities along with the presence of periaortic tissue in IgG4-CP, are often accompanied by aortic aneurysms, which belong to the group of the so-called inflammatory aneurysms.
Both the thoracic and abdominal aorta can be affected. Aortitis appears to involve more often the former, while the opposite is the case for IgG4-CP. There is a lack of definitions and different classification criteria have been used to describe these entities. This report provides an overview on the current evidence of aortic involvement in IgG4-RD. It discusses the clinical, epidemiologic, serologic and histopathologic characteristics, as well as the imaging techniques used for their diagnosis and the therapeutic options and treatment outcomes. The differential diagnosis and underlying pathogenetic mechanisms are also discussed.
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To evaluate the role of perfusion CT for monitoring inflammatory activity in patients with aortitis and chronic periaortitis undergoing immunosuppressive therapy.
Seventeen symptomatic patients (median age 68.5 years) who underwent perfusion-based computed tomography (CT) monitoring after diagnostic contrast-enhanced CT were retrospectively included in this study. Blood flow (BF), blood volume (BV), volume transfer constant (k-trans), time to peak, and mean transit time were determined by setting circular regions of interest in prominently thickened parts of the vessel wall or perfused surrounding tissue at sites where the perfusion CT color maps showed a maximum BF value. Differences in CT perfusion and, morphological parameters, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were tested for significance during therapy.
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3Chronic periaortitis: a fibro-inflammatory disorder

Section snippets

Nomenclature

Epidemiology, genetic and environmental determinants

Clinical manifestations

Laboratory findings

Pathogenesis

Pathology

Imaging and differential diagnosis

Treatment

Summary

Acknowledgements

Lancet

Am J Med

Am J Med

Lancet

Rheum Dis Clin North Am

Hum Pathol

Kidney Int

Lancet

Cardiovasc Pathol

J Vasc Surg

J Thorac Cardiovasc Surg

Lancet

Surgery

J Vasc Surg

J Pediatr Surg

Rheum Dis Clin North Am

J Pediatr Surg

J Urol

J Vasc Surg

J Urol

Am J Kidney Dis

Am J Med

Atherosclerosis

Neth J Med

Autoimmun Rev

J Vasc Surg

Eur J Vasc Endovasc Surg

Radiol Clin North Am

J Urol

The pathology of idiopathic retroperitoneal fibrosis

J Clin Pathol

The spectrum of chronic periaortitis

Histopathology

The clinical significance of retroperitoneal fibrosis

Surgery

Idiopathic retroperitoneal fibrosis is a macrophage-rich process. Implications for its pathogenesis and treatment

Am J Surg Pathol

Tuberculosis as a trigger of retroperitoneal fibrosis

Clin Infect Dis

Positron emission tomography (PET): evaluation of chronic periaortitis

Arthritis Rheum

Images in cardiovascular medicine. Chronic periaortitis

Circulation

Retroperitoneal fibrosis and systemic lupus erythematosus

Nephrol Dial Transplant

Retroperitoneal fibrosis and juvenile rheumatoid arthritis

Pediatr Nephrol

Peripheral inflammatory arthritis in patients with chronic periaortitis: report of five cases and review of the literature

Rheumatology (Oxford)

Periaortitis heralding Wegener's granulomatosis

J Rheumatol

Retroperitoneal fibrosis after surgery for aortic aneurysm in a patient with periarteritis nodosa: successful treatment with corticosteroids

J Intern Med

Systemic extrapancreatic lesions associated with autoimmune pancreatitis

J Gastroenterol

Chronic periaortitis: a spectrum of diseases

Curr Opin Rheumatol

IgG4-related sclerosing disease

World J Gastroenterol

Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease

J Gastroenterol

Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis

Lancet

Is it time to reconsider autoimmune pancreatitis?

J Gastroenterol

3
Chronic periaortitis: a fibro-inflammatory disorder