Chronic Periaortitis Research Articles

Human dental pulp stem cells (hDPSCs) promote the lipofibroblast transition in the early stage of a fibro-inflammatory process.

Introduction: In autoimmune diseases, particularly in systemic sclerosis and chronic periaortitis, a strict correlation between chronic inflammation and fibrosis exists. Since the currently used drugs prove mostly effective in suppressing inflammation, a better comprehension of the molecular mechanisms exerted by cell types implicated in fibro-inflammation is needed to develop novel therapeutic strategies. Mesenchymal stromal/stem cells (MSCs) are being matter of deep investigation to unveil their role in the evolution of fibrogenetic process. Several findings pointed out the controversial implication of MSCs in these events, with reports lining at a beneficial effect exerted by external MSCs and others highlighting a direct contribution of resident MSCs in fibrosis progression. Human dental pulp stem cells (hDPSCs) have demonstrated to hold promise as potential therapeutic tools due to their immunomodulatory properties, which strongly support their contribution to tissue regeneration. Methods: Our present study evaluated hDPSCs response to a fibro-inflammatory microenvironment, mimicked in vitro by a transwell co-culture system with human dermal fibroblasts, at early and late culture passages, in presence of TGF-β1, a master promoter of fibrogenesis. Results and Discussion: We observed that hDPSCs, exposed to acute fibro-inflammatory stimuli, promote a myofibroblast-to-lipofibroblast transition, likely based on BMP2 dependent pathways. Conversely, when a chronic fibro-inflammatory microenvironment is generated, hDPSCs reduce their anti-fibrotic effect and acquire a pro-fibrotic phenotype. These data provide the basis for further investigations on the response of hDPSCs to varying fibro-inflammatory conditions.

Pilot study of tocilizumab monotherapy for active chronic periaortitis

ObjectivesTo investigate the effectiveness and safety of TCZ (tocilizumab) monotherapy for chronic periaortitis (CP) patients at acute active stage.MethodsTwelve patients with definite or possible diagnosis of CP were enrolled and...

Non-infectious diseases of the aorta and large arteries

This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.

Chronische Periaortitis

Chronic Periaortitis Abstract. The term chronic periaortitis encompasses several fibroinflammatory diseases that manifest on or around the abdominal aorta which may be associated with aneurysm formation. Formerly distinct entities are grouped together, such as idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. They may also occur IgG4-related. The most common complications of these systemic inflammatory diseases are ureteric obstruction and secondary renal failure, but venous compression with congestion and thrombosis may also occur. This review focuses on the diagnosis and treatment of chronic periaortitis. The diagnosis is usually made by cross-sectional imaging, and various therapies, especially immunosuppressive agents, are used therapeutically, although good evidence is largely lacking. For bridging, a ureteric stent is often placed and in individual cases surgical therapy is also necessary. Due to the heterogeneity of the diseases and their rarity, there are hardly any standardized treatment procedures.

Comprehensive assessment and outcomes of patients with chronic periaortitis.

Chronic periaortitis (CP) is a less known but more frequently diagnosed fibro-inflammatory disorder, but we know little about it and data regarding follow-up and outcome are still very limited. This study aims to identify the clinicopathologic, laboratory, and radiologic features, as well as outcomes of CP patients. Patients with CP from HUVAC database were included in the study. CP was diagnosed based on compatible imaging findings and histopathological evaluation (if available), in addition to clinical findings. Demographics, laboratory, clinical, and imaging data were retrospectively reviewed from medical records. A total of 51 (male/female:37/14) patients were included in the study. Median (IQR) age was 63 (53-69) years and follow-up duration was 40 (4-60) months. 32 of the patients were IgG4-related CP. The most common form of CP in our cohort was idiopathic retroperitoneal fibrosis (82%), followed by inflammatory abdominal aortic aneurysms (12%) and peri-aneurysmal retroperitoneal fibrosis (8%). 8 (15.6%) patients had thoracic periaortitis and 16 (31.6%) venous involvement. Cyclophosphamide (CYC) combined with steroids was the most preferred treatment modality (43%), followed by rituximab (RTX) (31.3%). Follow-up imaging was done after a median (IQR) of 7(3-11) months, 30% of the patients were stable and 64.1% showed regression. A total of 18 (35.2%) had been taken off therapy at the last visit. Idiopathic retroperitoneal fibrosis was the most frequent presentation, whereas 15.6% of patients had thoracic involvement. Venous involvement was also not uncommon. Optimal time for follow-up imaging was determined as 6-9 months. Steroids along with CYC/RTX had a favourable outcome in the treatment of these patients.

Incidence, prevalence and mortality of chronic periaortitis: a population-based study.

To evaluate the epidemiology, presentation and outcomes of patients with chronic periaortitis from 1998 through 2018. An inception cohort of patients with incident chronic periaortitis from January 1, 1998 through December 31, 2018, in Olmsted County, Minnesota was identified based on comprehensive individual medical record review utilising the Rochester Epidemiology Project medical record linkage system. Inclusion required radiographic and/or histologic confirmation of periarterial soft tissue thickening around at least part of the infra-renal abdominal aorta or the common iliac arteries. Data were collected on demographic characteristics, clinical presentation, renal and radiographic outcomes, and mortality. Incidence rates were age and sex adjusted to the 2010 United States white population. Eleven incident cases of chronic periaortitis were identified during the study period. Average age at diagnosis was 61.8±13.4 years. The cohort included 9 men (82%) and 2 women (18%). Age- and sex-adjusted incidence rates per 100,000 population were 0.26 for females, 1.56 for males and 0.87 overall. Overall prevalence on January 1, 2015 was 8.98 per 100,000 population. Median (IQR) length of follow-up was 10.1 (2.5, 13.8) years. Overall mortality was similar to the expected age, sex, and calendar estimates of the Minnesota population with standardised mortality ratio (95% CI) for the entire cohort 2.07 (0.67, 4.84). This study reports the first epidemiologic data on chronic periaortitis in the United States. In this cohort of patients with chronic periaortitis, men were 4 times more commonly affected than women. Mortality was not increased compared to the general population.

Diagnostic Approaches for Large Vessel Vasculitides.

The large vessel vasculitides comprise giant cell arteritis (GCA), Takayasu arteritis (TAK), and chronic periaortitis. The diagnostic approach to these conditions involves the correct use and interpretation of clinical criteria, imaging techniques, and, in case of GCA, temporal artery biopsy. Ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) reveal a homogeneous, concentric, thickening of the arterial wall. MRI and CT may also reveal aneurysms and stenoses. 18F-Fluorodeoxyglucose (FDG)-PET shows increased FDG uptake of inflamed artery walls delineating increased metabolic activity. Ultrasound, FDG-PET, and MRI are the recommended imaging techniques in GCA and TAK. In patients with a high suspicion of GCA who present with visual disturbances, initiation of high-dose intravenous corticosteroids should not be delayed by imaging. Extracranial large vessel vasculitis may be confirmed by all three modalities, particularly by FDG-PET in case of atypical clinical pictures. In this article, we review the role of the GCA and TAK ACR classification criteria, temporal artery biopsy, conventional angiography, ultrasound, MRI, magnetic resonance angiography (MRA), CT angiography (CTA), and FDG-PET in the diagnostic approach of large vessel vasculitis.

Bile Acids Elevated in Chronic Periaortitis Could Activate Farnesoid-X-Receptor to Suppress IL-6 Production by Macrophages.

Chronic periaortitis (CP) is a rare autoimmune disease without effective treatment. By analyzing the serum bile acid spectrum in 28 CP patients with the ultra-performance liquid chromatography-tandem mass spectrometry, we found that the bile acids were significantly altered in CP patients, with significant increases in chenodeoxycholic acid (CDCA) and glycochenodeoxycholic acid (GCDCA) and decrease in deoxycholic acid (DCA). Signaling pathway enrichment analysis from the RNA sequencing results suggested that the altered gene sets in PBMC of CP patients were associated with bile acid metabolism. Furthermore, we found that pathological concentration of CDCA could significantly inhibited IL-6 expression in RAW 264.7 cells after LPS stimulation. Since CDCA is a well-known natural high-affinity ligand for the bile acid receptor farnesoid-x-receptor (FXR) while GW4064 is the synthetic specific agonist of this receptor, we then revealed that GW4064 significantly decreased IL-6 expression in RAW 264.7 cells and bone marrow-derived macrophages but not in FXR-/- macrophages upon LPS stimulation. The western blot results with the anti-FXR antibody showed significantly increased expression in the nuclear proportion, suggesting that FXR agonist promoted the transportation of FXR into the nucleus but did not increase the FXR expression in macrophages. Dual-luciferase report assay and ChIP assay demonstrated that upon activation, FXR could directly bind to the promoter site of IL-6, leading to the decreased expression of IL-6. Thus, bile acids, especially CDCA, may operate to damp inflammation via FXR-mediated downregulation of IL-6 in mononuclear cells and provide a protective mechanism for CP patients.

PROBLEMY W DIAGNOSTYCE ZWŁÓKNIENIA ZAOTRZEWNOWEGO

Retroperitoneal fibrosis (RPF) is a uncommon disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles the ureters or abdominal organs. The disease may be idiopathic or secondary to infections, malignancies, certain drugs or radiotherapy. Idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in aorta. In the article the most important diagnostic problems of RPF are reviewed.

Retroperitoneal Fibrosis Secondary to the Placement of Aorto-iliac Stents

Introduction: Retroperitoneal fibrosis (RF) is a rare disorder characterized by inflammation and fibrosis in the periphery of the abdominal aorta (1). In about two thirds of the cases this condition is idiopathic (2). It is known that inflammatory aneurysms could be associated with RF and regression of periaortic inflammation after endovascular treatment (EVAR) of the aneurysm was described (3). However, very little is known about the possibility of this condition being associated with the implantation of endovascular devices. In the literature there is only one report of a clinical case in which the RF was secondary to the implantation of a kissing stent of the common iliac arteries (4). Periaortitis and periaortic fibro-inflammatory alterations secondary to stent graft implantation in the correction of abdominal aortic aneurysms have also been demonstrated, with 5 cases described in the literature (5,6,7,8,9). In some of these cases, patients presented complications of RF, such urethral obstruction with hydronephrosis. Possibly, since the symptoms of the disease are non-specific, the condition tends to be underreported and underdiagnosed. The pathophysiology of this pathological process is not known; however, it was proposed that the angioplasty and stenting can disturb plaque integrity with antigen exposure, triggering a local inflammatory response (10). Methods: We describe two cases of retroperitoneal fibrosis secondary to endovascular intervention of arterial aortic iliac axes with angioplasty and stent/stent-graft placement. Results: We present our experience with two patients with Leriche Fontaine IIb arteriopathy, one with pre-occlusive stenosis of the left common iliac artery treated with percutaneous transluminal angioplasty (PTA) and stenting, other with morphologically significant stenosis of the infrarenal abdominal aorta where PTA and kissing stent with two aortoiliac stent-grafts was performed. Following the endovascular procedure, both patients had manifested marked lumbar pain and elevation of serum inflammatory markers. A periaortic inflammatory process suggestive of retroperitoneal fibrosis was visualized on computed tomography angiography (CTA). In both patients the clinical condition was reverted with steroid therapy without recurrence of symptoms. Conclusion: These cases demonstrate the possible relationship between the implantation of aortoiliac stents and the development of retroperitoneal fibrosis. The published literature is very scarce. This complication, like all forms of chronic periaortitis, responds well to corticotherapy with symptom resolution. Disclosure: Nothing to disclose

Fibrocytes in Chronic Periaortitis: A Novel Mechanism Linking Inflammation and Fibrosis.

Chronic periaortitis (CP) is a rare disease characterized by periaortic and periiliac fibroinflammatory tissue. The pathogenic mechanisms leading to tissue accumulation and activation of fibroblasts are unclear. This study was undertaken to explore the role of fibrocytes, circulating precursors of tissue fibroblasts, in patients with CP. We studied 44 patients with newly diagnosed CP and 30 healthy controls. Circulating fibrocytes were identified as Col1+CD45+ cells using flow cytometry. Retroperitoneal tissue biopsy samples from 9 CP patients were stained with anti-type I procollagen, anti-CXCR4, and anti-CD45 antibodies and analyzed by confocal microscopy to detect tissue-infiltrating fibrocytes. Circulating levels and tissue expression of CXCL12, a CXCR4 ligand that promotes fibrocyte homing, were investigated using enzyme-linked immunosorbent assay and immunohistochemistry, respectively. We also characterized T helper polarization in biopsy samples from CP patients and measured serum levels of a panel of cytokines that are hallmarks of T helper responses and capable of influencing fibrocyte differentiation. The frequency of circulating Col1+CD45+ fibrocytes was higher in patients than in controls (P = 0.0371). CD45+proCol1+ and CXCR4+proCol1+ cells were detected in all examined biopsy samples from CP patients. Serum levels of CXCL12 were also higher in CP patients than controls (P = 0.0056), and tissue-infiltrating inflammatory cells intensely expressed CXCL12. Increased serum levels of Th2 cytokines (e.g., interleukin-13 [IL-13] and IL-10) were found in patients, and immunohistochemistry revealed a dominant infiltration of GATA-3+ cells, also indicating Th2 polarization; Th2-skewed responses are known to promote fibrocyte differentiation. Our findings indicate that fibrocytes are enriched in the peripheral blood of CP patients and infiltrate target lesions. The accumulation of fibrocytes in the pathologic tissue might be driven by CXCL12, and Th2-skewed immune responses are likely to facilitate their differentiation.

Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients.

Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown.Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis).Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls.Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.

Endovascular treatment of immunoglobulin G4-related chronic periaortitis of abdominal aorta: a case report

免疫球蛋白G4相关慢性腹主动脉周围炎是一种罕见疾病，其临床表现无特异性，主要依靠影像学及免疫学检查辅助诊断，应予早期治疗。该文报道1例67岁男性免疫球蛋白G4相关慢性腹主动脉周围炎患者的诊疗，以加强对该疾病的认识。对于其并发的动脉瘤性疾病，腔内修复术是一种安全和有效的治疗选择。.

Chronic Periaortitis: an Update.

We aim to review traditional concepts and recent developments on the nosology, pathophysiology, clinical phenotypes and treatment of chronic periaortitis (CP). CP is a rare disorder hallmarked by a periaortic fibro-inflammatory tissue. It can present as an isolated disease, but it can also be associated with other autoimmune and fibro-inflammatory lesions (e.g., fibrosing mediastinitis, sclerosing pancreato-cholangitis) that are part of the spectrum of IgG4-related disease. In a subgroup of patients, it also involves the thoracic aorta (so-called "diffuse periaortitis"), which supports the notion of an inflammatory disorder of large arteries. The pathogenesis of CP is multifactorial: recent studies have elucidated the predisposing role of immunogenetic variants and exposures to environmental agents such as smoking and asbestos. CP is a rare immune-mediated disease that affects the abdominal aorta and the iliac arteries and, in some cases, the thoracic aorta. It may overlap with manifestations of IgG4-related disease, and its treatment comprises glucocorticoids, conventional and biological immunosuppressive agents.

Contrast-enhanced ultrasound of the abdominal aorta - current status and future perspectives.

Ultrasound has been established as an important diagnostic tool in assessing vascular abnormalities. Standard B-mode and Doppler techniques have inherent limitations with regards to detection of slow flow and small vasculature. Contrast-enhanced ultrasound (CEUS) is a complementary tool and is useful in assessing both the macro- and microvascular anatomy of the aorta. CEUS can also provide valuable physiological information in real-time scanning sessions due to the physical and safety profiles of the administered microbubbles. From a macrovascular perspective, CEUS has been used to characterize aortic aneurysm rupture, dissection and endoleaks post-EVAR repair. With regard to microvasculature CEUS enables imaging of adventitial vasa vasorum thereby assessing aortic inflammation processes, such as monitoring treatment response in chronic periaortitis. CEUS may have additional clinical utility since adventitial vasa vasorum has important implications in the pathogenesis of aortic diseases. In recent years, there have been an increasing number of studies comparing CEUS to cross-sectional imaging for aortic applications. For endoleak surveillance CEUS has been shown to be equal or in certain cases superior in comparison to CT angiography. The recent advancement of CEUS software along with the ongoing development of drug-eluting contrast microbubbles has allowed improved targeted detection and real-time ultrasound guided therapy for aortic vasa vasorum inflammation and neovascularization in animal models. Therefore, CEUS is uniquely suited to comprehensively assess and potentially treat aortic vascular diseases in the future.

Diffusion-weighted MRI and PET–CT in the follow up of chronic periaortitis

Aim of the present study is to compare magnetic resonance imaging (MRI) and positron emission tomography (PET) parameters in the follow up of chronic periaortitis (CP), with a focus on changes in the apparent diffusion coefficient (ADC) and standardized uptake values (SUV). 127 patients with CP were treated in our urology between 2007 and 2017. We identified 14 patients with parallel abdominal MRI and PET-CT examinations before therapy and in the follow up resulting in a total of 56 examinations. Relative contrast uptake and diffusion-weighted MRI parameters were compared to SUV in the corresponding PET-CT examinationsand laboratory infection markers. All examined MRI and PET-CT parameters showed significant changes between basis and follow-up examinations. Median ADC values increased significantly (p < 0.001) in the follow up. SUVmax and the other MR parameter (contrast uptake, DWI-signal) declined significantly. We observed a strong negative correlation between ADC and SUVmax (rho: - 0.61; p < 0.001). In addition, we found an inverse correlation of ADC with the inflammation markers ESR (rho: - 0.64; p < 0.001) and CRP (rho: - 0.54; p = 0.001). MRI and PET-CT showed comparable results in the individual follow up of CP and compared to laboratory inflammation markers. Our data support MRI as first imaging modality due to absent radiation and the necessity of repetitive follow-up examinations in patients with CP.

Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.

Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions.

The Role of PET Scanning in the Evaluation of Patients With Kidney Disease.

Patients with underlying kidney disease are often required to undergo imaging for a variety of purposes including diagnosis and prognosis. A test that is being increasingly used with for this group of patients is the positron emission test (PET) scan. In addition, combining the nuclear medicine technique (PET) with computed tomography scan allows additional imaging advantages over either alone. These imaging modalities are commonly used for a number of extrarenal indications (ie, cancer, coronary artery disease, central nervous system disease, infectious diseases, and others). They have also been used for diagnosis of acute tubulointerstitial nephritis, evaluation and management of retroperitoneal fibrosis, identifying infection within kidney and liver cysts, and distinguishing complex kidney cysts from kidney cancer in patients with underlying CKD. We will review PET scan utility in patients with kidney disease.

Ultrasound imaging in the diagnosis of large vessel vasculitis.

Nowadays noninvasive vascular imaging has an important role in the diagnostic work-up of the large vessel vasculitides (LVV), most importantly giant cell arteritis (GCA) and Takayasu arteritis. Among the imaging modalities available, ultrasound (US) has several important advantages, including low costs, rapid and repetitive availability without exposure to radiation, and high spatial resolution for assessment of large and medium-sized arteries. Therefore, US can be regarded the first line imaging method in suspected LVV. In patients with suspected GCA, US can replace temporal artery biopsy in certain clinical scenarios, and the application of US early in the diagnostic work-up of suspected GCA in specialized fast track clinics has been suggested to reduce the rate of visual ischaemic complications and associated costs. In other LVV such as Takayasu arteritis and chronic periaortitis, the diagnostic accuracy in comparison to other noninvasive imaging methods has not been formally tested but can be considered to be excellent. However, quality of US is highly dependent on the operator's experience, and assessment of the thoracic aorta which is frequently involved in GCA and TA is limited. The role of US in the follow-up of LVV under treatment is unclear. In view of the promising data supporting its value on the one hand and several uncertainties and controversies on the other hand, the present review article provides a comprehensive overview on current evidence for the application of US in the diagnosis and follow up of LVV. Recent multicentre study results and emerging trends such as the application of compression sonography in the diagnosis of GCA and the use of contrast enhanced ultrasound in disease activity assessment in Takayasu arteritis are discussed.

Inflamatorna aneurizma abdominalne aorte i retroperitonealna fibroza

The inflammatory aneurysm of abdominal aorta (IAAA) accounts for 3-11% of all abdominal aortic aneurysms, and occurs predominantly in men. It has similar pathological mechanisms like idiopathic retroperitoneal fibrosis (IRF) and perianeurysmal retroperitoneal fibrosis (PRF), thus presenting the same non-specific systemic inflammatory disorder known as chronic periaortitis (CP). Recognition of CP early in its course is important in order to reduce morbidity due to complications, such as renal failure and mortality from aortic rupture. However, the initial symptoms of CP are non-specific, which makes early clinical diagnosis extremely difficult. Various studies have shown favorable outcomes following corticosteroid treatment in nearly 90% of patients. The positive effects of adding different immunosuppressants to corticoid therapy have established such "duo-therapy" as a durable treatment option. Also, cessation of smoking has a positive effect on disease course and it should be an integral part of every treatment regimen. Operative repair of the abdominal aortic aneurysm is considered to be the definitive treatment for IAAA. The effects of both open and endovascular interventions on the inflammatory component however remain unclear. Resolution of periaortic fibrotic tissue after surgery for IAAA is still debated. Some investigators have reported a complete resolution while the others have noted partial regression and even persistence of the inflammatory cuff. Current treatment strategies are based upon "the best available evidence'', mostly derived from clinical experience obtained by case series involving a small number of patients. Studies of a higher evidence level are very much needed to adjust our approach to such patients and to establish guidelines for treating this complex and serious disorder.