Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases.

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.

Tocilizumab in refractory aortitis: study on 16 patients and literature review.

OBJECTIVES: Non-infectious aortitis is often refractory to standard immunosuppressive therapy. Since IL-6 has been implicated in the pathogenesis of aortitis, we assessed the efficacy of the anti-IL6 receptor monoconal antibody tocilizumab (TCZ) in a series of patients with refractory non-infectious aortitis. METHODS: Review of 16 patients (14 women/2 men) with refractory aortitis diagnosed by imaging (CT angiography, MR angiography, and/or PET) that were treated with TCZ. RESULTS: The mean age±SD was 51.4±20.1 years. The underlying conditions were: Takayasu arteritis (TakA) (n=7 cases), giant cell arteritis (GCA) (n=7), relapsing polychondritis (RP) (n=1), and aortitis associated with retroperitoneal fibrosis (n=1). TCZ was the first biologic drug used in all patients with GCA and in the patient with aortitis associated with retroperitoneal fibrosis but in only 2 of 7 TakA patients. In the remaining cases anti-TNF inhibitors were prescribed before TCZ (standard dose was 8 mg/kg/iv/4 weeks). After a mean±SD follow-up of 11.8±6.6 months most patients experienced clinical improvement, showing reduction of erythrocyte sedimentation rate from 43±36 mm/1st h to 5±4 mm/1st h at last visit. At TCZ onset, 25% of patients had fever and 19% polymyalgia rheumatica. These manifestations disappeared after 3 months of TCZ therapy. A corticosteroid sparing effect was also achieved (from 27.3±17.6 mg/day of prednisone at TCZ onset to 4.2±3.8 mg/day at last visit). TCZ had to be discontinued in a patient because of severe neutropenia. CONCLUSIONS: TCZ appears to be effective and relatively safe in patients with inflammatory aortitis refractory to corticosteroids or to other biologic immunosuppressive drugs.

Patología aórtica no urgente: diagnóstico clínico-radiológico de la aortitis / Nonurgent aortic disease: clinical-radiological diagnosis of aortitis

Aortitis es un término patológico que designa la inflamación de la pared aórtica, independientemente de su causa. Su presentación clínica es inespecífica y variable, con síntomas como dolor abdominal, fiebre y pérdida de peso. También pueden estar elevados los reactantes de fase aguda. Un amplio espectro de entidades puede ocasionar aortitis, desde procesos infecciosos hasta enfermedades autoinmunes (de las que las más frecuentes son la arteritis de Takayasu y la arteritis de células gigantes), cuyo pronóstico y tratamiento son muy variables. Son varias las técnicas de imagen que permiten evaluar tanto la luz como la pared vascular (como la tomografía computarizada multidetector, la resonancia magnética, la angiografía o la PET-TC). Esta revisión se centra en las enfermedades más frecuentes que provocan aortitis y en los hallazgos clínicos y radiológicos más relevantes que ayudan a diagnosticar y tratar adecuadamente esta entidad (AU)

Aortitis is a pathological term designating inflammation of the aortic wall, regardless of its cause. The clinical presentation of aortitis is nonspecific and variable. Symptoms include abdominal pain, fever, and weight loss; acute phase reactants may also be elevated. Aortitis can be caused by a wide spectrum of entities, including from infectious processes to autoimmune diseases (Takayasu arteritis and giant cell arteritis are among the most common of these causing aortitis), and the prognosis and treatment of these entities vary widely. Various imaging techniques can be used to evaluate the lumen and wall of the aorta (such as multidetector computed tomography, magnetic resonance imaging, angiography, or PET-CT). This review focuses on the most common diseases that cause aortitis and on the clinical and radiological findings that are most useful for diagnosing and treating this condition appropriately (AU)

IgG4-related inflammatory abdominal aortic aneurysm.

PURPOSE OF REVIEW: IgG4-related systemic disease is a recently proposed entity characterized by high serum IgG4 concentrations, sclerosing inflammation containing numerous IgG4-positive plasmacytes, dramatic responsiveness to steroid therapy, and occurrence of multiple organs. This review described that some cases of inflammatory abdominal aortic aneurysm (IAAA) have similar clinicopathological features that are now considered to represent aortic lesions of IgG4-related systemic disease under the concept of IgG4-related IAAA. RECENT FINDINGS: IgG4-related IAAA is characterized by high serum IgG4 and immunoglobulin E levels, high titers of antinuclear antibodies, and high prevalence of allergic disorders such as bronchial asthma. The patients show a risk of developing IgG4-related systemic diseases in other organs during their life. Histologically, sclerosing inflammation containing numerous IgG4-positive plasmacytes is observed predominantly in the adventitia. Similar lesions have also been reported in the thoracic aorta and large arteries. SUMMARY: Recognition of the fact that IgG4-related systemic disease could involve the vascular lesions offers potential new management of those. However, only 3 years have passed since the first report of IgG4-related IAAA. Further studies are necessary to elucidate other vascular lesions associated with IgG4-related systemic disease, the usefulness of steroid therapy for the management of IgG4-IAAA, and underlying pathological and immunological characteristics.

Incidence, etiology, histologic findings, and course of thoracic inflammatory aortopathies.

BACKGROUND: The aims of this study were to detect the incidence of thoracic histologically proven aortitis in a large series of 788 patients operated on for thoracic aortic disease, to describe the surgical and histologic features of inflammatory thoracic aortopathies, and to evaluate the frequency of postsurgical complications and mortality. METHODS: Thirty-nine patients (4.9%) were affected by aortitis (mean age, 72.6 +/- 9.6). There were 24 women (61.5%). Thirty-four (87.2%) were operated on because of aneurysms and 5 because of dissection. In all cases the diagnosis of aortitis was incidental and was made on the basis of histopathologic findings. RESULTS: Histologically, there were 30 cases of giant cell aortitis (76.9%), 3 inflammatory aneurysms (7.7%), 2 cases of aspecific lymphoplasmacellular aortitis (5.1%), 1 of Takayasu aortitis, 1 of systemic erythematosus lupus-associated aortitis, and 1 of Behçet's disease-associated aortitis. The only case of infectious aortitis was a syphilitic aortitis. In 79.5% of cases, inflammatory infiltrates were moderate to severe in degree; the most widespread inflammation was seen in Takayasu aortitis, systemic erythematosus lupus-associated aortitis, and in Behçet's disease. The overall in-hospital mortality was 10.3% (4 of 39 patients). Neurologic complications occurred in 4 patients (10.3%). CONCLUSIONS: During surgery of the thoracic aorta, an inflammatory etiology of aneurysms is found in almost 5% of cases. The inflammatory process is in a histologically advanced phase, often with systemic development. Surgery can be associated with high morbidity and mortality.

Aortitis and ascending aortic aneurysm: description of 52 cases and proposal of a histologic classification.

Noninfectious aortitis typically involves the ascending aorta and causes aneurysms that result in aortic root repair. Aortitis is clinically categorized into groups that include Takayasu disease, giant cell aortitis, and isolated aortitis. We present a histopathologic classification of 52 patients with aortitis, without reference to clinical findings, which are often unknown to the diagnostic pathologist. The largest group (43 patients) was designated necrotizing aortitis (NA), characterized by zonal medial laminar necrosis, rimmed by giant cells. Healed areas were common and were characterized by extracellular accumulation of proteoglycans imparting the appearance of medial degeneration. NA had a bimodal age distribution with a separation at age 65 (adult NA versus elderly NA). Adult NA (24 patients; 50% female; age range, 24-60) was generally isolated, but 2 patients had associated autoimmune disease (Crohn disease and lupus erythematosus, respectively). Elderly NA (19 patients; 94% female; age range, 68-80) was likewise usually isolated, but 1 patient had temporal giant cell arteritis and 1 seronegative arthritis. Subsequent complete rheumatologic workup on 17 patients with NA was negative. Adult NA differed significantly from elderly NA (fewer women, P = .002; greater adventitial scarring, P = .007). The second group of aortitis was designated non-NA (NNA), characterized by the absence of necrosis, with diffuse medial inflammation. The NNA group was composed of 3 men and 6 women, all older than 65 years (mean, 72 +/- 6 years). Four had a history of temporal arteritis. NNA patients differed from elderly NA (more frequent temporal arteritis, P = .03; less medial destruction and proteoglycan deposits, P < .01; increased medial T-lymphocytes, P = .05; and more frequent dissection, P = .002). We conclude that NA is usually isolated, has distinct histologic features based on age less than or more than 65 years, and is clinicopathologically distinct from NNA. NNA is less often isolated and best classified as giant cell aortitis. Adult NA has histologic features classically associated with Takayasu disease but is limited primarily to the ascending aorta and has no sex predominance.

[Nonspecific aortoarteritis (classification and surgical treatment)]. / Nespetsificheskii aortoarteriit (klassifikatsiia, khirurgicheskoe lechenie).

Three hundred cases of nonspecific aortoarteritis are reviewed. A new classification of the condition is proposed identifying 3 stages (acute inflammation, subacute recurrent inflammation and chronic condition), 3 types of morphologic changes (stenosing, deforming and aneurysmatic), 3 sites of lesion (aortic branches, the thoracoabdominal aorta and combined lesions) and 4 degrees of organ ischemia. Ten syndromes are distinguished in the clinical pattern, and their relative incidences are specified. A total of 253 operations, including 218 reconstructive ones, were performed: 83 on aortic branches, 87 on thoracoabdominal aorta, 40 on the abdominal aorta, and 11 for aortic aneurysms. Reconstructive surgery of renal arteries was performed in 89 patients, and of visceral arteries, in 55. The need for anti-inflammatory treatment is emphasized. Surgical treatment produces better results, as compared to conservative treatment alone.