Chronic Periaortitis – Review and Presentations

Chronic periaortitis is a rare inflammatory condition predominantly affecting the abdominal segment of the aorta. This can present as IgG4 related inflammatory disease, idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis andinflammatory abdominal aortic aneurysm (IAAA). Aortitis can also be a manifestation of a number of rheumatological large vessel vasculitides such as Takayasu arteritis and giant cell arteritis (GCA). We present three interesting cases of chronic periaortitis and a literature review. The first case showsa classic picture ofIgG4 periaortitis. The second case illustrates periaortitis with retroperitoneal fibrosis, ureteric involvement and hydronephrosis, following abdominal aortic aneurysmal stenting. The final case presents as widespread periaortitis due to Takayasu's disease involving the entire aorta including the arch and root of the subclavian artery

A Case of IgG4-Related Disease with Pachymeningitis and Periaortitis / 대한내과학회지

Immunoglobulin G4 (IgG4)-related disease (RD) is an immune-mediated, systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. IgG4-RD has become recognized with increasing frequency since the turn of the century and may affect almost any organ. IgG4-RD also involves the meninges but, to the best of our knowledge, no case of IgG4-related intracranial pachymeningitis with periaortitis has been reported in Korea to date. Here, we report on a 65 year-old male with IgG4-RD involving the meninges and aorta.

A Case of Infective Thoracoabdominal Aortic Aneurysm with Rapid Expansion during Steroid Therapy for Retroperitoneal Fibrosis / 日本心臓血管外科学会雑誌

A 66-year-old man who suffered from intermittent abdominal and back pain underwent medical examinations at our hospital. A high value of leukocyte, inflammatory reaction and IgG4 was detected, and computed tomography demonstrated that there was thickened soft tissue around the abdominal aorta which extended to the superior mesenteric artery and the renal arteries. He was given a diagnosis of retroperitoneal fibrosis, and prednisolone (PSL) was administered. Although the decrease in thickness of the soft tissue around the aorta was seen, the enlargement of the aorta mainly near the orifice of the celiac artery was shown. We were consulted on this thoracoabdominal aortic aneurysm (Crawford type IV) at this time, and considered that this aneurismal change had occurred secondary to chronic periaortitis. In a few weeks, the rapid expansion of this aneurysm was occurred, so we planned early surgical treatment after tapering of PSL. He underwent graft replacement of thoracoabdominal aorta with rifampicin-bonded graft, because the infection could not be denied as a cause of this aneurysmal change. Although <i>Streptococcus pneumoniae </i>was detected in the specimens from the periaortic tissue, false lumen and aortic wall in the culture test, he had a good post operative course with prolonged antibiotic therapy.

Immunoglobulin G4 Non-Related Sclerosing Disease with Intracardiac Mass Mimicking Mitral Stenosis: Case Report

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.

A Case of Periaortitis Diagnosed with Abdomen CT in a Patient with Malignant Fibrous Histiocytosis

Periaortitis is a very rare disease, characterized by a fibro-inflammatory tissue which develops around abdominal aorta and iliac arteries, and spreads into the structures of retroperitoneum. Computed tomography (CT) or magnetic resonance imaging (MRI) is the modality of choice for the diagnosis. Here, we report a case of periaortitis diagnosed with abdomen CT in patient with malignant fibrous histiocytoma.

A Case of Chronic Periaortitis with Retroperitoneal Fibrosis

A 73-year-old man with a history of hypertension and ascending aortic dissection was hospitalized for aggravated abdominal pain and general ache for 3 months. Follow-up CT showed aggravated abdominal aortic hematoma with aneurysm, atherosclerotic periaortitis and bilateral hydronephrosis. An initial laboratory finding showed elevated levels of inflammatory markers and renal dysfunction. Positron emission tomography-CT showed an increased standardized uptake values level in the aortic arch, descending thoracic aorta, major branch, abdominal aorta, and common iliac artery. For bilateral hydronephrosis, a double J catheter insertion was performed. Tissue specimens obtained from previous surgery on the aorta indicated the infiltration of lympho-plasma cells without granuloma formation in the aortic wall. After a combined therapy of high dose steroid therapy with azathioprine, the patient's initial complaints of abdominal pain, weakness and azotemia improved. This case was diagnosed as chronic periaortitis based on aortic inflammation at biopsy, which was complicated with retroperitoneal fibrosis and ureteric obstruction.

A Case of Wegener's Granulomatosis with Periaortitis and Pachymeningitis / 대한류마티스학회지

Wegener's grandulomatosis (WG) is a systemic small-vessel vasculitis characterized by necrotizing granulomatous lesions and anti-neutrophil cytoplasmic antibodies (ANCA). WG frequently affects the upper and lower respiratory tracts and kidney but involvement of the pachymeninges or large vessels has rarely been reported. We report the first case of WG with periaortitis and pachymeningitis in Korea. A 57-year-old female complained of a stabbing headache, paresthesia below the thoracic 4th nerve level, and lower extremity weakness. During her course, she developed otitis media with sensorineural hearing loss, nodular scleritis, and microhematuria. A neuroimaging study showed an intradural mass in the area from the cervical 7th spine to the thoracic 5th spine, left cerebral convexity, and the left petrous area. A chest computed tomography scan revealed an enhanced and thickened aortic arch. A dural biopsy showed necrotizing granulomatous inflammation, and anti-MPO ANCA was positive. Under a diagnosis of WG, she received high-dose glucocorticoid and oral cyclophosphamide with improvement.

Fibrosis retroperitoneal idiopática: un enigma médico / Retroperitoneal fibrosis: a medical enigma

La fibrosis retroperitoneal es una enfermedad rara de causa desconocida. Fue descrita por primera vez en la literatura inglesa en 1948 por Ormond. Sin embargo, la primera descripción conocida de esta enfermedad fue realizada por el urólogo francés Albarran en 1905. Se caracteriza por una inflamación crónica e inespecífica del retroperitoneo, la cual puede atrapar y obstruir las estructuras que allí se encuentran, particularmente a los uréteres. Es frecuente que la enfermedad se detecte en etapas avanzadas, cuando se manifiesta por falla renal crónica. Los avances en las técnicas imaginológicas y el mayor conocimiento de la entidad han permitido que el diagnóstico se realice con mayor frecuencia en etapas más tempranas, cuando los pacientes aún no se encuentran obstruidos. Como resultado de las observaciones que muestran que los aneurismas inflamatorios de la aorta abdominal difieren de la enfermedad de Ormond sólo en el diámetro de la aorta inflamada, se ha sugerido que ambos síndromes representan variaciones de la misma patología, la cual se ha llamado "periaortitis crónica".

Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond, in 1948. However, the first known description of this disease was done by the French urologist Albarran, around 1905. It is characterized by chronic nonspecific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the urethers. This disease frequently is detected only after severe renal failure is present. However, advances in imaging techniques and increased awareness of the disease have resulted in an earlier diagnosis in unobstructed patients. As a result of the observations that showed that the only difference between an inflammatory aneurysm of the abdominal aorta and Ormond's disease is the diameter of the vessel, it had been suggested that both syndromes represent variations of the same pathology, which has been named "chronic periaortitis".