[Syncope, pain in the large joints, and painful swelling of the right eye in a 51-year-old patient : Pitfalls in the diagnosis and treatment of a rare disease]. / Synkope, Schmerzen der großen Gelenke und schmerzhafte Rötung des rechten Auges bei einer 51-jährigen Patientin : Fallstricke in Diagnostik und Therapie einer seltenen Erkrankung.

In the present case we report on a 51-year-old patient diagnosed with Cogan syndrome. This vasculitis of variable vessel size is a rare disease that poses a major challenge for the correct diagnostics and therapy. In the classic setting, it comprises a triad of non-syphilitic interstitial keratitis as well as hearing loss with vestibular dysfunction. A vascultis-related aortitis, an uncertain, more likely degenerative structure in combination with strongly elevated inflammation parameters was misinterpreted as infective endocarditis for a long time and treated with anti-infective medications. After diagnosis the patient recovered following treatment with high-dose steroids and in the further course cyclophosphamide and tumor necrosis factor­α blockers.

Infectious aortitis with multiple mycotic aneurysms caused by Streptococcus agalactiae.

We report a case of a 66-year-old man with multiple thoracoabdominal mycotic aortic aneurysms caused by Streptococcus agalactiae (S agalactiae). The infectious aortitis (IA) was diagnosed by transesophageal echocardiography and computed tomography and confirmed by positive blood cultures. The patient was treated with antibiotics, but, after worsening of the aortitis, a successful surgical procedure was performed. A review of the literature is presented together with a series of 7 other cases of IA caused by S agalactiae.

Intramural aortic abscess mimicking chronic aortic dissection.

A 77-year-old patient was referred for progressive fatigue and dyspnea on exertion. Preoperative imaging evaluations including transthoracic echocardiography and computed tomography were suggestive of a chronic ascending aortic dissection with an intramural hematoma. Intraoperatively, the intramural structure was identified as an abscess cavity.

Intimal angiosarcoma causing abdominal aortic rupture.

Intimal angiosarcomas are rare and difficult to diagnose preoperatively. Complete surgical resection is essential, but long-term survival is unlikely. We report a patient who presented with a contained ruptured infrarenal aorta with clinical and radiologic findings suggestive of infectious aortitis. Surgical resection, regional debridement, and reconstruction were completed using a cadaveric arterial homograft. However, pathologic evaluation revealed a high-grade intimal sarcoma.

Lupus Aortitis Successfully Treated with Moderate-dose Glucocorticoids: A Case Report and Review of the Literature.

Lupus aortitis is a rare and potentially life-threatening disorder. Previous studies have reported the utility of high-dose systemic glucocorticoids or surgery as the treatment, although there have been no related controlled trials. We herein report a 49-year-old woman with a 35-year history of systemic lupus erythematosus who was diagnosed with aortitis. Her symptoms and laboratory and imaging abnormalities rapidly resolved upon the administration of moderate-dose glucocorticoids. We subsequently performed a literature review of similar cases to identify the appropriate treatment and discuss these cases. A study of further cases will be needed to identify the characteristics of patients who would benefit from moderate-dose glucocorticoid therapy.

Aortitis which developed after the administration of granulocyte-colony stimulating factor.

Drug-induced aortitis is rare; thus, the diagnosis of drug-induced aortitis could be delayed unless clinicians are aware of the disease entity. Herein, we describe the case of a 66-year-old woman who developed aortitis after administration of granulocyte-colony stimulating factor (G-CSF) during chemotherapy for her breast cancer. Thickening of the aortic wall was clearly detected by computed tomography (CT) and magnetic resonance imaging. After excluding the other possible aetiologies, an association between G-CSF and the development of aortitis was highly suspected. Corticosteroid treatment rapidly regressed the aortitis, as confirmed by follow-up CT examination. G-CSF analog is generally well tolerated; however, there are limited case reports of G-CSF-associated aortitis, suggesting the causative effect of G-CSF in the development of aortitis. Currently, G-CSF-associated aortitis has received little attention among rheumatologists. As the delayed diagnosis results in irreversible changes in the aorta, not only oncologists but also rheumatologists should be aware of this unrecognized disease entity, G-CSF-associated aortitis.

Myocarditis and carotidynia caused by Granulocyte-Colony stimulating factor administration.

A 59 year-old woman was treated with adjuvant chemotherapy for triple negative breast cancer (TNBC) stage IB. She received pegfilgrastrim as secondary prophylaxis of neutropenia. After administration of pegfilgrastrim on day 11, she was hospitalised because of carotidynia and myocarditis that improved with antibiotics and steroids as an infection was suspected. Once she was recovered, another cycle of chemotherapy with pegfilgrastrim was administrated. At this time, the patient presented to our hospital with fever, odynophagia and chest pain, with diagnosis of myocarditis coupled with cardiogenic shock. She received antibiotics and steroids, advanced life support and also a pericardial window was done, with recovery of her condition. After a complete evaluation and exclusion of other possible aetiologies, we concluded that pegfilgrastrim was responsible for inducing carotidynia and myocarditis. Few cases have been published about Granulocyte-Colony stimulating factor (G-CSF) induced carotidynia and aortitis. However, this is the first reported case about G-CSF induced myocarditis and carotidynia.

Saprochaete capitata aortitis in an immunocomopetent patient after myocardial revascularization.

Saprochaete species infection is a rare fungal disease reported so far only in immunocompromised patients. We describe the first case of aortitis caused by Saprochaete capitata, presenting as ascending aorta aneurysm, with secondary endophthalmitis in an immunocompetent patient. Infection by Saprochaete capitata is potentially fatal, with a mortality ranging from 50% to 90% of cases. In the present case aortic aneurysm caused by Saprochaete capitata aortitis was successfully treated by the combination of accurate diagnosis with surgical and specific antifungal therapy.

Tubercular aortitis presenting as primary aortoenteric fistula: Report of an uncommon case.

Tubercular aortitis presenting as primary aortoenteric fistula (AEF) is a rare entity. We present a 78-year-old male who presented with upper gastrointestinal bleed and also had abdominal pain and pulsating abdominal mass and on evaluation was found to have tubercular AEF which was successfully repaired with surgery and the patient recovered with antitubercular therapy along with the surgery. This case highlights the importance of high index of suspicion with early institution of surgical repair along with antitubercular therapy for tubercular AEF with good results.

Non-aneurysmal infectious aortitis: a case report.

Infectious aortitis is a rare clinical entity that is most often associated with an abdominal aortic aneurysm. There have been very few documented cases of aortitis in the setting of a normal-sized caliber aorta. We present a 67-year-old patient who presented to our Emergency Department with a history of recent fevers, fatigue, and abdominal pain. Based on an abdominal computed tomography scan, the patient was initially thought to have ruptured an abdominal aortic aneurysm of infectious etiology with extension into the left psoas muscle. Explorative laparotomy, however, revealed an infected abdominal aorta secondary to a retroperitoneal abscess with no evidence of aneurysm or pseudoaneurysm. The pathophysiology of the disease suggests that infectious aortitis and mycotic aneurysm represent extremes along a spectrum of the same disease. Establishing a diagnosis of aortic infection before the formation of an aneurysm or rupture is very difficult, but essential in preventing the devastating complications.

Refractory PMR with aortitis: life-saving treatment with anti-IL6 monoclonal antibody (tocilizumab) and surgical reconstruction of the ascending aorta.

Aortitis is uncommon but well described in patients with polymyalgia rheumatica (PMR). While glucocorticoid remains the mainstay therapy for large-vessel vasculitis, there have been cases where tocilizumab therapy led to clinical and serological improvement in patients with relapsing or refractory disease. We report a case of life-threatening PMR with aortitis in the absence of manifestations related to giant cell arteritis, which, having failed to respond to corticosteroid therapy, was successfully treated with tocilizumab and emergency reconstruction of the ascending aorta. This case adds to the literature supporting the potential value of interleukin-6 inhibition in rare rheumatological conditions such as inflammatory aortitis.

Septic rupture of an atherosclerotic plaque of the ascending aorta.

Infectious aortitis has become a rare disease thanks to the widespread use of antibiotics. We report the case of a patient who, 15 days after initiation of antibiotics for bacteraemia due to methicillin-resistant Staphylococcus aureus (MRSA), developed acute chest pain followed by haemodynamic instability. A tamponade due to a rupture into the pericardium of the ascending aorta at the site of an atherosclerotic plaque was diagnosed by an emergent chest contrasted computed tomography (CT). Intraoperatively, the septic nature of the rupture was suspected. All aortic atherosclerotic plaque samples grew MRSA. Postoperatively, the patient had an uneventful recovery after 12 weeks of antibiotic therapy. Transoesophageal echocardiography and chest CT were normal at 3 months after cessation of antibiotics. This case report permits the review of some characteristics of this disease, its physiopathology as well as the therapeutic implications.

Infectious thoracic aortitis: a literature review.

Infectious thoracic aortitis (IA) remains a rare disease, especially after the appearance of antibiotics. However, if left untreated it is always lethal. It usually affects patients with atherosclerotic aortic disease and/or infective endocarditis. Mycotic aneurysm is the most common form of presentation, although a few reports of nonaneurysmal infectious thoracic aortitis have also been described. Various microorganisms have been associated with infectious thoracic aortitis, most commonly Staphylococcal, Enterococcus, Streptococcus, and Salmonella species. It is extremely important to establish an early diagnosis of IA, because this condition is potentially life-threatening. However, diagnosis is frequently delayed since clinical manifestations are usually nonspecific. Antibiotherapy in combination with complete surgical excision of the infected aorta is the best choice of treatment.

Atypical Cogan's syndrome with aortitis.

Cogan's syndrome is characterized by non syphilitic interstitial keratitis and Meniere's syndrome-like audiovestibular function disorder, as well as various systemic manifestations, including fever, arthralgia, vasculitis, and aortitis. We report the case of atypical Cogan's syndrome with aortitis; the patient's symptoms caused by active inflammation, including inflammatory ocular manifestations, audiovestibular symptoms, and aortitis, were improved by early administration of corticosteroids, however, her reduced visual acuity was not improved because of post-inflammatory change in her left eye.