Diagnosis of aortitis]

Aortitis can be a component of a variety of diseases, such as Takayasu arteritis, giant cell arteritis, Beh?et's syndrome, Cogan's syndrome, spondylarthropathies, rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, Erdheim-Chester's disease and a variety of infectious processes like syphilis, Salmonella and others. The presentation is variable: aortic valve regurgitation, aneurysm, dissection, stenosis of the aorta or its initial branches. Sometimes systemic manifestations are at first presentation like fever or inflammatory syndrome. The differential diagnosis may be difficult in some situation like inflammatory aortic atherosclerotic aneurism, or retroperitoneal fibrosis. Some aortitis remain idiopathic. Corticosteroid and sometimes surgery are mostly required to avoid life-threatening complications.     

Typical and atypical Cogan's syndrome: 32 cases and review of the literature

OBJECTIVE: To report our experience on a multicentre series of 32 patients with either typical or atypical Cogan's syndrome, to combine our results with a detailed review of the literature, and to compare the clinical manifestations of typical and atypical Cogan's syndrome. METHODS: Patients were identified from a survey conducted with physicians affiliated to the French National Society for Internal Medicine, and were classified into typical or atypical Cogan's syndrome according to the Haynes criteria. Clinical data were collected in a standardized manner. A comprehensive literature review using the Medline database and the reference lists of identified articles was performed. RESULTS: Seventeen patients had typical Cogan's syndrome and 15 had atypical Cogan's syndrome. Apart from non-syphilitic interstitial keratitis, the ocular manifestations of patients with atypical Cogan's syndrome were mainly uveitis and episcleritis. All but one patient presented with Ménière-like syndrome, and at the end of follow-up 11 were deaf and 19 additional patients had developed a significant decrease in auditory acuity. Twenty-five patients (78%) developed systemic manifestations, including aortitis in four. Comparison of typical and atypical Cogan's syndrome showed that some systemic manifestations were more common in atypical Cogan's syndrome, but these differences may be explained by reporting bias in the literature. CONCLUSION: Differences regarding the associated systemic manifestations of typical and atypical Cogan's syndrome may reflect reporting bias in the literature. However, the diversity of the ocular and audiovestibular manifestations and the acceptable lengthy delay between the two types of involvement in atypical Cogan's syndrome should make one cautious before accepting this diagnosis as the diagnosis may mimic various other systemic diseases.     

Cardiovascular manifestations of Cogan syndrome. Apropos of a case]

The authors describe a case of Cogan's syndrome in a patient with ulcerative colitis complicated by several cardiovascular manifestations including bilateral coronary ostial stenosis, rapidly progressive aortic regurgitation and aneurysm of the thoracic aorta, thrombosis of the common iliac artery and pericardial symphysis. This rare form of inflammatory arteritis, the diagnosis of which is usually made on the finding of associated ocular and auditory involvement, is distinct from other types of angiitis by the predisposition to severe cardiovascular complications which influence the vital prognosis. The differential diagnosis with more common collagen diseases with cardiovascular complications is discussed.     

Atypical Cogan's syndrome mimicking encephalitis

Cogan's syndrome is a rare autoimmune multisystem disease. The main clinical features of typical Cogan's syndrome are vestibuloauditory dysfunction and interstitial keratitis. The authors present a case of atypical Cogan's syndrome with headache, fever, deafness, trigeminal neuralgia and electroencephalographic abnormality which mimicked viral encephalitis.     

Cogan syndrome or sarcoidosis?

INTRODUCTION: The Cogan's syndrome is characterized by the association of vestibulo-auditory dysfunction, non syphilitic interstitial keratitis or another significant inflammatory eye lesion. Some authors consider this disease as a vasculitis, because it is frequently associated with systemic manifestations. Based on Cogan's diagnostic criteria, Cogan's syndrome may be part of other systemic diseases, as polyarteritis nodosa or Wegener's granulomatosis. EXEGESIS: We report the case of a patient who presented with a Cogan's syndrome and developed further sarcoidosis. CONCLUSION: If Cogan's syndrome is characterized as systemic disease because of its association with aortitis or other vasculitis, on the other hand, clinical presentation may be part of many other systemic diseases.     

Cogan's syndrome with refractory abdominal aortitis and mesenteric vasculitis.

Cogan's syndrome is a rare multisystem disease characterized by ocular inflammation, vestibuloauditory dysfunction, and vasculitis. We report a 26-year-old Caucasian woman who died from Cogan's syndrome. Her case illustrates that patients with Cogan's syndrome can have abdominal aortitis and mesenteric vasculitis, and that the vasculitis can be refractory to methotrexate, cyclophosphamide, cyclosporine, and chlorambucil.     

Association of Crohn's disease and Cogan's syndrome

Summary We describe the case of a young woman with Cogan's syndrome and associated Crohn's disease. Systemic disease was manifested by oligoarthritis and valvular involvement. Crohn's disease has previously been described in two cases of Cogan's syndrome. We suggest therefore that Crohn's disease is pathogenetically related to Cogan's syndrome. For both, systemic involvement is characteristic, and immune-mediated mechanisms and vasculitis are thought to play a major pathogenetic role. Furthermore, both respond to treatment with corticosteroids.     

POSTERIOR SCLERITIS--AN UNUSUAL MANIFESTATION OF COGAN'S SYNDROME

Cogan's syndrome is characterized by a non-luetic interstitialkeratitis associated with vertigo, tinnitus and profound deafness.Evidence of a systemic vasculitis is found in up to 50% of patients.Atypical forms of Cogan's syndrome have been described in whichthe ocular inflammatory disease may be more severe. We describe a case of atypical Cogan's syndrome in associationwith bilateral posterior scleritis. Serial B-scan ultrasoundmeasurements of posterior scleral thickness were found to beuseful in assessing disease activity, in combination with clinicalfindings. Combination therapy with prednisolone and cyclosporincontrolled the ocular disease but the deafness was irreversible.The length of follow-up of this case highlights the frequentrelapses and difficult management problems which may be faced. This multisystem disease requires the close co-operation ofophthalmologist, physician and otorhinolaryngologist. Aggressivetherapeutic intervention with high-dose combined immunosuppressiveagents may be necessary to control severe ocular inflammatorydisease. KEY WORDS: Cogan's syndrome, Posterior scleritis, B-scan ultrasound, Immunosuppression     

Lack of response to corticosteroids and pulse cyclophosphamide therapy in Cogan's syndrome

Summary A 17-year-old girl with Cogan's syndrome is described. Total and irreversible hearing loss occurred which was unresponsive to corticosteroids and immunosuppressive therapy. The girl died a year later from subarachnoid haemorrhage. The lethal prognosis in Cogan's syndrome despite the available treatment is emphasized.     

Vascular involvement in Cogan's syndrome. A case report

Vascular manifestations of Cogan's syndrome are rarely reported. We report the case of a young woman followed for typical Cogan's disease. Serious vascular involvement was found only during work-up for arterial hypertension. This case highlights potentially asymptomatic nature of extensive vasculitis affecting large and medium-sized vessels in Cogan's disease. Careful screening is required to prevent life-threatening complications.     

Chlamydia pneumoniae infection as a trigger for a Cogan's syndrome

Cogan's syndrome is often preceded by upper respiratory tract symptoms. The only reported specific agent possibly involved in pathogenesis of the Cogan's syndrome was Chlamydia pneumoniae. Positive IgA, IgM and IgG antibodies against C. pneumoniae in our patient suggest possibility of Chlamydia's role as a trigger for the vasculitis.     

Cogan's syndrome: unsuccessful outcome with early combination therapy.

Interstitial keratitis and vestibuloauditory symptoms (vertigo and hearing loss) are the typical signs of Cogan's syndrome, a rare inflammatory vascular disease. Signs of vasculitis in many organ systems may appear, among which neurologic problems are sometime predominant. The efficacy of glucocorticoids on the ocular and systemic symptoms is established, but their effect on hearing loss is unknown. We describe a case of Cogan's syndrome with neurological involvement in which early treatment with combination therapy (prednisolone and cyclosporin) failed to bring ear inflammation under control.     

Cogan's syndrome as an extra-articular manifestation of rheumatoid arthritis

Summary We describe a patient with long-standing rheumatoid arthritis who developed rheumatoid vasculitis and Cogan's syndrome, to our knowledge the third such case to be reported.     

Cogan's syndrome: A new possible complication of antiphospholipid antibodies?

Summary A 55-year-old woman with a six-year history of Sjögren's syndrome (SS) and the positivity of IgG and IgM antiphospholipid antibodies (aPL) developed a sudden onset of sensorineural hearing loss associated with vertigo. This suggested the presence of an atypical Cogan's syndrome (CS), which might be a focal, neurological complication of aPL.     

Vasculitis and Cogan's syndrome

Cogan's syndrome is a rare syndrome manifested by nonsyphilitic interstitial keratitis and audiovestibular symptoms including hearing loss, tinnitus, and vertigo. Systemic manifestations may occur. Systemic vasculitis and aortic insufficiency secondary to aortitis and valvulitis are two of the most serious manifestations.     

Atypical Cogan's syndrome with cutaneous vasculitis: a case

The authors report the case of a 81 years-old-woman who had simultaneously atypical Cogan's syndrome, cutaneous vasculitis and diverticular sigmoiditis. The relations being between these three diseases are discussed.     

Cogan's syndrome: a systemic vasculitis.

Nonsyphilitic interstitial keratitis with vestibuloauditory dysfunction (Cogan's syndrome) is a rare clinical entity. We have reviewed 53 cases (including one of our own) of this disease. In 72 per cent of the affected patients there was an underlying systemic process, often a vasculitis. Ten per cent had fatal or near fatal aortic valvular disease, which has been shown to be amenable to surgical intervention. Other systemic manifestations have included congestive heart failure, gastrointestinal hemorrhage, adenopathy, splenomegaly, hypertension, musculoskeletal involvement and eosinophilia. The clinical course is extremely variable, ranging from months to over 15 years with a minimal five year survival of 28 per cent. Medical therapy with corticosteroids has been beneficial but has only limited effect on symptoms of vestibuloauditory dysfunction. Cogan's syndrome appears to be a manifestation of a systemic disorder which is often apparent only after long-term follow-up.     

Two surgically treated patients with severe symptoms of Takayasu arteritis

Takayasu arteritis, also known as the aortitis syndrome, is a panarteritis that affects the arcus aorta and its major branches, seen usually in young women. As Takayasu arteritis has a progressive nature, surgery is usually indicated only in complications of the arteritis. In this report we present two surgically treated cases with severe complications of Takayasu arteritis. We also review the syndrome.     

Cogan's syndrome in childhood

We present a case of Cogan's syndrome in childhood presenting with arthralgia, myalgia, anorexia, uveitis, aortic regurgitation, and intermittent fever and who responded well to corticosteroid therapy and successful valvular replacement. The extensive diagnostic methods and a review of the literature are covered and intended to familiarize pediatricians with this rare but treatable disorder.     

Abnormal collateral arterial systems in Takayasu's arteritis and Leriche's syndrome evaluated by whole body acquisition using multislice computed tomography

Takayasu's arteritis causes various arterial changes, including wall thickening, occlusion, aneurysm, and calcification. Similarly, Leriche's syndrome causes occlusion of the abdominal aorta or iliac arteries accompanied by ischemic symptoms of the distal lower extremities. Both diseases are potentially associated with the development of unexpected collateral arteries throughout the body. We evaluated whole body arteries in subjects with Takayasu's arteritis and Leriche's syndrome using 16-slice multislice CT and concluded that it was a useful tool to detect unexpected collateral arterial systems in Takayasu's arteritis or Leriche's syndrome in a non-invasive fashion.