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'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.     

Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature

Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. Systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.     

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 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 recurrent carotid and aortic aneurysms: a potentially fatal disorder mimicking Marfan syndrome

Cogan's syndrome (CS) is a rare multisystemic disease characterized by vestibuloauditory dysfunction, inflammatory eye disease and vasculitis. Aortic aneurysms due to aortitis are under-recognized in CS, and are potentially fatal, with two of eight reported cases dying from aneurysm/arterial rupture. The presence of aneurysms was not recognized antemortem in both cases, highlighting the importance of early detection to prevent this potentially fatal outcome. We report a 17-year-old Chinese male with recurrent carotid and aortic aneurysms, bilateral sensorineural hearing loss, and recurrent scleritis who was initially thought to have Marfan syndrome. The diagnosis of CS was made 4.5 years after initial presentation, during which time recurrent aneurysms due to active aortitis (with aneurysmal rupture on one occasion) necessitated four surgical procedures. Treatment with methotrexate and corticosteroids resulted in no recurrence of aneurysms over 2 years of follow-up. This case illustrates (i) the challenges in diagnosing CS when various manifestations occur separately over a relatively long time period, (ii) that detection of aortic aneurysms in young patients, especially if recurrent, should prompt investigations for vasculitis/aortitis to prevent potentially fatal aneurysm rupture, and (iii) that methotrexate and high-dose corticosteroids may be effective in preventing development of further aneurysms in CS patients.     

Ear-nose-throat manifestations of autoimmune rheumatic diseases

Ear-nose-throat (ENT) manifestations of connective tissue disorders represent a diagnostic challenge for clinicians as they often constitute the initial sign of an otherwise asymptomatic autoimmune disease. Moreover, in patients with known autoimmune rheumatic diseases, ENT manifestations can be overlooked. Hearing disturbances may be seen in patients with systemic lupus erythematosus, Wegener's granulomatosis, relapsing polychondritis, polyarteritis nodosa, Cogan's syndrome, Sj?gren's syndrome, and less frequently in Churg-Strauss syndrome and Adamantiades-Beh?et's disease. Nose and paranasal sinuses are variably affected during the course of Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and sarcoidosis. Recurrent mucosal ulcerations are common in systemic lupus erythematosus and Adamantiades-Beh?et's disease. Xerostomia is a common feature of primary and secondary Sj?gren's syndrome; salivary gland enlargement may be also seen in these patients, as well as in patients with sarcoidosis. The cricoarytenoid joint can be involved during the course of rheumatoid arthritis, ankylosing spondylitis and gout; osteoarthritic changes have also been described. Motility disorders of the upper and/or the lower portions of the esophagus have been reported in patients with dermatomyositis/polymyositis, systemic sclerosis and systemic lupus erythematosus. Trigeminal nerve dysfunction may occur in patients with Sj?gren's syndrome, systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Peripheral facial nerve palsy has been described to complicate the course of Sj?gren's syndrome and sarcoidosis.     

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     

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.     

Successful full-term pregnancy in a woman with Cogan’s syndrome: a case report

Cogan’s syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. Major clinical features are interstitial keratitis and vestibuloauditory dysfunction. Associations between CS and systemic vasculitis as well as aortitis also exist. The present report is the first case in the literature of pregnancy associated with Cogan syndrome, which posed a therapeutic challenge. There was a relapse of the ocular symptoms only during the first trimester of pregnancy, but the pregnancy was otherwise uneventful. The relevant literature is reviewed both with regard to the relationship of CS to pregnancy and the therapeutic approach in this situation.     

Echocardiographic identification of cardiac abnormality in scleroderma and related disorders.

Although pathologic examination may readily disclose cardiac abnormality in patients with scleroderma, clinical identification of primary heart involvement can be difficult. In order to assess left ventricular systolic function, chamber size and wall thickness, and to determine whether pericardial effusion is present, echocardiograms were obtained in 11 patients with progressive systemic sclerosis (PSS) and in 13 patients with forms of scleroderma in which visceral involvement has been considered rare or absent: three with CREST syndrome, three with morphea, three with diffuse fasciitis with eosinophilia, and four with mixed connective tissue disease. Increased left ventricular wall thickness was noted in 13 of 23 (57 per cent) patients who could be evaluated, including six (46 per cent) from subgroups other than PSS. Left atrial dimension was increased in 12 patients (52 per cent) whereas the left ventricular end-diastolic dimension was increased in only three (13 per cent). Mitral valve closure velocity, an index of left ventricular compliance, was diminished in 10 (42 per cent) patients. However, left ventricular systolic contractile performance was normal in all. Pericardial effusion was detected in five patients (21 per cent), including one patient each with morphea, mixed connective tissue disease and diffuse fasciitis. Cardiac abnormalities were evident even in patients with PSS and no renal or severe pulmonary involvement. Thus, primary cardiac involvement, characterized by left ventricular wall thickening, decreased left ventricular compliance, left atrial enlargement and pericardial effusion, may be common in patients with scleroderma. These abnormalities occur in patients with PSS as well as in those with “nonsystemic” forms of scleroderma and are readily detected by echocardiography.     

Immune-mediated inner ear disease

Immune-mediated inner ear disease (IMIED) is a syndrome that includes the subacute onset of sensorineural hearing loss, often accompanied by vertigo and tinnitus. This constellation of symptoms may occur as a primary disorder in which no other organ involvement is evident, or it may complicate certain systemic conditions, including Wegener's granulomatosis, Cogan's syndrome, polyarteritis nodosa, and systemic lupus erythematosus. The precise disease mechanisms remain undefined, largely because of the difficulty obtaining relevant tissue specimens in untreated patients. However, if treated promptly with aggressive immunosuppression, the devastating sequelae of IMIED may be avoided. In this article, we review the pathophysiology, clinical evaluation, diagnostic testing, and therapy of IMIED.     

The cardiovascular manifestations of the hypereosinophilic syndrome. Prospective study of 26 patients, with review of the literature.

The major cause of morbidity and mortality in patients with the hypereosinophilic syndrome is cardiac dysfunction. A review of 65 cases from the literature (historic series) revealed the following cardiovascular manifestations to be most common: dyspnea (60 per cent), signs of congestive heart failure (75 per cent), murmur of mitral regurgitation (49 per cent), cardiomegaly (37 per cent), T wave inversions on electrocardiogram (37 per cent) and pathologic findings of endocardial fibrosis, myocardial inflammation and mural thrombus formation (57 per cent). We have prospectively followed 26 patients with the hypereosinophilic syndrome for up to nine years (average follow-up prospectively was 3.3 years, retrospectively 5.7 years). Common cardiac findings in our 26 patients were dyspnea (42 per cent), chest pain (27 per cent), signs of congestive heart failure (38 per cent), murmur of mitral regurgitation (42 per cent), cardiomegaly (35 per cent) and T wave inversions (35 per cent). Thus, these patients demonstrated cardiovascular manifestations similar to those in the historic series, although the literature review showed a higher incidence of overt congestive heart failure.Of 22 patients having echocardiograms, 55 per cent demonstrated some clinical, roentgenographic or electrocardiographic evidence of cardiac involvement, but 82 per cent had echocardiographic abnormalities. This suggests that the echocardiogram is a sensitive and perhaps early indicator of cardiac involvement in this disease. Common echocardiographic findings included increased left ventricular wall thickness (68 per cent), left ventricular mass (73 per cent) and left atrial size (37 per cent). Prospective echocardiographic follow-up of 18 patients (for up to four and a half years) revealed that seven of eight untreated or inadequately treated patients had increases in left ventricular wall thickness, whereas all 10 adequately treated patients had decreases (eight of 10) or no change (two of 10) in left ventricular wall thickness. This suggests that adequate antihypereosinophilic therapy (with prednisone and/or hydroxyurea) may stabilize and, in some cases, reverse the cardiac manifestations of the hypereosinophilic syndrome.In previous studies, congestive heart failure due to eosinophilic cardiomyopathy has been reported to be very resistant to therapy. In our patients with congestive heart failure, treatment has been almost invariably effective when digitalis and diuretics were combined with adequate antihypereosinophilic therapy.     

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.     

Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome

Cogan's syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be difficult if the various manifestations occur separately, but early recognition is important because prompt treatment may prevent deafness. Two cases are presented here illustrating the features of this disease, and providing histological evidence of systemic vasculitis in both.     

Late onset systemic lupus erythematosus.

This study describes the clinical and laboratory features and the natural history of 31 patients with late onset (in the sixth decade or later) systemic lupus erythematosus (SLE). Patients with late onset SLE constitute a distinct subset of the general lupus population that accounts for approximately 12 per cent of the cases. Advanced age modifies the expression of SLE in terms of clinical presentation (pleuritis and/or pericarditis are the most common presenting manifestations) and pattern of organ involvement (pulmonary abnormalities are more common, whereas lymphadenopathy, Raynaud's phenomenon, neuropsychiatric disease, alopecia and skin rash are less common). Because SLE is not usually considered to be a disease that affects the elderly, and because the pattern of SLE in the older age group may differ substantially from the seen in younger patients, there is often a delay in diagnosis (median of 10 months, with a delay of over one year in 32 per cent of patients). In light of the high incidence of steroid complications in older patients (40 per cent in our series), and because these patients with SLE have a relatively good prognosis (five year survival of 92.3 per cent; nine year survival of 83.1 per cent), therapy should be more conservative in late onset SLE.     

Avascular necrosis in systemic lupus erythematosus. Silent symmetric osteonecroses.

The symptomatic joint disease secondary to avascular necrosis of bone (ANB) in 31 of 375 (8 per cent) patients with systemic lupus erythematosus (SLE) was characterized by polyarticular (90 per cent) and symmetric (83 per cent) involvement. Progressive deterioration requiring orthopedic intervention occurred in 11 (35 per cent) patients. No distinguishing clinical or treatment differences were found comparing patients with symptomatic ANB and selected cortico-steroid-treated, asymptomatic, control subjects with SLE. Complete roentgenographic surveys revealed symmetric, polyarticular abnormalities of subchondral and cortical bone consistent with ANB in eight of 31 (26 per cent) of the control subjects. A positive correlation was found between roentgenographic ANB and disease treatment with high doses of corticosteroids. The study suggests that ANB may be present in excess of 30 per cent of the patients with SLE and is most often subclinical, asymptomatic and nonprogressive.     

Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature

Typical Cogan syndrome (CS) is a disease of young adults consisting of flares of interstitial keratitis (IK) and sudden onset of Ménière-like attacks. The prognosis of typical CS is excellent with life-threatening aortic insufficiency (AI) developing in only 10% of reported cases. Serious systemic necrotizing vasculitis (SNV) rarely, if ever, complicates typical CS. Atypical S (vestibuloauditory dysfunction with types of inflammatory eye disease other than IK) frequently overlaps other defined rheumatologic syndromes, is associated with vasculitis in 21% of cases, and carries a less favorable prognosis than typical CS. Topical ocular corticosteroids can usually control IK, and a short trial of systemic corticosteroids is warranted as soon as possible after the onset of hearing loss. SNV should be treated initially with prednisone and may also require the administration of cytotoxic agents, while aortitis and AI can be controlled with the administration of prednisone and surgical replacement of the aortic valve.     

The effect of heroin and multiple drug abuse on the electrocardiogram

The electrocardiograms (ECG) of 75 asymptomatic individuals admitted to a methadone treatment program were analyzed to determine the effect of street heroin and other drugs of abuse. All patients were free of cardiac, renal, or pulmonary disease and had no laboratory abnormalities. Two groups of patients were studied. In Group I there were 34 patients on heroin only which was taken within 24 hours of the ECG. Abnormalities were noted in 55 per cent; QT_c prolongation was found in 19 per cent, and significant bradyarrhythmias were found in 19 per cent. In Group II there were 41 patients on methadone with multiple drug abuse; changes were found in 66 per cent. QT_c prolongation was noted in 34 per cent, prominent U waves in 32 per cent, and bradyarrhythmias were found in 32 per cent.It is now a well-known fact that the sudden death syndrome in addicts may not be a pharmacologic or dosage-related phenomenon. The existence of conduction, depolarization, and repolarization abnormalities, as well as bradyarrhythmias in a significant number of drug-dependent individuals may play a role in the production and facilitation of lethal arrhythmias and may be the mechanism of the acute fatal reaction.     

Fever in systemic lupus erythematosus.

The frequency, causes, clinical and laboratory features, and outcome of febrile episodes in 160 hospitalized patients with systemic lupus erythematosus were reviewed. Eighty-three febrile episodes were identified in 63 patients and were ascribed to active lupus erythematosus alone (60 per cent), infections (23 per cent) and miscellaneous causes (17 per cent). Bacteremia was present in nine of the 19 infectious episodes and resulted in a fatal outcome in a third of the patients. Leukocytosis, neutrophilia, shaking chills and normal levels of anti-DNA antibodies were associated with infection in febrile patients with lupus erythematosus.