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Diagnostic Difficulties of Cogan Syndrome

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Diagnostic Difficulties of Cogan Syndrome ical C lin as Masiak et al., J Clin Case Rep 2016, 6:1 C e f R o l e DOI: 10.4172/2165-7920.1000691 a p n o r r t u s o J Journal of Clinical Case Reports ISSN: 2165-7920 Case Report Open Access Diagnostic Difficulties of Cogan Syndrome Anna Masiak1*, Agnieszka Waśkowska2, Paweł Lipowski2, Żaneta Smoleńska1, Tomasz Przewoźny3 and Zbigniew Zdrojewski1 1Clinic of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland 2Clinic of Ophthalmology, Medical University of Gdańsk, Poland 3Clinic of Otolaryngology, Medical University of Gdańsk, Poland Abstract Cogan’s syndrome (CS) is a very rare disorder of unknown origin characterised by inflammatory eye disease and vestibuloauditory symptoms. According to the International Chapel Hill Consensus Conference Nomenclature of Vasculitides, CS is defined as “variable vessel vasculitis”. We present the case of a 44-year old male, primarily diagnosed and treated for large/medium vessel vasculitis probably due to indeterminate colitis, who finally developed CS. Difficulties in the diagnosis of this rare disease are also discussed. Keywords: Cogan syndrome; Exophtalmos; Inflammatory eye disease; Chronic diarrhea; Sudden hearing loss Introduction Cogan’s syndrome (CS) is a rare disorder characterised by inflammatory eye disease and vestibuloauditory symptoms. In about 30% of cases it can be associated with other systemic inflammatory manifestations such as fever, arthralgia, skin rash, gastrointestinal tract involvement and vasculitis [1]. Vasculitic manifestations include mainly arteritis affecting small, medium or large arteries and according to the International Chapel Hill Consensus Conference Nomenclature of Vasculitides, CS is defined as “variable vessel vasculitis” [1]. The onset of the disease is preceded in approximately 20% of cases by an infection, especially Chlamydia psittaci [2] and Chlamydia trachomatis [3]. Recent evidence strongly suggests that CS is an autoimmune disease that is mediated by a hypersensitive response to infectious agents associated with vasculitis [3-5]. We present the case of a 44-year old male, primarily diagnosed and treated for large/medium vessel vasculitis probably resulting from indeterminate colitis, who developed CS with inflammatory eye disease and vestibuloauditory symptoms. We Figure 1: Right corneal lesions in the slit-lamp examination. Circular scars in the discuss the diagnostic difficulties in this rare disease. cornea (asterix), corneal infiltrates (best seen on the background of the pupil and around it), superficially and deeply ingrown vessels (arrow). Case Report of the adipose tissue of the right orbit, a narrowing of both the left A 44-year old male, a pigeon-fancier, was admitted to the subclavian artery and the left internal carotid artery with thickening of hospital due to general weakness secondary to recurrent periods of the wall up to 3 mm. Gastroscopy revealed chronic gastritis (confirmed fever (maximum 38.5°C), chronic watery diarrhea accompanied by histopathology). Despite the absence of inflammatory lesions in the by severe headache and ophthalmopathy with decreased vision. All intestine in colonoscopy, the histopathological examination revealed the symptoms occurred several months before hospitalization and non-specific chronic colitis. The patient was negative for antinuclear gradually intensified. Physical examination revealed only bilateral antibody (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA). exophthalmos. Laboratory tests had shown leukocytosis (white blood Immunological tests revealed only the presence of anti-Saccharomyces cell count 17.9 109/l), thrombocytosis (platelet count 600 109/l), mild cerevisiae antibodies (ASCA) and immunological complexes (Table 1). anemia (hemoglobin level 11,6 g/dl), marked elevation of inflammatory Thyroid dysfunction was excluded as a reason for the ophthalmopathy markers (ESR 100 mm/h, CRP 10.9 mg/l) and an active urine (normal levels of thyroid hormones, no anti-TSH antibodies). Multiple sediment. Based on laboratory, serological and microbiological test, organ involvement, laboratory (leukocytosis, thrombocytosis, mild immunodeficiencies, Lyme disease, toxoplasmosis, the most common viral infections (hepatitis B and C, cytomegovirus and HIV), atypical respiratory (Mycoplasma pneumonia and Chlamydia pneumonia) and *Corresponding author: Anna Masiak, Department and Clinic of Internal Medicine, urogenital (Ureaplasma urealyticum) infections were excluded. The Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland, Tel: +48 58 349 25 12; Fax: +48 58 349 28 32; E-mail: [email protected] blood, urine and stool cultures were also negative. Ophthalmological examination revealed changes suggestive of viral or autoimmune Received December 09, 2015; Accepted January 04, 2016; Published January aetiology (Figure 1). Despite empiric antibiotic therapy (amoxicillin 09, 2016 with clavulanic acid, ciprofloxacin) there was no clinical improvement Citation: Masiak A, Waśkowska A, Lipowski P, Smoleńska Z, Przewoźny T, et and elevated leukocytosis, and inflammatory markers were still present al. (2016) Diagnostic Difficulties of Cogan Syndrome. J Clin Case Rep 6: 691. doi:10.4172/2165-7920.1000691 (Table 1). Diagnostic Computed Tomography (CT) of the head, neck, thorax and abdomen, magnetic resonance imaging (MRI) of the orbits, Copyright: © 2016 Masiak A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted echocardiography, carotid and thyroid Doppler ultrasound imaging were use, distribution, and reproduction in any medium, provided the original author and performed. Pathological findings included inflammatory infiltration source are credited. J Clin Case Rep Volume 6 • Issue 1 • 1000691 ISSN: 2165-7920 JCCR, an open access journal Citation: Masiak A, Waśkowska A, Lipowski P, Smoleńska Z, Przewoźny T, et al. (2016) Diagnostic Difficulties of Cogan Syndrome. J Clin Case Rep 6: 691. doi:10.4172/2165-7920.1000691 Page 2 of 5 anemia, marked elevation of inflammatory markers), imaging 20 mg, daily) and ciclosporine (150 mg twice a day) orally, and both (thickening of the arterial walls) and immunological (lack of ANCA, 0.1% dexamethasone and 0.05% ciclosporine topically in eye drops. the presence of antiendothelial cell antibodies), after exclusion of active Two twenty two months after the onset of the initial symptoms, even infections, led to the most probable diagnosis of the large/medium though continuous immunosuppression has been provided, the patient vessel vasculitis in the course of indeterminate colitis. A total dose of 2.5 suffered sudden left sensorineural hearing loss with vertigo, paralytic g (5 × 500 mg) of intravenous (iv.) methylprednisolone with subsequent right-beating horizontal-rotatory nystagmus (Figure 2a) and tinnitus oral steroid therapy (prednisone 1 mg per kg per day) in tapered doses with accompanying nausea and fever. In Romberg’s test he fell to the left, and azathioprine (2 mg per kg per day) were administered. Central indicating the free phase of nystagmus. Otolaryngological examination ulceration of the left cornea was initially treated with antibiotics revealed no abnormalities. Pure tone audiometry (PTA) showed left- in drops (levofloxacin and azithromycin) and then a solution of 0.1% sided sensorineural hearing loss (Figure 2b). Videonystagmography dexamethasone and 0.05% cyclosporine in eye drops was used to (VNG) revealed left canal paralysis. In addition to immunosuppressive reduce extant symptoms in both eyes. Regression of the exophthalmos, therapy, lidocaine, hydrocortisone, pentoxifylline and vitamins (B1 and relief of the diarrhea and fever, as well as general improvement were B6) were applied but with no hearing improvement. Despite treatment, achieved. The inflammatory bowel disease (IBD) was finally excluded by after two weeks the patient developed right-sided sensorineural gastroenterologists on the basis of magnetic resonance enterography and hearing loss and the left-sided hearing loss was intensified. There double-performed histopathological examination of the intestinal wall. was no hearing improvement after systemic steroids either (Figure During the following twenty months there were periods of 2c). Brainstem auditory evoked potentials (BAEPs) (Figure 2d) exacerbations and improvement in ophthalmic changes (exophthalmia, revealed no retrocochlear type of hearing deterioration. The result of a conjunctival chemosis) as well as in inflammatory markers in laboratory tympanometric examination was normal (Figure 2e and 2f). The patient tests (but these were not so high as before the immunosuppressive reported persistent tinnitus localised in both ears, which was slightly treatment). The patient was treated with prednisone (the average dose reduced after the use of a hearing-aid in the right ear. In the meantime fluid in the right sub-Tenon’s space and the rotary horizontal nystagmus to the right were observed. In laboratory tests inflammatory markers (ESR 79 mm/h, CRP 266 mg/l) and leukocytosis (WBC 27.1 109/l) were elevated, but ANCA remained negative (Table 1). During the following week the same symptoms appeared in association with the other ear. A series of treatments in a hyperbaric chamber was prescribed as additional therapy but with no improvement. Blurred vision with aggreveated corneal lesions, in this circumstance recognized as interstitial keratitis (IK), also reappeared. Based on
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