Cogan’s syndrome – A case series
Sažetak
Introduction. Cogan’s syndrome is a rare variable vessel vasculitis. It can be typical and atypical. Basis of the treatment comprises glucocorticoids, and in patients with systemic manifestations, immunosuppressive drugs. Case report. We wanted to present the experience of the Clinic for Rheumatology and Clinical Immunology of the Military Medical Academy, Belgrade, in diagnosing and treating patients suffering from Cogan’s syndrome. The analysis included 7 patients. Patients’ demographic characteristics, disease manifestations, course of the disease, applied treatment and treatment outcome were analysed. Five of the patients were women and 2 were men, with the average age of 39 ± 13 (25–65) years. The typical form of the disease manifested in 1 patient. In 6 patients, the first manifestation was the audiovestibular dysfunction. In 1 patient, systemic manifestations were the first to appear. In the cases where the disease manifested atypically, 3 patients developed conjunctivitis, 2 episcleritis, and 1 uveitis. They all had systemic manifestations. One female patient was diagnosed with aortitis and aortic insufficiency. They all tested positive for inflammatory biohumoral syndrome. Four patients had positive antinuclear antibodies, 3 anticytoplasmic antibodies, and 1 positive rheumatoid factor. They were all treated with glucocorticoid and immunosuppressive drugs. Methotrexate was administered to all the patients in doses up to 20 mg per week. Pulses of cyclophosphamide were administered to 2 female patients. All patients went successfully into remission. The female patient with the typical form of the disease experienced permanent bilateral hearing loss. Conclusion. Patients with a rapidly developed audiovestibular dysfunction should be viewed as suffering from Cogan’s syndrome from the viewpoint of differential diagnosis. A timely treatment with glucocorticoids can prevent hearing loss and the development of systemic manifestations of the disease. Precedence should be given to methotrexate when selecting an immunosuppressive drug.
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REFERENCES
Cogan DG. Syndrome of no syphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945; 33: 144‒9.
Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syn-drome: Studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine (Baltimore) 1980; 59(6): 426‒41.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Confer-ence Nomenclature of Vasculitides. Arthritis Rheum 2013; 65(1): 1‒11.
Vollertsen RS, McDonald TJ, Younge BR, Banks PM, Stanson AW, Ilstrup DM. Cogan's syndrome: 18 cases and a review of the literature. Mayo Clin Proc 1986; 61(5): 344‒61.
Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan syn-drome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006; 81(4): 483‒8.
Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P. Study Group for Cogan's syndrome . Typical and atypical Cogan's syndrome: 32 cases and review of the literature. Rheumatology (Oxford) 2004; 43(8): 1007‒15.
Majoor MH, Albers FW, van der Gaag R, Gmelig-Meyling F, Huizing EH. Corneal autoimmunity in Cogan's syndrome? Re-port of two cases. Ann Otol Rhinol Laryngol 1992; 101(8): 679‒84.
Helmchen C, Arbusow V, Jäger L, Strupp M, Stöcker W, Schulz P. Cogan's syndrome: Clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol 1999; 119(5): 528‒37.
Yamanishi Y, Ishioka S, Takeda M, Maeda H, Yamakido M. Atypical Cogan's syndrome associated with antineutrophil cy-toplasmic autoantibodies. Br J Rheumatol 1996; 35: 601‒3.
Lunardi C, Bason C, Leandri M, Navone R, Lestani M, Millo E, et al. Autoantibodies to inner ear and endothelial antigens in Cogan's syndrome. Lancet 2002; 360(9337): 915‒21.
Mazlumzadeh M, Matteson EL. Cogan's syndrome: An audio-vestibular, ocular, and systemic autoimmune disease. Rheum Dis Clin North Am 2007; 33(4): 855‒74, vii-viii.
Espinoza GM, Prost A. Cogan's syndrome and other ocular vas-culitides. Curr Rheumatol Rep 2015; 17(4): 24.
Allen NB, Cox CC, Cobo M, Kisslo J, Jacobs MR, McCallum RM, et al. Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan's syn-drome. Am J Med 1990; 88(3): 296‒301.
Pouchot J, Vinceneux P, Bouccara D, Sterkers O, Bodelet B. Metho-trexate as a steroid-sparing agent in Cogan's syndrome: Com-ment on the concise communication by Richardson. Arthritis Rheum 1995; 38(9): 1348‒9.
Cochrane AD, Tatoulis J. Cogan's syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses. Ann Thorac Surg 1991; 52(5): 1166‒7.
Gasparovic H, Djuric Z, Bosnic D, Petricevic M, Brida M, Dotlic S, et al. Aortic root vasculitis associated with Cogan's syndrome. Ann Thorac Surg 2011; 92(1): 340‒1.
Sevgi DD, Sobrin L, Papaliodis GN. Cogan syndrome with se-vere medium and large vessel vasculitis. Digit J Ophthalmol 2016; 22(1): 32‒4.
Dekker JJ, Dinant HJ, van Soesbergen RM. Cogan's syndrome as an extra-articular manifestation of rheumatoid arthritis. Clin Rheumatol 1996; 15(4): 374‒7.
Froehlich F, Fried M, Gonvers JJ, Saraga E, Thorens J, Pecoud A. Association of Crohn's disease and Cogan's syndrome. Dig Dis Sci 1994; 39(5): 1134‒7.
Kessel A, Vadasz Z, Toubi E. Cogan syndrome-pathogenesis, clinical variants and treatment approaches. Autoimmun Rev 2014; 13(4‒5): 351‒4.
Bonaguri C, Orsoni J, Russo A, Rubino P, Bacciu S, Lippi G, et al. Cogan's syndrome: anti-Hsp70 antibodies are a serological marker in the typical form. Isr Med Assoc J 2014; 16(5): 285‒8.
Bovo R, Ciorba A, Trevisi P, Aimoni C, Cappiello L, Castiglione A, et al. Cochlear implant in Cogan syndrome. Acta Otolaryn-gol 2011; 131(5): 494‒7.
Pasanisi E, Vincenti V, Bacciu A, Guida M, Berghenti T, Barbot A, et al. Cochlear implantation and Cogan syndrome. Otol Neurotol 2003; 24(4): 601‒4.
Bacciu A, Pasanisi E, di Lella F, Guida M, Bacciu S, Vincenti V. Cochlear implantation in patients with Cogan syndrome: Long-term results. Eur Arch Otorhinolaryngol 2015; 272(11): 3201‒7.
Richardson B. Methotrexate therapy for hearing loss in Cogan's syndrome. Arthritis Rheum 1994; 37(10): 1559‒61.
Riente L, Taglione E, Berrettini S. Efficacy of methotrexate in Cogan's syndrome. J Rheumatol. 1996; 23(10): 1830‒1.
Tayer-Shifman OE, Ilan O, Tovi H, Tal Y. Cogan's syndrome: Clinical guidelines and novel therapeutic approaches. Clin Rev Allergy Immunol 2014; 47(1): 65‒72.