Author(s): Bacciu A, Buzio C, Giordano D, Pasanisi E, Vincenti V,
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Abstract OBJECTIVES: Churg-Strauss syndrome (CSS) is a systemic vasculitic disorder of unknown etiology that affects small-to-medium-size blood vessels. Patients affected by CSS frequently show ear, nose, and throat manifestations, which are often present at the time of disease onset. The purpose of this study was to determine the frequency of nasal polyposis in a series of 29 patients with CSS and to correlate the nasal findings to the total health situation of these patients. STUDY DESIGN: Retrospective analysis. SETTING: Department of Otolaryngology and Department of Clinical Medicine, Nephrology and Health Science, University of Parma. METHODS: Twenty-nine patients with CSS were identified. Of the 29 patients, 17 (58.6\%) had nasal polyposis and were enrolled in this study. The nasal polyps were graded according to the Lund and Mackay endoscopic and radiological classifications. RESULTS: At diagnosis, endoscopic intranasal evaluation identified nasal polyposis of grade 3 in nine cases (52.9\%), grade 2 in six cases (35.2\%), and grade 1 in the remaining case (5.8\%). After corticosteroid and immunosuppressive therapy, clinical remission was achieved in 14 patients (82.3\%), whereas 3 patients experienced a relapse. Posttreatment endoscopic evaluation showed a permanent disappearance (grade 0) of nasal polyps in eight patients (47\%). The other nine patients (52.92\%) were found to have a small polyp situated in the middle meatus (grade 1). CONCLUSIONS: Nasal polyposis in patients with CSS may represent the initial phase of the syndrome, though patients often have concurrent pulmonary disease. Corticosteroid therapy either alone or combined with immunosuppressive drugs usually yielded improvement or stabilization.
This article was published in Laryngoscope
and referenced in Journal of Genetic Syndromes & Gene Therapy