SESSION TITLE: Miscellaneous Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: MAGIC syndrome (Mouth and Genital ulcers with Inflamed Cartilage) was first described by Firestein in 1985. It was thought in the first 5 cases published then to be a combination of relapsing Polychondritis and Behcet disease. Since then there are few cases published. We describe a young female who presented with severe disease necessitating tracheotomy during pregnancy and discuss the management followed by review of the literature.
CASE PRESENTATION: A 33 years old Jordanian female attended our ENT clinic for assessment of hoarseness of voice and cough. She had repeated attacks of hoarseness of voice and difficulty of breathing in the past for which she responded to oral steroids. No definite diagnosis was given to the patient although asthma was suggested. In the ENT clinic examination then showed airway inflammation and crusting and so impression of Rhinoscleroma was made and she was found to be pregnant her steroids were stopped. She presented after 1 week with upper airway obstruction which necessitated emergency tracheotomy. Review of her history revealed recurrent mouth & genital ulcers and her investigations showed inflamed cartilage (Bronchoscopy and biopsy) .Radiology and bronchoscopy showed significant subglottic stenosis. She was managed with steroids and Azathioprine. After delivery of her child she was reassessed, tracheostomy capped and she had undergone dilation of the stenosed segment.
DISCUSSION: Since its description in 1985 by Firestein ,MAGIC syndrome has rarely been reported . The recent literature has reported more vascular disease( aneurysms) and evidence that ani-TNF medications use in resistant disease.What remain to be answered: is MAGIC syndrome a new disease or a combination of polychondritis and Behcet.The reported cases come from different ethnicities and the numbers are small to generalize.The other challenge was the use of immunosuppressants in a pregnant patient which successfully completed pregnancy with normalization of the airway mucosa with therapy and hopefully recovery of the airway patency with dilatation and lazer therapy.
CONCLUSIONS: MAGIC syndrome is a rare disease and even challenging when it presents in pregnancy to present .Our patient thought of abortion but she was successfully managed with steroids and Azathioprine. We discuss the clinical, radiological presentation of the disease and review the literature. Our case would be the first reported in the Middle East and the first to diagnose during pregnancy.
Reference #1: 1. Kotter I, Deuter C, Gunaydin I, Zierhut M. MAGIC or not MAGIC--does the MAGIC (mouth and genital ulcers with inflamed cartilage) syndrome really exist? A case report and review of the literature. Clin Exp Rheumatol. Sep-Oct 2006;24(5 Suppl 42):S108-112.
Reference #2: 2. Imai H, Motegi M, Mizuki N, et al. Mouth and genital ulcers with inflamed cartilage (MAGIC syndrome): a case report and literature review. Am J Med Sci. Nov 1997;314(5):330-332.
Reference #3: 3.Firestein GS, Gruber HE, Weisman MH, Zvaifler NJ, Barber J, O'Duffy JD. Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behcet's disease. Am J Med. Jul 1985;79(1):65-72.
DISCLOSURE: The following authors have nothing to disclose: Abdulmalik Alsaied, Abdelhaleem Bella, Manal Jalil, Adel Lotfy, Raed Bukhari
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