Background/Purpose: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is a rare and poorly understood entity with clinical features of both relapsing polychondritis (RP) and Behcet’s disease (BD). This study characterized the clinical features of MAGIC syndrome and derived a disease definition for use in future studies.

Methods: Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome. Clinical characteristics were compared between the pooled cases of MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of three different criteria to classify MAGIC syndrome were evaluated.

Results: Of the 96 patients with RP in the cohort study, 13 (14%) had MAGIC syndrome. The systematic review identified 27 additional MAGIC syndrome cases in literature. Patients reported in the literature were more likely to have ear chondritis (96% vs 54%, p < 0.01) and less likely to have nasal chondritis (63% vs 100%, p = 0.02), airway chondritis (19% vs 69%, p < 0.01), and costochondritis (7% vs 85%, p < 0.01) when compared with patients in the cohort study. When comparing clinical manifestations seen in BD, patients with MAGIC reported in the literature had a greater prevalence of BD-related cutaneous involvement (erythema nodosum, pseudofolliculitis or pathergy) than patients in the cohort study (48% vs 8 %, p = 0.01). Raynaud’s phenomenon was reported in 54% patients in the cohort study but was not described in any cases of MAGIC syndrome reported in literature. Anti-collagen II antibodies were not reported in any MAGIC syndrome cases in literature.

Pooling the 40 cases together and comparing them with non-MAGIC RP, there was significantly higher prevalence of almost all BD-related features in patients with MAGIC syndrome, including BD-related ocular involvement (uveitis or retinal vasculitis (, 28% vs 4%, p < 0.01), BD-related cutaneous involvement (erythema nodosum, pseudofolliculitis or pathergy (, 35% vs 1%, p < 0.01), GI involvement (23% vs 4%, p < 0.01) and CNS involvement (8% vs 0, p = 0.04). There was also a higher prevalence of aortitis (23% vs 1%, p < 0.01) and Raynaud’s phenomenon (54% vs 11%, p < 0.01) and anti-collagen II antibodies (50% vs 9%, p = 0.04) in MAGIC syndrome. Fulfillment of either McAdam’s or Damiani’s Criteria for RP plus the International Criteria for Behçet's Disease (ICBD) had excellent sensitivity (98%) to classify cases of MAGIC syndrome. Use of presence of oral and genital ulcers to define MAGIC syndrome had lower sensitivity (88%). In contrast, the International Study Group (ISG) criteria performed poorly for defining MAGIC syndrome with low sensitivity (43%).

Conclusion: MAGIC syndrome can be diagnosed in a substantial proportion of patients with RP. These patients have features of RP, BD, and other unique features such as aortitis, Raynaud’s phenomenon and anti-collagen II antibodies. Fulfillment of McAdam’s or Damiani’s criteria for RP and the ICBD criteria for BD can be used to define MAGIC syndrome in future research studies.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-prospective-observational-cohort-study-and-systematic-review-of-40-patients-with-mouth-and-genital-ulcers-with-inflamed-cartilage-magic-syndrome/