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.

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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/