Background/Purpose: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is an extremely rare condition that encompasses symptoms of relapsing polychondritis (RP) and Behcet’s disease (BD). Little is known about the prevalence of clinical symptoms in patients with MAGIC and to what extent patients with MAGIC are similar to other patients with RP. The study objectives were to determine the frequency of patients with MAGIC syndrome and BD in a cohort of patients with RP and compare the clinical, laboratory and radiographic characteristics as well as treatment and outcomes.

Methods: Patients who met McAdams or Damiani’s diagnostic criteria for RP and were 18 years old or older were included in this analysis. The International Criteria for Bechet’s Disease (ICBD) was used for the diagnosis of BD. MAGIC syndrome was defined by presence of recurrent oral and genital ulcers plus chondritis. Categorical and continuous variables were compared using the Fisher’s exact test and Kruskal-Wallis test, respectively.

Results: Eighty-four patients were included in this analysis. Most of the patients were female (n=72, 86%) and white (n=73, 87%).  Median age of symptom onset was 36 years (IQR 28-42). Among patients with RP, 13 (15%) met diagnostic criteria for BD, all of whom had MAGIC syndrome. Although referred for RP, MAGIC syndrome was not recognized by any of the referring care providers. When comparing patients with RP and MAGIC syndrome, there were no significant differences between race or sex (white, 85% vs 87%, p=0.68; female 83% vs 100%, p=0.20). Patients with MAGIC syndrome were more likely compared to RP to have diagnostic delay (median 10.1 years (IQR 3-18) vs 6.9 years (IQR 1-9.75), p=0.04), a higher prevalence of Raynaud’s phenomena (54% vs 11%, p = 0.002), uveitis or retinal vasculitis (23% vs 4%, p =0.04), chronic GI symptoms (54% vs 13%, p = 0.002), and elevated anti-collagen II antibodies (60% vs 9 %, p=0.03). Although not statistically significant, patients with MAGIC syndrome also had a higher percentage of pulmonary embolism (15% vs 3%), unprovoked DVT (15 vs 3%), aortitis (8% vs 1%), airway chondritis (69% vs 41%), GI ulcers or inflammation (15% vs 4%) and erythema nodosum or pseudofolliculitis (8% vs 1%).  There were no significant differences in the prevalence of ear or nose chondritis, costochondritis, hearing loss, joint involvement, neurologic disease, elevated inflammatory markers, or HLA-B51 status. There were no significant differences in treatment or outcome measures including mortality, ICU admissions and tracheotomy between MAGIC and RP.

Conclusion: Fifteen percent of patients with RP meet diagnostic criteria for MAGIC syndrome and BD. Patients with MAGIC syndrome have distinct clinical and laboratory characteristics including prevalent vascular symptoms such as Raynaud’s, thromboembolic disease, and aortitis. Recognizing the wide pattern of organ involvement and clinical characteristics in patients with MAGIC syndrome may help identify these patients earlier. Larger studies to determine whether anti-collagen II antibodies in patients with MAGIC play a causal role in disease and/or function as a diagnostic biomarker are warranted.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/magic-syndrome-in-a-cohort-of-patients-with-relapsing-polychondritis/