MMP case series, supplementary material

Methods

A retrospective review study of clinical and diagnostic characteristics and treatment response in 145 patients with MMP. Results: Monosite involvement was seen in 41.4% and multisite involvement in 58.6% patients. The oral mucosa was affected in 86.9%, followed by the ocular mucosa (30.3%), skin (26.2%), genital mucosa (25.5%), nasal mucosa (23.4%) and pharyngeal and/or laryngeal mucosa (17.2%).

Ocular disease developed during disease course in 41.7% of patients with initially other mucosal site involvement. The malignancy rate was significantly higher in patients with autoantibodies against laminin-332 compared to MMP patients without laminin-332 autoantibodies (35.3% vs. 10.9%, p=0.007). Systemic immunosuppressive or immunomodulatory therapy were administered in 77.1% of the patients, mainly in patients with multisite involvement (p<0.001), ocular involvement (p<0.001) and pharyngeal and laryngeal involvement (p=0.002).

The remaining patients (22.9%) received topical therapy. Adverse events were frequently reported. Limitations: retrospective design.

Conclusion: MMP presents with a heterogeneous clinical presentation and new symptoms may develop during the disease course. Cancer screening should be considered in MMP and in particular with autoantibodies against laminin-332.