MMP case series, supplementary material
Description
Background: The variable clinical severity of MMP often leads to a diagnostic and therapeutic delay. Objective: To describe the characteristics in a large cohort of patients with MMP. 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.