Orofacial Granulomatosis: Clinical and Therapeutic Features in an Italian Cohort And Review of the Literature.

BACKGROUND Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10different Italian Centers and summarized the most recent literature data. METHODS a review ofpatients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG. RESULTS Thirty-nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lipsand 28.2% suffered from Melkersson-Rosenthal Syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG.Oral aphthosis and cervical lymphadenopathy were also described.The mean diagnostic delay was 3.4 years.Histological evaluation was performed in 34/39 patients (87.2%); non-caseating granulomas were found in 73.5% of them.Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%) and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results.Anti-TNF-α and anti-IgE monoclonal antibodieswere used in 6(15.4%) and 1(2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response. CONCLUSIONS OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial,managementis difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establishregistry databases and address challenges of long-term management.