Abstract
Lymphangioleiomyomatosis (LAM) represents an uncommon disorder characterized by cystic lung destruction and chronic respiratory failure. Lung damage caused by various mechanisms may represent a hypothesis for studying the association between LAM and rheumatoid arthritis (RA), which is the most prevalent autoinflammatory rheumatic disease and may affect the lungs as an extra-articular manifestation. Despite their distinct clinical presentations, the pathophysiology of both disorders includes dysregulated immunological function, abnormal cellular development, and inflammation. Current research suggests a potential relationship between RA and LAM, as some RA patients have been reported to develop LAM. However, the association of RA and LAM raises important therapeutic dilemmas. For this reason, the trajectory of a patient who was identified in our medical records as suffering from both LAM and RA, treated with many novel molecules and biological therapy, but with a negative outcome due to respiratory and multiorgan failure, has been exemplified. The delay in the diagnosis of LAM is due to a correlation between RA and LAM, worsening the vital prognosis and also hindering pulmonary transplantation. In addition, extensive research is essential for understanding the potential connection between these two disorders and discovering any similar mechanisms involved that may underlie their occurrence. This may contribute to the development of new therapeutic options that target shared pathways implicated in the pathogenesis of RA and LAM.