Necrotising sinusitis with orbital complication in patient with macrophage activation syndrome (MAS)

Abstract

Background: Macrophage activation syndrome (MAC) is a potentially fatal clinical-laboratory syndrome of uncontrolled hyperinflammation arising as a result of hereditary or acquired immune-mediated processes of cellular overactivation and nonmalignant proliferation of tissue macrophages/histiocytes, which can cause multiorgan failure.
Case report: We present a clinical case of 15-years old child, who was diagnosed with juvenile idiopathic arthritis in 2019. The disease started with macrophage activation syndrome. In the next years the child had multiple hospitalizations in the Pediatrics clinic, but in the course of the disease it developed the picture of severe necrotic pansinuitis with an orbital complication, which required an immediate surgical intervention. Ever since the child had an ongoing necrotizing process in the area of the nasal passages, sinuses, upper jaw and hard palate. Other complications were breakthrough of the hard palate, loss of healthy teeth from the upper dentition and creation of direct communication between the oral cavity and the left maxillary sinus.
Conclusions: The diagnosis of MAS is difficult to make, but increased awareness of this disease is an essential for its recognition. The struggle with autoimmune diseases often lasts for years with periods of exacerbation and remission of symptoms. Complications related to them can affect different organs and systems and require interdisciplinary approach.