Sclerosing mediastinitis and periaortitis in a patient with IgG4 disease. A case report

Abstract

IgG4-related disease is a systemic inflammatory condition, where IgG4-positive plasma cell complexes play a fundamental role, which infiltrate various organs and systems and manifest with elevated serum IgG4 levels. The diagnosis of this disease is complex and the clinical findings are not pathognomonic, however, it should be considered among the differential diagnoses of inflammatory diseases with multiple organ involvement. The most affected organs are the salivary glands, biliary tract, pancreas, retroperitoneum, lungs, trachea and skin; however, recently, the presence of sclerosing mediastinitis has been described as a form of IgG4 disease, whose early recognition and diagnosis are essential, since a delay in the initiation of adequate treatment leads to progressive fibrosis with irreversible damage and poor prognosis for the patient. The following is the case of a patient with imaging findings of sclerosing mediastinitis, with infradiaphragmatic adenopathies and periaortitis as main manifestations and in whom, together with blood tests and clinical history, the diagnosis of IgG4 disease was made. Special emphasis is made on the imaging findings and their importance for the radiologist when suspecting and/or diagnosing this disease.