Superior vena cava obstruction and pulmonary artery involvement as rare findings of immunoglobulin G4-related disease

We describe a case of immunoglobulin G4-related lung disease presenting as chronic pulmonary hyper- tension with involvement of right pulmonary artery and superior vena cava. Immunoglobulin G4- related disease is a rare systemic sclerosing disease with autoimmune entity that causes fibrotic, often mass-like manifestations that variably affect different or gan systems and can be mis-taken with other disorders. Timely diagnosis requires awareness on the part of clinicians and pathologists as well as radiologists to the variable manifestations of this newly recognized disorder. < Learning objective: Pulmonary hypertension is a rapidly growing field in cardiopulmonary medicine and the learning objective of this article is to highlight this relatively new subset of pulmonary hypertension and to alert the caring physicians. >