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Immunoglobulin (Ig) G4-related disease (also known as 'IgG4-related sclerosing disease', 'IgG4-related systemic disease' or 'hyper-IgG4-disease') is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ.

The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.