Recurrent chylothorax in a patient with serositis as the only manifestation of IgG4-related disease: the diagnostic challenge and consideration

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterised by IgG4-positive plasma cell infiltration into different tissues and organs. Among the heterogeneous clinical features of IgG4-RD, serositis, including pleural and pericardial effusions, is a rare and poorly understood presentation. We described a woman in her late 70s who developed recurrent chylothorax and failed to respond to corticosteroids, immunosuppressives and intrapleural octreotide injection while being treated for newly diagnosed IgG4-RD with serositis as the only manifestation. Her chylothorax was thought to be due to fibroinflammatory damage of the lymphatic duct system from her IgG4-RD, as other differential diagnoses have been largely excluded. We demonstrate the difficulty in establishing the diagnosis of IgG4-RD when only serositis is present, the importance of meticulous workup ruling out other causes and clinical judgement in identifying disease complications versus evaluating alternative diagnoses.