Severe pulmonary vein stenosis in association with IgG4 gammopathy: An unusual case

IgG4-related disease (IgG4-RD)is a multifactorial and systemic immune-mediated disease. Pulmonary vein stenosis related to IgG4 is a rare entity. We report a case of a 57-year-old male who presented with vague symptoms of weakness, easy fatigue, shortness of breathing, chest tightness, and occasional palpitations. The patient was admitted, and investigations were arranged in the form of anterior mediastinotomy and pleural biopsies were taken to look for a possible diagnosis. The histopathology was reported as IgG4-related immunopathy. A transoesophageal echocardiogram was performed and this showed severe stenosis of left superior pulmonary vein. In patients with rare pathologies such as IgG4-RD, a transesophageal can be a useful diagnostic aid especially in patients with severely impaired renal functions which does not allow utilization of contrast computed tomography.