IgG4-RSD is a novel clinical entity that shows IgG4 producing plasma cells in affected organs with fibrotic and sclerotic changes, and elevated serum IgG4 levels. IHerein, we report one case of IgG4-RLD, presenting with pleural effusion, with lab values showing high IgG4 levels and pleural biopsy showing infiltrated plasma cells with strong immunoreactivity for IgG4. A 55year old Caucasian female, nonsmoker with no significant past medical history presented to the clinic for b/l hand swelling and occasional dyspnea. Physical examination was unremarkable except for decreased breath sounds left lung, and b/l hand swelling.There was no evidence of synovitis involving hands or any other joints. Range of motion was normal. The routine blood tests Immunological markers were normal except high IgG-4levels Pleural fluid aspiration was suggestive of exudative effusion. Pleural biopsy was performed that was suggestive of massive lymphoplasma cell infiltration in the pleura, with immunostaining revealing predominant IgG4 positive plasma cells greater than 30hpf.He was started on steroid therapy with clinical significant improvement in few weeks. IgG4RSD is an unrecognized condition about which knowledge is now growing rapidly., Acknowledging that IgG4RSD may present as pleural effusion is essential for early management and preventing complications
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