TY - JOUR T1 - Acquired C1-inhibitor deficiency presenting with nephrotic syndrome JF - BMJ Case Reports JO - BMJ Case Reports DO - 10.1136/bcr-2019-230388 VL - 12 IS - 7 SP - e230388 AU - Jamie Willows AU - Katrina Wood AU - Helen Bourne AU - John Andrew Sayer Y1 - 2019/07/01 UR - http://casereports.bmj.com/content/12/7/e230388.abstract N2 - Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma. Both the acquired C1-INH deficiency and the nephrotic syndrome resolved when the underlying B-cell lymphoma was treated with rituximab and bendamustine, suggesting the underlying lymphoproliferative malignancy was driving both disorders. ER -