• Rachel Jen, Bradly Biagioni, Pearce Wilcox, and Bob Schellenberg.
• Chest. 2014 Mar 1;145(3 Suppl):218A.

Session TitleILD Case Report Posters IISESSION TYPE: Case Report PosterPRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PMINTRODUCTION: HIGM are a set of disorders with intrinsic B-lymphocyte defects with class switching. This results in high or normal levels of IgM, but low or no production of other immunoglobulins, leading to immunodeficiency. At the same time, uncontrolled B-cell activation without class switch triggers lymphoproliferation and autoimmune phenomena.Case PresentationA 19-year-old male presented with increasing non-productive cough for two weeks. His past medical history is significant HIGM, for which he was under long-term treatment with subcutaneous immunoglobulin; query mixed connective tissue disease; and NSIP. Prior to his presentation, he had self-administered a short course of oral prednisone and azithromycin, and stopped his immunoglobulin therapy. After admission for community acquired pneumonia, his condition rapidly deteriorated and he was intubated for impending hypoxemic respiratory failure. Bronchoscopy performed in ICU was negative for infection. The CT scan showed diffuse mediastinal lymphadenopathy and bilateral ground glass opacification with interlobular septal thickening. He was subsequently taken to the operating room for surgical mediastinoscopy and wedge lung biopsy as he did not improve significantly with broad-spectrum antimicrobial coverage. The biopsies showed reactive lymph nodes and acute exacerbation of NSIP. Thus, he was treated with pulse steroids with a single dose of cyclophosphamide. He improved clinically and was extubated shortly afterward. In consultation with the rheumatology and immunology service it was decided that the patient was suffering from autoimmune disease secondary to HIGM. He was started on a course of weekly Rituximab in total of four treatment to suppress the autoimmune phenomenon.DiscussionILD is a recognized complication in immunodeficiency. Our patient's biopsy has lymphoid hyperplasia with a typical feature of NSIP. He has previously been assessed by multiple physicians in Europe and North America. His diagnosis of HIGM is in general agreement but treatment of his NSIP is debated among his previous physicians. Some physicians believed that NSIP should be treated as part of HIGM with immunoglobulin supplementation but some physicians recommended treatment of NSIP with immunosuppressant. We suggested Rituximab treatment decrease B-cell activation to suppress the autoimmune phenomenon as targeted therapy.ConclusionsA combination of Rituximab with immunoglobulin replacement could be a safe and effective therapy to control infection, lymphoproliferation and autoimmunity in patients with HIGM.Reference #1: Hennig C, Baumann U, Ilginus C, Horneff G, Foell J, Hansen G. Successful treatment of autoimmune and lymphoproliferative complications of patients with intrinsic B-cell immunodeficiencies with rituximab. Br J Haematol. 2010 Feb;148(3):445-8DISCLOSURE: The following authors have nothing to disclose: Rachel Jen, Bradly Biagioni, Pearce Wilcox, Bob SchellenbergNo Product/Research Disclosure Information.

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