title: [Atherosclerotic and hemorrhagic diseases in a patient with primary immune deficiency].
abstract: A 59-year-old man, who suffered from periodic fever with continuous elevation of the C-reactive protein (CRP) level was referred to our hospital. He had frequent respiratory infections and diarrhea since his childhood. The serum immunoglobulin (Ig) G level was low (537 mg/dl) while IgA and IgE were undetectable. The serum IgM level was elevated (737 mg/dl). Based on these clinical features, he was diagnosed with primary immune deficiency, hyper IgM syndrome. He had past histories of aortic aneurysm, which had been repaired surgically in his fifties. His persistent proteinuria made us to perform renal biopsy, which revealed nephrosclerotic changes. During the hospitalization, multiple events of subcortical brain hemorrhage, subarachnoid hemorrhage, and pulmonary alveolar hemorrhage occurred. Bleeding time and coagulation tests were normal. Antinuclear antibody, anti-neutrophil cytoplasmic antibody, or anti-cardiolipin antibody was absent. Herein, we described the first case of the immune deficiency associated with severe arteriosclerosis and hemorrhage.
authors.affiliation: Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU).
authors.forename: Mari
authors.initials: M
authors.lastname: Kamiya
authors.forename: Makoto
authors.initials: M
authors.lastname: Soejima
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Iwai
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Miyasaka
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Kohsaka
mesh_terms: D050197:Atherosclerosis / Q000150:complications*; D017809:Fatal Outcome; D006474:Hemorrhagic Disorders / Q000150:complications*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008297:Male; D008875:Middle Aged