CLINICAL ANALYSIS OF THE COMMON VARIABLE IMMUNODEFICIENCY IN PATIENTS WITH LUNG DISEASES: EXPERIENCE IN JAPAN

Abstract

SESSION TITLE: Monday Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM PURPOSE: Common variable immunodeficiency (CVID) is a heterogeneous subset of primary immunodeficiencies, which is characterized by hypogammaglobulinemia, defective antibody production and increased susceptibility to infection. Clinically, recurrent respiratory infections are a significant problem because those might lead to bronchiectasis, declining in lung function, and impairment of their quality of life. It is reported that over 90% of CVID patients suffer from the upper and lower respiratory tract infections due to increased susceptibility to bacterial pathogens. The purpose of this study is to assess clinical characteristics of CVID in our patients with lung diseases and to reveal the relationship between CVID and respiratory infections, especially lower respiratory tract infections. METHODS: From April 2016 to September 2018, consecutive 3417 patients with lung diseases, whose serum immunoglobulin were measured, were retrospectively studied. Among those cases, 451 patients with hypogammaglobulinemia were detected, and 336 patients were diagnosed with primary hypogammaglobulinemia, and 112 were secondary as a result of medications with corticosteroids or immunosuppressant, and so on. Of 336 patients with primary hypogammaglobulinemia, 37 patients were clinically diagnosed with CVID. Clinical features and lower respiratory infection frequency of those cases were analyzed retrospectively. RESULTS: Anong total of 37 patients with CVID, 7 cases showed low levels of IgG, IgA, and IgM (Gr. A), 10 did low levels of IgG and IgA (Gr. B), 20 did low levels of IgG and IgM (Gr. C), and 2 did low levels of IgA and IgM (Gr. D). Depending upon each Gr., 6 cases (85.7%), 6 (60%), 9 (45%), and 0 (0%) had any previous histories of lower respiratory infections, respectively. In addition, 4 (66.6%), 3 (50%), 1 (11%), 0 (0%) of each Gr. experienced recurrent infections. Bacteriologically, Klebsiella spp. (7), Pseudomonas spp. (5) and Haemophilus influenzae (4) were the three most common bacterial pathogens detected in respiratory samples (sputum, bronchial washings) from CVID patients. Radiologically, bronchiectasis (19) and nodular shadows (12) were often detected in Chest CT among those patients with CVID. CONCLUSIONS: The present study revealed that lower respiratory infections occurred in CVID patients with high frequency, and that majority of them suffered from recurrent infections, which might lead to bronchiectasis and bacterial colonization of the respiratory tract. Especially, low immunoglobulin levels of IgG, IgA, and IgM were at high risk for infections. CLINICAL IMPLICATIONS: Clinicians should take into consideration the possibility of CVID when a patient suffers from recurrent infections and show hypogammaglobulinemia. DISCLOSURES: No relevant relationships by NAOKI ARAI, source=Web Response No relevant relationships by Hitomi Goto, source=Web Response No relevant relationships by Kenji Hayashihara, source=Web Response No relevant relationships by Kentaro Hyodo, source=Web Response No relevant relationships by Yuka Kitaoka, source=Web Response No relevant relationships by Sosuke Matsumura, source=Web Response No relevant relationships by Yukiko Miura, source=Web Response No relevant relationships by Mizu Nonaka, source=Web Response No relevant relationships by SHUJI OH-ISHI, source=Web Response No relevant relationships by Takefumi Saito, source=Web Response No relevant relationships Added 03/12/2019 by Yuika Sasatani, source=Web Response, value=Salary Removed 03/12/2019 by Yuika Sasatani, source=Web Response No relevant relationships by TAKAFUMI SHIMADA, source=Web Response No relevant relationships by YUKI TAKAGI, source=Web Response