597 Long Term Follow-up of Patients with Common Variable Immunodeficiency (Cvid) in Rio De Janeiro, Brazil: Clinical Phenotypes and Prognosis

BACKGROUND: CVID comprises a variety of clinical phenotypes that may influence the prognosis of the disease. Our goal was to investigate the clinical phenotypes and prognosis of a series of patients with CVID. METHODS: We evaluated 11 patients with CVID, according to the PAGID criteria in long-term clinical follow-up (> 10 years). Most patients were on regular use of intravenous immunoglobulin (IVIg), provided free of charge by the government. Clinical evaluation was performed monthly and exams every 6 months to 1 year, including immunological evaluation, hematologic, biochemical, autoimmune, stool, urine analysis, chest CT, abdominal ultrasound and specific investigations of infectious diseases and malignancies, when needed. RESULTS: The average follow-up was 21.9 years (12–34). Among the 11 patients, the mean current age was 39.8 years (16 to 62), 73% were female and 82% white. The age at symptoms onset ranged from 4 to 31 years (mean = 18) and diagnosis occurred between ages 11 and 47 (mean = 28). Most patients (55%) had the phenotype of infectious complications only, 27% had infections and immune thrombocytopenic purpura and 18% had infections and solid neoplasias. The most common infections were recurrent sinusitis (100%), pneumonia (82%), giardiasis (36%) and tuberculosis (18%). None of the patients developed lymphoproliferative and / or inflammatory complications. With regard to immunological changes, we observed that 4 patients (36%) experienced an increase in CD8 T lymphocytes and inversion of CD4/CD8 ratio. Adherence to the use of IVIg was good in 50% of patients, fair in 38% and unsatisfactory in 12%. All patients have good quality of life, performing their routine activities of study, work and leisure. CONCLUSIONS: In the population studied, the most frequent phenotypes were infectious complications or infectious complications + autoimmunity. Tuberculosis can be an important infectious complication in patients with CVID in endemic areas. The delay in the diagnosis of CVID, around 10 years, indicates the need to improve the diagnosis of PID in our country. With proper clinical management and good adherence to the use of IVIg, patients with CVID in developing countries may have survival and quality of life similar to those described in developed countries.