Relationships between bronchiectasis and time to achieving target trough immunoglobulin G levels in patients with common variable immunodeficiency.

Background: The main treatment of common variable immunodeficiency (CVID) is to maintain immunoglobulin G (IgG) levels within the target range. However, trough IgG levels differ among patients with similar body mass index (BMI) and those receiving the same dose of immunoglobulin replacement therapy (IGRT). A crucial factor that underlies these differences is the presence of extensive bronchiectasis, which is associated with the immunoglobulin salvage pathway. Objective: We compared trough IgG levels in patients with CVID and with and in those without bronchiectasis who had received the same dose of IGRT for 2 years to determine the association of IgG level with infection frequency. Method: This retrospective cohort study included 61 patients with CVID, of whom 21 had bronchiectasis. We reviewed the electronic records for demographic variables, baseline immunoglobulin levels, mean trough IgG levels over 2 years, efficacy levels (trough IgG level - baseline IgG level), the time interval from treatment initiation to achieving the target trough IgG level (700 mg/dL), and the number of infections. Results: The median age of the patients was 39 years (IQR, 27-51), and 29 were women (47.5%). There were no significant differences between the groups in terms of age, age at diagnosis, delay in diagnosis, sex, BMI, IGRT type (subcutaneous or intravenous), and baseline immunoglobulin levels. Trough IgG and efficacy levels were lower (P < 0.001 and P = 0.016, respectively), the time required to achieve the target IgG level was longer in patients with bronchiectasis than in those without bronchiectasis, and this time interval was significantly associated with the infection frequency. Trough IgG and albumin levels were correlated (p = 0.007), with minor differences between the groups (p = 0.04). Conclusion: Bronchiectasis was significantly associated with a longer time to achieve the target IgG levels. These long-term differences between the patients with and those without bronchiectasis have significant clinical implications.

High digit ratio (2D:4D) is associated with attack frequency and severity in hereditary angioedema patients.

BACKGROUND: Hereditary angioedema (HAE) caused by a deficiency or dysfunction of the plasma protease C1-inhibitor is a rare autosomal-dominant disorder. We explored a possible correlation between the ratio of the second and fourth finger lengths (2D:4D) and the frequency of HEA attacks, and whether the ratio might predict laryngeal attack. METHOD: We evaluated 35 HEA patients aged 19 to 66 years; 3 were subsequently excluded. The 2D:4D ratio was calculated by dividing the length of the second finger by that of the fourth finger of both hands. A structured clinical questionnaire exploring HAE course and treatment over the prior year was administered. RESULTS: Of the 32 participants, 56.25 % (n = 18) were female. Of them, those with high 2D:4D ratios suffered significantly more laryngeal attacks than others; 93.3% of patients with high 2D:4D ratios experienced ≥5 attacks annually, significantly more than those with low ratios. Among type 2 HEA patients, 75 % of those experiencing ≥5 attacks annually had high 2D:4D ratios; all patients with low 2D:4D ratios reported <5 attacks annually. These significant effects were found for right-hand 2D:4D ratios and not left-hand 2D:4D ratios. CONCLUSION: The data suggest that intrauterine sex hormone exposure, which affects the 2D:4D ratio, is significantly associated with HEA attack frequency and severity, and laryngeal edema.