• Iyad Somerville posted an update 1 year ago

    The observation of strong associations among autoimmunity, splenomegaly, enteropathy, and granulomas in Docetaxel molecular weight the univariate analysis prompted us to perform a multivariate analysis of these four variables. The results (see Table E7 in this article’s Online Repository at http://www.jacionline.org) suggested that splenomegaly and granulomas, as well as enteropathy and autoimmunity, form 2 pairs, which suggests that a patient is likely to have both splenomegaly and granulomas or both enteropathy and autoimmunity. Data for this analysis were available for 2134 patients (96.5% of the total 2212 patients), of whom 119 were deceased. We found that age at diagnosis and age at onset were both significantly associated with patient survival (P < .001). Older age at onset and older age at diagnosis were both associated with an increased risk of death at any time. A 1-year increase in age at diagnosis was associated with the risk of death increasing by 4.5% (for the age at onset, it is 3%). A longer diagnostic delay was also associated with an increased risk of death (1.7% per 1-year increase) and thus reduced survival times. The presence of a lymphoma and solid tumor was also associated with an increased risk of death. There was no strong evidence that any of the other variables examined were significantly associated with patient survival. Notably, in this data set there was also no difference between the group of patients with noninfectious complications and those without. In a multivariate analysis on the joint effect of the aforementioned variables, age at diagnosis was the main correlating factor with patient survival. To illustrate this, the survival of patients with early- and late-onset CVID is depicted in a Kaplan-Meier plot (Fig 3). The calculation of average immunoglobulin treatment intervals and monthly doses showed a statistically significant difference between centers (P < .001). There was a median intravenous immunoglobulin (IVIG) treatment interval of 30 days when all centers were analyzed together. The same value was obtained from both the patient-level and interval-level analyses. Most centers had exactly 30 days as the mean treatment interval (17/27). In the remaining 10 centers the median treatment interval varied from 21 up to 41 days (see Table E8 in this article's Online Repository at http://www.jacionline.org). There was a larger variation between centers in the average monthly dose (IVIG and subcutaneous immunoglobulin [SCIG], Table IV). The median dose for all centers combined was approximately 460 mg/kg/mo. The patient-level analysis indicated that the median dose in the centers ranged from 129 mg/kg/mo (Prague) up to 750 mg/kg/mo (Thessaloniki and Ippokration). When only considering the IVIG route, there was a median dose of 455 mg/kg/mo. This median dose varied from 130 mg/kg/mo (Prague) to 638 mg/kg/mo (Dresden).