Clinical immunological characteristics of anti-interferon-γ autoantibodies syndrome: a 3 year prospective cohort study.

Anti-interferon-γ autoantibodies (AIGAs) syndrome is susceptible to disseminated opportunistic infections due to increased AIGAs, but its clinical immunological characteristics remain unrecognized. We conducted a prospective cohort study between January 2021 and December 2023, recruiting patients with opportunistic infections who were categorized into AIGAs-positive and AIGAs-negative groups. Clinical immunological data and outcomes were documented. A subset of AIGAs-positive patients received glucocorticoid treatment, and its effectiveness was evaluated. A total of 238 patients were enrolled, with 135 AIGAs-positive and 103 AIGAs-negative patients. AIGAs-positive patients showed higher rates of multiple pathogen dissemination, shorter progression-free survival (PFS), and increased exacerbation frequency. They also showed elevated erythrocyte sedimentation rate (ESR), globulin (GLB), immunoglobulin (Ig)G, IgE, and IgG4 levels. Among the 70 AIGAs-positive patients monitored for at least six months, three subtypes were identified: high AIGAs titer with immune damage, high AIGAs titer without immune damage, and low AIGAs titer without immune damage. Of the 55 patients followed for 1 year, decreasing AIGAs titer and immune indices (GLB, IgG, IgE, IgG4) were observed. Among the 31 patients with high AIGAs titer and immune damage treated with low-dose glucocorticoids at the stable phase, reductions were observed in immune indices and AIGAs titer in 67.74% of cases. In summary, AIGAs-positive patients exhibit infectious and immunological characteristics. Elevated AIGAs, IgG, IgG4, and IgE indicate abnormal immune damages. AIGAs titer generally decrease over time. Stable-phase AIGAs-positive patients can be categorized into three subtypes, with those having high AIGAs titer and increased immune indices potentially benefitting from glucocorticoid treatment.

[A retrospective survey of diagnostic techniques of pulmonary embolism in 13 general hospitals in Guangxi area].

OBJECTIVE: To survey different diagnostic techniques in diagnosing pulmonary embolism (PE). METHODS: Hospital records of PE cases in 13 AAA general hospitals in Guangxi area from 1995 to 2007 were studied retrospectively. Probable PE was defined as the diagnosis based on the clinical data and non-specific imaging, while the definite PE was defined as those with the diagnosis confirmed by specific imaging or autopsy. The percentage of various diagnostic methods of PE was analyzed. RESULTS: From 1995 to 2007, 237 definite PE and 223 probable PE were found in 13 hospitals, and they accounted for 51.52% and 48.48%, respectively, for all patients diagnosed as having PE. The percentage of definite PE cases during 1995-2001 and 2002-2007 were 14.63% and 55.13%, respectively (chi (2)=24.522, P<0.01). Among 237 definite PE, 2 positive diagnostic techniques were employed in 17 patients. Twenty-seven (11.39%), 214 (90.30%), 6 (2.53%), 5 (2.11%) and 2 (0.84%) patients were diagnosed by pulmonary angiography, CT pulmonary angiography (CTPA), ultrasonography, magnetic resonance imaging (MRI) and autopsy, respectively. No ventilation-perfusion lung scanning was performed in these patients. Compared with other diagnostic imaging, the percentage of CTPA in diagnosis of PE increased slightly since 2003. CONCLUSION: CTPA is the first choice in the diagnosis of PE in Guangxi area, and more attention should be paid to other diagnostic imaging techniques.