J Clin Immunol. 2025 May 13;45(1):92. doi: 10.1007/s10875-025-01880-4. ABSTRACT BACKGROUND: Although some reports indicate ocular involvement in Inborn Errors of Immunity (IEI) patients, the characteristics of this association remain unclear. Increased awareness can facilitate early diagnosis and prevention of visual complications. OBJECTIVE: To determine the prevalence and characterize ophthalmological manifestations in patients with IEI. METHODS: … Read more

J Clin Immunol. 2025 May 13;45(1):93. doi: 10.1007/s10875-025-01885-z. ABSTRACT Anti-interferon-gamma autoantibody (AIGA)-associated adult-onset immunodeficiency (AOID) is an emerging disease that can lead to serious opportunistic infections, which has a history of 20 years since it was first reported in 2004. It’s a hard-detected AOID caused by AIGA. In recent years, there has been an increasing … Read more

J Clin Immunol. 2025 May 8;45(1):91. doi: 10.1007/s10875-025-01879-x. NO ABSTRACT PMID:40338378 | DOI:10.1007/s10875-025-01879-x

J Clin Immunol. 2025 May 7;45(1):90. doi: 10.1007/s10875-025-01869-z. ABSTRACT Patients with primary immunodeficiency disease (PID) have an increased susceptibility to infection and may experience negative impacts on health-related quality of life (HR-QOL) and activities of daily living. This prospective observational study of patients aged ≥ 12 years with PID assessed HR-QOL, work impairment, and disease-related … Read more

J Clin Immunol. 2025 Apr 28;45(1):89. doi: 10.1007/s10875-025-01882-2. ABSTRACT Monogenic defects that impair the control of inflammation and tolerance lead to profound immune dysregulation, including autoimmunity and atopy. Studying these disorders reveals important molecular and cellular factors that regulate human immune homeostasis and identifies potential precision medicine targets. Here, we provide a detailed immunological assessment … Read more

J Clin Immunol. 2025 Apr 23;45(1):88. doi: 10.1007/s10875-025-01875-1. ABSTRACT PURPOSE: Chronic inflammation in inborn errors of immunity(IEI) caused by the infections or immune dysregulation is associated with the amyloid A (AA) amyloidosis development. This study aims to analyze the clinical characteristics, management strategies, and outcomes of patients with IEI complicated by AA amyloidosis, focusing on … Read more

J Clin Immunol. 2025 Apr 21;45(1):87. doi: 10.1007/s10875-025-01881-3. NO ABSTRACT PMID:40257485 | DOI:10.1007/s10875-025-01881-3

J Clin Immunol. 2025 Apr 16;45(1):86. doi: 10.1007/s10875-025-01878-y. ABSTRACT Unexplained neurological symptoms can pose a diagnostic challenge in patients with inborn errors of immunity (IEI) where the aetiology can be varied, and diverse pathologies may require contrasting treatments. Brain biopsy, the process of sampling brain tissue directly, has historically provided histological and microbiological information and … Read more

J Clin Immunol. 2025 Mar 28;45(1):85. doi: 10.1007/s10875-025-01877-z. ABSTRACT The life-threatening coronavirus disease 2019 (COVID-19) affects about 1 in 1,000 healthy people under 50 without underlying conditions. Among patients with critical COVID-19 pneumonia, rare germline variants at genes controlling type I IFN immunity have been reported in up to 5% of patients. Causal etiologies in … Read more

J Clin Immunol. 2025 Mar 27;45(1):84. doi: 10.1007/s10875-025-01873-3. ABSTRACT PURPOSE: The safety and tolerability of elapegademase (elapegademase-lvlr; Revcovi®) a PEGylated recombinant adenosine deaminase (ADA), were demonstrated in two Phase 3 clinical trials in the U.S. and Japan in patients with ADA-deficient severe combined immunodeficiency (ADA-SCID). Elapegademase replaced Adagen® (pegademase, a PEGylated bovine ADA) in 2018. … Read more