Pharmacological and Psychosocial Strategies in the Management of Severe Behavioral Problems in Autism Spectrum Disorder: A Case Study.

Autoimmune pancreatitis (AIP), first proposed by Yoshida ., is classified into types 1 and 2 and, recently, “type 3 (unspecified).” Type 1 AIP, also referred to as lymphoplasmacytic sclerosing pancreatitis, is a key manifestation of immunoglobulin G4-related disease (IgG4-RD), defined by periductal lymphoplasmacytic infiltration, storiform fibrosis, occlusive phlebitis, and often elevated serum IgG4. It is more common in Asians than Europeans/ Americans, and while AIP accounts for only 6% of chronic pancreatitis, its prevalence has risen (e.g., the incidence of type 1 AIP in Japan has increased from 0.8 to 3.1/100,000, 2007-2016), warranting clinical attention. The 2011 International Consensus Diagnostic Criteria (ICDC) integrates serum IgG4 levels, imaging, extrapancreatic involvement, pathology, and glucocorticoid response for AIP diagnosis. Long recognized that as the core serological biomarker for type 1 AIP, serum IgG4 is a subtype of IgG: It maintains immune homeostasis by participating in immune regulation and suppressing excessive inflammation, and its abnormal elevation is closely linked to the immune-mediated inflammatory mechanism of type 1 AIP. A study has shown that the sensitivity and specificity of serum IgG4 for type 1 AIP diagnosis are 72 and 93%, respectively. However, 10-30% of type 1 AIP patients have normal serum IgG4 levels (<135 mg/dL), defined as serum IgG4-negative (IgG4-N). In contrast, patients with serum IgG4 concentrations ≥135 mg/dL are classified as serum IgG4-positive (IgG4-P). These serum IgG4-N patients face significant diagnostic dilemmas: their misdiagnosis rate is notably higher. In addition, for those with pancreas-limited disease, the rate of unnecessary surgical interventions is significantly higher than that in serum IgG4-P patients, due mainly to the difficulty in distinguishing AIP from pancreatic cancer. Studies on differences between serum IgG4-N and serum IgG4-P patients are conflicting (e.g., recurrence rate discrepancies). Pathology is critical for diagnosis (ICDC requires >10 IgG4-P cells/high-power field [HPF]), with most tissue obtained through endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB). However, existing research focuses on EUS-FNA/FNB method/needle type effects on diagnosis, with few studies comparing histopathological features (e.g., tissue IgG4 count, confirmation rate) between serum IgG4-N and serum IgG4-P patients or exploring serum IgG4-tissue IgG4 correlation, a gap limiting type 1 AIP typing and management. This study retrospectively analyzed 78 type 1 AIP patients (Changhai Hospital Affiliated with Naval Medical University, Dec 2019-2023) stratified by serum IgG4 (serum IgG4-N: <135 mg/dL; serum IgG4-P: ≥135 mg/dL). Clinical baseline, laboratory/imaging data, diagnosis-treatment strategies, and histopathology were compared between groups, and serum IgG4-tissue IgG4 correlations were explored. The goal was to clarify the association of serum IgG4 typing with type 1 AIP clinicopathological features, providing a basis for accurate diagnosis and stratified management.