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Non-malignant, non-cirrhotic splanchnic vein thrombosis (NC-SVT) is an infrequent yet not negligible cause of portal hypertension (PHT). Despite often associated with myeloproliferative neoplasms (MPN), prothrombotic disorders (PD) or local factors (LF), up to 30% of cases are idiopathic. Long-term management is debated, especially for cases with no underlying factors. To describe the clinical course and management of a single-centre NC-SVT-patients cohort. A retrospective analysis of NC-SVT patients referred to our centre between November 2009 and September 2021, with at least three-months follow-up, was performed. Etiology, thrombosis extension, clinical presentation, and therapeutic strategy at SVT onset were retrieved. After referral, clinical, biochemical, and radiological outcomes were analyzed. A total of 22 NC-SVT patients were included (50% male, mean age at referral 53.1 years). Mean follow-up time was 29 months (10-66). The most frequent cause was MPN (31.2%), followed by LF (27.3%), and PD (13.6%). Most SVT (95.5%) involved the portal vein, six of which with spleno/mesenteric extension. One case had isolated intrahepatic involvement. Seven (15%) presented with PHT-related complications (three ascites, four variceal bleeding) and nine (40.1%) developed esophageal varices. All MPS and PD received long-term anticoagulation, except four cases (three excessive bleeding risk, one patient refusal). Most MPS cases (57%) underwent etiological therapy. Eight patients received beta-blockers at SVT onset, four of which as secondary prophylaxis of variceal bleeding. At referral, beta-blockers were added in two cases with radiological/biochemical evidence of PHT progression. During follow-up, four patients developed esophageal varices, but no PHT-related complications occurred. No significant variation from referral of biochemical parameters (platelet count, coagulation, bilirubin, albumin, aminotransferases) was detected. All SVT (100%) were stable/improved, and spleen length showed no significant variation. A single case of extrasplanchnic thrombosis occurred. We report excellent mid-term outcomes in a well-phenotyped NC-SVT-patients cohort. Additional prospective studies are needed. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4 (IgG4)-related disease (RD) is an immune-mediated disease, characterized by fibrosis and inflammatory pseudotumors. It affects multiple organs, being pancreas (P) and hepatobiliary tree (HB) the main targets. The diagnosis is usually complex, and the clinical manifestations provide the treatment indication. Our aim was to study the clinical, radiological, and histological behavior of a cohort of patients with IgG4-RD. A retrospective analysis of IgG4-RD patients prospectively collected between May 2012 and October 2022 was performed. Clinical, serologic, radiologic, and histopathologic data were collected, together with therapeutic strategy. A total of 13 IgG4- RD patients were included (84.6% male, 65.8 years). Twelve had HB involvement. Eight had both P and HB involvement, 2 of whom also had other organs (lacrimal and salivary glands, lungs). One patient had isolated P. The main clinical manifestation was cholestasis (92.3%), followed by cholangitis (53.8%) and pancreatic pain (53.8%). Circulating IgG4 levels were high in all tested patients (10 of 13), but above 4ULN in only 2. Two of the 9 patients with P involvement developed diabetes. Liver or pancreas biopsy was performed in 6 patients, lymphoplasmacytic infiltrate was the most common finding. Of these, IgG4 immunohistochemistry was performed in 4 patients, 2 of whom were above the 10/HPF diagnostic cutoff. The most common CT-scan/MRI finding was swollen pancreas, followed by biliary tract irregularities. One P and 1 HB pseudotumors, but no malignancies were described. Mean delay in diagnosis was 6 months (1-48). Eleven patients were treated with steroids, with good response. Of them, 3 patients were switched to azathioprine maintenance therapy. One steroid-refractory patient received rituximab. Despite the limited number of patients, this study confirms the heterogeneity of IgG4- RD manifestations, diagnostic challenges, and response to therapy. Further studies are needed to better understand this enigmatic disease. [ABSTRACT FROM AUTHOR]