Unraveling Intravascular Lobular Capillary Hemangioma: A Comprehensive Scoping Review.

This scoping review focuses on intravascular lobular capillary hemangioma (ILCH), a rare and distinct subset of lobular capillary hemangioma (LCH). This study provides a comprehensive overview of ILCH, delving into its clinical characteristics, origins, pathogenesis, diagnostic methods, treatment options, and outcomes. Despite its rarity, ILCH presents unique diagnostic and management challenges due to its intravascular origin. The review emphasizes the importance of accurate differentiation from other vascular lesions and underscores the need for histopathological confirmation. This article discusses the presentation of ILCH in the reported literature. The pathogenesis remains uncertain, with factors such as trauma, inflammation, hormonal changes, and medications being considered potential contributors. Histopathological features, imaging techniques, and diagnostic tools are discussed, highlighting the distinct histological architecture of ILCHs and the importance of immunohistochemical staining for accurate diagnosis. Surgical excision is the primary approach for managing ILCH due to its potential complications, including superior vena cava (SVC) occlusion and thrombosis. This review concludes by outlining potential directions for future research, including investigating genetic and molecular mechanisms underlying ILCH development, developing targeted therapies, building patient registries for collaborative efforts, and exploring minimally invasive surgical techniques. The importance of long-term patient outcome studies and international collaborations is emphasized to enhance our understanding of this rare vascular anomaly.

Arterial Tortuosity Syndrome: Unraveling a Rare Vascular Disorder.

Arterial tortuosity syndrome (ATS) is a rare genetic disorder characterized by abnormal twists and turns of arteries, leading to cardiovascular complications. This syndrome, first reported around 55 years ago, is inherited in an autosomal recessive manner and affects both genders. ATS manifests primarily in childhood, with arterial abnormalities disrupting blood circulation, increasing shear stress, and causing complications, such as atherosclerosis and strokes. This article reviews the genetics, etiology, pathophysiology, clinical presentation, diagnosis, associated conditions, management, and challenges of ATS. The syndrome's genetic cause is linked to mutations in the SLC2A10 gene, affecting collagen and elastin synthesis. Arterial tortuosity, a complex phenomenon, arises from factors such as vessel elongation, anatomic fixation, and vessel diameter. ATS is one of many conditions associated with arterial tortuosity, including Marfan syndrome and Loeys-Dietz syndrome. Recent studies highlight arterial tortuosity's potential as a prognostic indicator for adverse cardiovascular events. Management requires a multidisciplinary approach, and surveillance and prevention play key roles. Despite challenges, advancements in understanding ATS offer hope for targeted therapies and improved patient care.

Advances in Understanding and Managing Autoimmune Hepatitis: A Narrative Review.

Autoimmune hepatitis (AIH) is a chronic liver disease characterized by immune-mediated destruction of hepatocytes, leading to inflammation and fibrosis. In recent years, significant advances have been made in understanding the pathogenesis, epidemiology, diagnosis, and treatment of AIH. This comprehensive narrative review aims to provide an up-to-date overview of these advances. The review begins by outlining the historical background of AIH, dating back to its initial recognition in the 1940s, and highlights the evolution of diagnostic criteria and classification based on autoantibody profiles. The epidemiology of AIH is explored, discussing its varying prevalence across different regions and the role of genetic predisposition, viral infections, and drug exposure as risk factors. Furthermore, the review delves into the pathogenesis of AIH, focusing on the dysregulated immune response, involvement of T cells, and potential contribution of the gut microbiome. Clinical presentation, diagnostic criteria, and liver biopsy as crucial tools for diagnosis are also discussed. Regarding management, the review provides an in-depth analysis of the standard first-line treatments involving glucocorticoids and azathioprine, as well as alternative therapies for non-responsive cases. Additionally, emerging second and third-line treatment options are examined. In conclusion, this narrative review highlights the complexity of AIH and underscores the importance of early diagnosis and individualized treatment approaches to improve patient outcomes. Further research and clinical trials are needed to optimize AIH management and ensure a better long-term prognosis for affected individuals.