Left main coronary artery thrombosis complicating an idiopathic retroperitoneal fibrosis in a 37-year-old man with long-standing type I diabetes mellitus. | LitMetric
Retroperitoneal fibrosis is an uncommon collagen vascular disease of unknown etiology, characterized by the replacement of normal retroperitoneal tissue with fibrosis and/or chronic inflammation usually surrounding the abdominal aorta and the iliac arteries and extending into adjacent anatomic structures. No cases of acute coronary syndrome in the setting of retroperitoneal disorder have been published as yet. We report a 37-year-old man with a 14-year history of type I diabetes mellitus who was admitted to the endocrinology department for a routine check up and glycemic re-equilibration and who was later diagnosed to have an idiopathic retroperitoneal fibrosis. The patient presented during his hospitalisation with a non ST elevation myocardial infarction caused by an isolated thrombus located inside the left main coronary artery successfully treated with manual thrombectomy.
Objective: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated.
Methods: A total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010.
Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objectives: Idiopathic retroperitoneal fibrosis (IRF) is a rare autoimmune-mediated condition characterized by fibro-inflammatory tissue development around the abdominal aorta and iliac arteries. Ureteral entrapment and hydronephrosis are the most common manifestations and acute renal failure or chronic renal insufficiency may occur as the consequence. Glucocorticoids and immunosuppressants may be effective but the therapeutic response and outcome are heterogeneous and hard to predict.
The patient was a 57-year-old man who developed bilateral thigh pain and chest tightness one year ago. Chest CT scan showed reticular shadows, thickened interlobular septa in both lung fields, and pericardial effusion. Three months ago, his symptoms worsened.
Department of Renal and Transplant Medicine, West London Renal and Transplant Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
Objectives: To determine the clinical outcomes of patients with immunoglobulin 4-related disease (IgG4-RD) treated with a defined B cell depletion protocol using rituximab.
Methods: Patients were included if they had (1) an IgG4-RD diagnosis at Imperial College Healthcare NHS Trust between February 2017 and October 2022, and (2) >9 months of follow-up data available following the first rituximab dose. The rituximab protocol targeted B cell depletion to < 10 cells/microliter for a maintenance period of two years.
Division of Hematology, University of British Columbia, Vancouver, British Columbia, Canada; and Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.
