Background: IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition classically causing retroperitoneal fibrosis, aortitis, thyroiditis, or pancreatitis. Diagnosis includes the presence of lymphoplasmacytic infiltrate (with >40% ratio IgG4+:IgG plasma cells) and fibrosis. Cardiac involvement may include aortic, pericardial, or coronary disease. Coronary manifestations encompass obstructive intra-luminal lesions, external encasing pseudo-tumour on imaging, or lymphoplasmacytic arteritis.
Case Summary: : A fit and healthy 50-year-old man was found deceased. His only known medical condition was treated Hashimoto's thyroiditis. Post-mortem examination demonstrated an isolated severe stenosis of the left anterior descending (LAD) coronary artery without histopathological evidence of acute myocardial infarction. Coronary plaque histopathology showed florid IgG4-positive plasma cell infiltrate throughout all layers of the artery with dense fibrous tissue connective tissue stroma, all features consistent with coronary artery IgG4-RD. : A 48-year-old man collapsed at work. Computed tomography scan 1 week prior reported an ill-defined para-aortic retroperitoneal soft tissue density. No cardiac symptoms were reported in life. Post-mortem examination showed coronary arteritis and peri-arteritis with sclerosing peri-aortitis in the LAD. There was myocardial fibrosis of the anterior left ventricle and focal myocarditis of the right ventricle.
Discussion: IgG4-related disease presenting as sudden cardiac death without any preceding symptoms is very rare (six prior cases identified on literature review). Reported targeted successful interventions for intracoronary IgG4-RD diagnosed in life have included steroid therapy and B cell depleting therapy (i.e. rituximab). If cardiac symptoms are present in a patient with known IgG4-RD, cardiac investigations should be promptly arranged.
A 72-year-old male patient presented fatigue, anemia, elevated total protein, IgG, IgG4, IL-6, and vascular endothelial growth factor (VEGF) levels. Initial diagnostics suspected multiple myeloma. A plane computed tomography (CT) scan showed pneumonia and the enlargement of generalized lymph nodes.
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.
Objective: About 40% of IgG4-related disease (IgG4-RD) patients face recurrence, severely impacting their quality of life. We aimed to explore the characteristics of the perception of recurrence risk in patients with IgG4-RD.
Methods: A qualitative study design with a descriptive phenomenological approach was used.
BACKGROUND IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease potentially affecting every part of the human body. Because of variability in clinical presentation, IgG4-RD can be challenging to diagnose. Untreated disease can lead to irreversible organ damage such as fibrosis.
