The journal of the Royal College of Physicians of Edinburgh
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J R Coll Physicians Edinb · Jan 2014
Case ReportsA masquerading mass: an unusual presentation of IgG4-related systemic disease with tubulointerstitial nephritis.
IgG4 tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4 renal disease. When IgG4-TIN is accompanied by other systemic manifestations the disease is known as IgG4-related systemic disease (IgG4-RSD). ⋯ This case highlights the importance of recognising IgG4-RSD as a non-malignant disease with presentations having commonly shared features including tumour-like swelling of involved organs and its ability to mimic many systemic diseases. In the majority of patients it can be treated successfully with corticosteroids.
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J R Coll Physicians Edinb · Jan 2014
Case ReportsPulmonary hypertension and hepatic encephalopathy: lethal complications of Rendu-Osler-Weber disease.
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterised by epistaxis, mucocutaneous telangiectasia with systemic manifestations due to visceral telangiectasia and arterio-venous malformations (AVMs). We describe unusual complications of HHT in a 68-year-old male who developed high-output cardiac failure with pulmonary hypertension in combination with hepatic encephalopathy due to hepatic AVMs. This case shows the importance of a multi-system approach to HHT and considers the treatment of its hepatic complications.