ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide.
Amyloidosis is a collective term for a debilitating and progressive disease characterized by the extracellular deposition of abnormal proteins. These abnormal proteins can either be deposited in a localized place, such as a single organ, or, as is the case with our patient, deposited throughout the entire body.Unlike our patient, who was in his early 30s, the average age of patients diagnosed with AL amyloidosis is 65 years, and <10% of patients are younger than 50 years. 1 Although bleeding disorders are associated with AL amyloidosis, our patient had coagulopathy caused by amyloidosis-induced acquired factor X deficiency, which is remarkably rare. In addition, our patient previously had idiopathic pancreatitis diagnosed, which was also eventually attributed to significant amyloidosis affecting the organ, based on autopsy
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