Proteinase 3 (PR3) anti-neutrophil cytoplasmic antibodies (ANCA) and anti-double-stranded DNA (anti-dsDNA) antibodies have been associated with a variety of nephritic diseases, most recognizably granulomatosis with polyangiitis and systemic lupus erythematosus (SLE) glomerulonephritis, respectively. We report the first clinical case of positive PR3 and dsDNA in a patient with renal Immunoglobin light chain (AL) amyloidosis. A 75-year-old man presented to the hospital with chronic fatigue, weight loss, and a recent diagnosis of left ventricular infiltrative cardiomyopathy secondary to AL amyloidosis. Autoimmune serology was significant for PR3-ANCA and anti-dsDNA antibodies. A renal biopsy confirmed AL amyloidosis with diffuse Congo red stain. This case report is the first of its kind, showing atypical antibody presentation in the setting of amyloidosis.
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| http://dx.doi.org/10.7759/cureus.47667 | DOI Listing |
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