Amyloidosis proteinuria

Nephrotic syndrome may complicate the course of many primary and secondary glomerulopathies. Diabetic nephropathy is the most common cause of nephrotic proteinuria (not always accompanied by full-blown nephrotic syndrome). Lupus nephritis and renal amyloidosis are much rarer secondary glomerulopathies resulting in nephrotic syndrome. The prevalence of primary glomerulopathies differs between Blacks and Whites (focal segmental glomerulosclerosis is more common... 

A 63-year-old woman with rheumatoid arthritis was given intramuscular gold sodium thiomalate and began to have nausea, vomiting, anorexia, and watery diarrhea (71). A year later the watery diarrhea became more frequent (more than 10 times within a day) and she developed proteinuria. Biopsies from the stomach, duodenum, and kidney showed systemic amyloidosis. This was a rare case of secondary systemic amyloidosis associated with rheumatoid arthritis. It is not clear from the report what the role of gold was in this case. 

Where heart failure due to amyloidosis has already set in, we have had no success. In one patient macroglossia became less edematous, and caliper measurements confirmed that a previous width increase (of 1 cm each 4 months for 8 months) was arrested for 2 more comfortable years by the use of melphalan, before the reappearance of Bence Jones proteinuria and further width increase heralded relapse and death. 

IgG (53% myelomatosis) patients present on average with a serum level of 4.3 g/100 ml (because of the longer T, see Section 2.1). They have the most severe immune paresis, so that infection requiring hospitalization is common (60% within three years) but less hypercalcemia (33%), renal failure (16%), Bence Jones proteinuria (60%), and amyloidosis. Apparent hyponatremia (8%, see Section 7.5.3), viscosity syndrome (4%, see Section 7.5.6), and cryoglobulinemia (4%, see Section 7.5.5) with myelomatosis relate to IgG paraproteins. 

Nephrotic syndrome is characterized by proteinuria greater than 3.5 g/day per 1.73 m, hypoproteinemia, edema, and hyperlipidemia. A hypercoagulable state may also be present in some patients. The syndrome may be the result of primary diseases of the glomerulus, or be associated with systemic diseases such as diabetes mellitus, lupus, amyloidosis, and preeclampsia. Hypoproteinemia, especially hypoal-buminemia, results from increased urinary loss of albumin and an increased rate of catabolism of filtered albumin by proximal tubular cells. The compensatory increase in hepatic synthesis of albumin is insufficient to replenish the protein loss, probably because of malnutrition. 

In the clinical trials, it was found that colchicine prevents febrile attacks in 60 % of patients and significantly reduces the number of attacks in another 20-30 % [37]. In familial Mediterranean fever patients, death occurs mainly due to amyloidosis. However, treatment with colchicine greatly altered the prognosis by arresting amyloidosis and reversing proteinuria [38]. 

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