A 41-YEAR-OLD WOMAN WITH PROTEIN-S DEFICIENCY AND DIFFUSE PROLIFERATIVE LUPUS NEPHRITIS - IS PROTEIN-S DEFICIENCY ASSOCIATED WITH A HYPERINFLAMMATORY RESPONSE

A 41-year-old woman with complete protein S (PS) deficiency who develo ped diffuse proliferative lupus nephritis is reported, She was referre d to our hospital with nephrotic syndrome and thrombocytopenia, Her me dical history included colorectostomy and amputation of the extremitie s because of repeated thrombotic episodes during her teens without any evidence of systemic lupus erythematosus, The diagnosis of PS deficie ncy was made from the patient's clinical course, undetectable serum PS in either the active or inactive form, normal protein C activity, and no evidence of the antiphospholipid syndrome, However, there was no d efinitive family history, A depressed level of complements and a posit ive antinuclear acid antibody suggested a diagnosis of systemic lupus erythematosus, The patient had a rapidly progressive course and died o f disseminated intravascular coagulation. An autopsy showed generalize d thrombotic lesions and diffuse proliferative lupus nephritis on both ordinal light and immunoperoxidase microscopy. Our observations sugge st that PS-deficient patients may have a hyperinflammatory response. ( C) 1997 by the National Kidney Foundation, Inc.