Pure lupus podocytopathy first presenting as thrombotic thrombocytopenic purpura-like syndrome.

ABSTRACT

Lupus podocytopathy (LP) is an uncommon proteinuric disorder in the spectrum of lupus nephropathy. Its histological features are similar to those described in minimal change disease (MCD) with or without mesangial immune deposits. Although infrequent, a close relationship between systemic lupus erythematosus (SLE) and thrombotic thrombocytopenic purpura (TTP) is well accepted. Proteinuria in the setting of SLE has previously been associated with the development of TTP-like syndrome. As far as we know, LP first presenting as a TTP-like syndrome has never been reported. Here, we describe the case of a previously healthy 45-year-old woman who developed simultaneously these two conditions and then we briefly review the literature on the topic, emphasizing the previous cases of concurrent initial diagnosis of both SLE and MCD (n = 7) and SLE and TTP (n = 72). In conclusion, renal biopsy is central to the management of SLE patients with nephrotic syndrome. Furthermore, in a SLE patient with anemia and thrombocytopenia, TTP should be part of the differential diagnosis, even when no schistocytes were detected in peripheral blood smear.