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.

Lupus podocytopathy superimposed on diabetic glomerulosclerosis

“…[12] Subsequent case reports on LP revealed variable presentations of LP with concurrent extra renal or serologic activities, before or after clinical SLE diagnosis, casting challenges on the LP diagnosis. For example, LP has been reported as a co-current presentation with chylous ascites as the manifestation of SLE in a 3 year old girl, [13] podocytopathy presenting as TTP-like syndrome in a 45 year old woman with subsequently diagnosed SLE, [14] or collapsing podocytopathy presenting with heamophagocytic lymphohisocytosis as the manifestation of SLE. [15] In addition, hydroxychloroquine has also been implicated to induce podocytopathy in Treatment timelines and treatment effects on proteinuria and renal function.…”

Unusual association of lupus and thrombotic thrombocytopenic purpura like syndrome: clinical experience of a rare presentation

“…SLE-associated TTP-like MAHA is a condition characterized by an unexplained MAHA in a patient who fulfilled the ACR criteria for SLE; this condition may be associated with the other classic findings of TTP as renal impairment, fever, and neurological symptoms without a severe reduction in ADAMTS13 activity, or diarrhea to differentiate it from hemolytic uremic syndrome [13] . TTP should be kept in mind when dealing with SLE patient presented with MAHA as both conditions can occur concomitantly and can share similar manifestations [14] .…”