Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus

Abstract

Thrombotic thrombocytopenic purpura (TTP) occurring in patients with systemic lupus erythematosus (SLE) is rare and can be difficult to diagnose because of overlapping features of the two disorders. The aim of this study is to further characterize this uncommon association in terms of presenting features, diagnostic difficulties and treatment outcome. This is the largest series from a single centre with 6 patients diagnosed over a 6-year period. Two thirds of the patients had a simultaneous diagnosis of TTP and SLE. Half of the patients had a positive Coombs test along with clear features of TTP. Five patients received plasmapheresis as initial treatment while 1 patient received plasma infusions only. Four out of 5 patients responded to plasmapheresis and only 1 patient required cytotoxic therapy. TTP in association with SLE appears to be underdiagnosed and a positive Coombs test is not against the diagnosis of TTP in this setting. Most of the patients respond well to plasmapheresis. In case of a poor response, cytotoxic drugs should be considered early.

Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcomeof 24 patients treated at a Tertiary Care Center in Saudi Arabia

“…Few small reports have been published about Saudi patients with TTP until now 9-11. In the present study, we analyzed the clinical and laboratory characteristics, complications, therapy and outcome of 24 TTP patients treated at our large tertiary care center.…”

Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study

“…The diagnosis of TTP in patients with SLE might be challenging in clinical field because both disorders share many overlapping clinical features, 7,8 and delayed diagnosis of TTP might cause fatal results. The majority of previous investigations regarding TTP developing in patients with SLE have been case reports, 2,[9][10][11][12][13][14][15] and thus the clinical characteristics and risk factors and risk factor for mortality in these patients have not been defined clearly.…”

Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy

“…Up to 100 isolated cases of SLE/TMA association were reported in the literature [1,2,[8][9][10][11][12][13][14][15][16][17][18][19][20]. However, an exact frequency cannot be calculated because patients with SLE and HUS/TTP share very similar clinical features [2,21].…”