Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study

Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is associated with more deleterious outcomes in patients with systemic lupus erythematosus (SLE). However, ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels and ADAMTS13 inhibitor were not routinely assayed in most previous studies. The objective of this study is to compare the characteristics and outcomes of immune-mediated TTP (iTTP) in patients with and without SLE. Methods: The medical data of 28 patients with iTTP from Peking Union Medical College Hospital were analysed. ADAMTS13 activity and ADAMTS13 inhibitor were measured in all patients. Results: All 28 patients had ADAMTS13 inhibitor and severe ADAMTS13 deficiency. iTTP was considered SLE-related (SLE-TTP) in 10 patients and primary (primary iTTP) in 18 patients. Renal involvement on presentation was more severe in patients with primary iTTP as determined by higher serum creatinine (162.7 ± 110.6 vs 73.3 ± 13.4 μmol/L, p < 0.01) and more prevalent acute kidney injury (72.2% vs 10.0%, p < 0.01) than in patients with SLE-TTP. More patients with SLE-TTP were treated with steroid pulse therapy (90.0% vs 16.7%, p < 0.01) and intravenous immunoglobulin (IVIG) (50.0% vs 5.6%, p = 0.01) compared to patients with primary iTTP. After adjustments for age and treatment, including steroid pulse therapy and IVIG treatment, the likelihood of clinical remission of SLE-TTP was significantly increased compared to that of primary iTTP (HR 7.6 [1.2, 50.1], p = 0.03). Mortality was also lower among patients with SLE-TTP than among patients with primary iTTP (0 vs 38.9%, p = 0.03).

The Efficacy and Safety of Plasma Exchange in the Treatment of Thrombotic Thrombocytopenic Purpura

“…Among them, 6 cases were secondary to SLE, accounting for 85.71% of secondary TTP. SLE is an autoimmune disease formed by antibodies and immune complexes that mediate organ tissue damage [ 15 ]. SLE is more common in women, accounting for 90% [ 16 ].…”

Successful treatment of thrombotic thrombocytopenic purpura with plasmapheresis and anti-CD20 antibodies in a patient with immune thrombocytopenia and systemic lupus erythematosus

“…In a comparison of outcomes of immune-mediated TTP between patients with SLE (n = 8) and those without TTP (n = 10), mortality rates were higher, with a longer duration of treatment before remission, in patients with SLE compared in patients without SLE [16] . However, another study showed the patients with primary TTP (n = 18) had more severe renal involvement compared to patients with SLE-related TTP (n = 10); clinical remission was more frequent and mortality rates were lower in patients with SLE (n = 18) [17] . Although it is difficult to draw firm conclusions due to the small number of study populations, it does not seem to be the case that SLE-related TTP has a worse prognosis than primary TTP.…”

“… [16] However, another study showed the patients with primary TTP (n = 18) had more severe renal involvement compared to patients with SLE-related TTP (n = 10); clinical remission was more frequent and mortality rates were lower in patients with SLE (n = 18). [17] Although it is difficult to draw firm conclusions due to the small number of study populations, it does not seem to be the case that SLE-related TTP has a worse prognosis than primary TTP.…”

Immune-Mediated Hematological Disorders as Uncommon Presentation Forms of Systemic Lupus Erythematosus

“…The fifth patient was a young female who presented a typical microangiopathic hemolytic anemia [15] with low ADAMTS13 activity whose diagnosis was acquired TTP. Such a rare disease results from autoantibodies against ADAMTS13, a von Willebrandcleaving protease, leading to formation of ultra-large von Willebrand factor multimers with unregulated platelet adhesion and aggregation, disseminated microthrombi, organ ischemia, and high erythrocyte shearing rates [9,15,31,32]. SLE was diagnosed sequentially.…”

“…SLE was diagnosed sequentially. It is known that acquired TTP can be secondary to other disorders including autoimmune diseases, notably SLE [31]. Actually, SLE can be diagnosed before or concurrently with TTP diagnosis, or even following its recovery [33].…”