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. 2020 Aug 28;15(1):225.
doi: 10.1186/s13023-020-01510-9.

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

Cai Yue  1 , Jian Su  2 , Xiaohong Fan  1 , Li Song  3 , Wei Jiang  4 , Jinghua Xia  1 , Tao Shi  1 , Xuan Zhang  5   6 , Xuemei Li  7
Affiliations

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

Cai Yue et al. Orphanet J Rare Dis. .

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).

Conclusions: Renal involvement was less severe in patients with SLE-TTP than in patients with primary iTTP. The treatment responses and outcomes of SLE-TTP were no worse and perhaps even better than those of primary iTTP. When TTP is diagnosed in SLE patients, the ADAMTS13 level and ADAMTS13 inhibitor profile should be considered in addition to clinical features.

Keywords: ADAMTS13 protein; Acquired thrombotic thrombocytopenic purpura; Systemic lupus erythematosus; Thrombotic microangiopathies.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Flow chart of patient selection. Abbreviations: iTTP, immune-mediated TTP; UCTD, undifferentiated connective tissue disease; SLE, systemic erythematosus; SLE-TTP, SLE-associated TTP; TTP, thrombotic thrombocytopenic purpura
Fig. 2
Fig. 2
Outcomes of patients with immune-mediated thrombotic thrombocytopenic purpura. a. Cumulative incidence of a clinical response. b. Cumulative incidence of clinical remission. c. Cumulative incidence of survival. iTTP, immune-mediated TTP; SLE, systemic lupus erythematosus; TTP, thrombotic thrombocytopenic purpura
See this image and copyright information in PMC

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