Kidney-limited thrombotic microangiopathy in patients with SLE treated with romiplostim

Tomov, SV; Lazarchick, John; Self, SE; Bruner, ET; Budisavljevic, MN

doi:10.1177/0961203313477900

Cited by 12 publications

(15 citation statements)

References 35 publications

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“…She responded to romiplostim, which enabled her to deliver safely at 34 weeks of gestation 7 . Of note, the third patient presented renal thrombotic microangiopathy shortly after treatment with romiplostim 8 . In fact, venous and arterial thrombotic events have been described in patients treated with romiplostim.…”

Section: Rheumatologymentioning

confidence: 93%

“…To date, only 4 cases of refractory SLE-associated thrombocytopenia successfully treated with thrombopoietin-receptor agonists have been reported 6,7,8,9 (Table 1). One patient presented thrombocytopenia in the context of Evans syndrome 6 and a pregnant woman with SLE had at 27 weeks of gestation thrombocytopenia refractory to corticosteroids, IVIG, rituximab, IV cyclophosphamide, and eltrombopag.…”

Section: Rheumatologymentioning

confidence: 99%

“…To date, there are only 4 published cases of patients with SLE and immune thrombocytopenia successfully treated with these agents 6,7,8,9 and 1 case in whom this treatment was not effective 10 . We describe 2 additional cases of patients with SLE and refractory immune thrombocytopenia who responded to thrombopoietin-receptor agonists.…”

Section: To the Editormentioning

confidence: 99%

See 2 more Smart Citations

Effectiveness of Thrombopoietin-receptor Agonists in the Treatment of Refractory Immune Thrombocytopenia Associated to Systemic Lupus Erythematosus

Magnano¹,

Enríquez²,

Esteve³

et al. 2014

J Rheumatol

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Section: Rheumatologymentioning

confidence: 93%

Section: Rheumatologymentioning

confidence: 99%

See 1 more Smart Citation

Effectiveness of Thrombopoietin-receptor Agonists in the Treatment of Refractory Immune Thrombocytopenia Associated to Systemic Lupus Erythematosus

Magnano¹,

Enríquez²,

Esteve³

et al. 2014

J Rheumatol

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“…Localized microangiopathy to be defined histologically is, in this sense, similar to kidney-limited thrombotic microangiopathy. [ 21 , 22 ] It should be discriminated from systemic thrombotic microangiopathy characterized by microangiopathic hemolytic anemia and thrombotic thrombocytopenia [ 23 ] ; however, microangiopathic hemolytic anemia and thrombotic thrombocytopenia in the patients with extremely sever PAH were previously described in the literatures. [ 4 , 5 ]…”

Section: Discussionmentioning

confidence: 99%

Decreased haptoglobin levels inversely correlated with pulmonary artery pressure in patients with pulmonary arterial hypertension

Nakamura

Kato²,

Nakaya³

et al. 2017

Medicine

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We investigated the serum haptoglobin levels in patients with pulmonary arterial hypertension (PAH) based on the hypothesis that haptoglobin levels would reflect subclinical hemolysis due to microangiopathy in pulmonary arterioles.This cross-sectional study included 3 groups of patients attending Hokkaido University Hospital: PAH, chronic thromboembolic pulmonary hypertension (CTEPH), and connective tissue diseases (CTD) without PAH (CTD-non-PAH) group. Serum haptoglobin levels were measured by standardized turbidimetric immunoassay in all patients. Demographic data, laboratory results, right heart catheter, and echocardiographic findings were extracted from the medical records. Decreased haptoglobin levels were defined as below 19 mg/dL based on the 95th percentile of healthy controls.Thirty-five patients in PAH group including 11 with idiopathic PAH (IPAH) and 24 with CTD-associated PAH (CTD-PAH), 27 in CTEPH group, and 32 in CTD-non-PAH group were analyzed. Serum haptoglobin levels in PAH group (median 66 mg/dL) were significantly lower than those in CTEPH group (median 94 mg/dL, P = .03) and CTD-non-PAH group (median 79 mg/dL, P = .03). The prevalence of decreased haptoglobin levels was 26% in PAH group, 15% in CTEPH group, and 6% in CTD-non-PAH group. Serum haptoglobin levels had a significant negative correlation (r = −0.66, P < .001) with mean pulmonary artery pressure in PAH group, particularly in CTD-PAH subgroup (r = −0.74, P < .001), but no correlation in IPAH subgroup (r = −0.52, P = .13) and in CTEPH group (r = −0.17, P = .41). Follow-up cases of CTD-PAH showed lowering pulmonary artery pressure led to normalizing serum haptoglobin levels.Serum haptoglobin levels decreased in PAH patients and inversely correlated with pulmonary artery pressure in CTD-PAH patients, suggesting their potential as a surrogate marker for CTD-PAH.

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“…76 Contrasting to these results a young SLE patient developed thrombotic microangiopathy with renal failure after romiplostim therapy. 77 Maroun et al 78 reported on three patients with SLE thrombocytopenia in which administration of eltrombopag was successful as steroid-sparing treatment. Another patient with refractory thrombocytopenia achieved a complete response after eltrombopag treatment.…”

Section: Thrombopoietin Receptor Agonistsmentioning

confidence: 99%

Lupus thrombocytopenia: pathogenesis and therapeutic implications

Galanopoulos¹,

Christoforidou²,

Bezirgiannidou³

2017

MJR

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disorder that is frequently complicated by hematological manifestations such as hemolytic anemia, leukopenia and thrombocytopenia. 1,2 These disorders are included in the diagnostic criteria of SLE both in the earlier (1982), as well as in the revised criteria of the American College of Rheumatology (2003), and the 2012 Systemic Lupus International Collaborating Clinics (SLCC) criteria. 3-5 Thrombocytopenia (<100x10 9 /L) has been reported in 20% to 40% of patients with SLE 2,6-8 and is usually attributed to an autoimmune mechanism similar to that of idiopathic immune thrombocytopenia (ITP). It may be the first manifestation of lupus in up to 16% of patients, presenting months or as early as 10 years before diagnosis. 9,10 It can also be a complication of therapeutic agents such as azathioprine, methotrexate and rarely hydroxychloroquine. Severe thrombocytopenia (<20 to 50x10 9 /L according to study definition) is relatively rare, occurring in about 3-10% of patients. 11,12 Occasionally, thrombo-Cite this article as: Galanopoulos N, Christoforidou A, Bezirgiannidou Z. Lupus thrombocytopenia: pathogenesis and therapeutic implications.

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Kidney-limited thrombotic microangiopathy in patients with SLE treated with romiplostim

Cited by 12 publications

References 35 publications

Effectiveness of Thrombopoietin-receptor Agonists in the Treatment of Refractory Immune Thrombocytopenia Associated to Systemic Lupus Erythematosus

Effectiveness of Thrombopoietin-receptor Agonists in the Treatment of Refractory Immune Thrombocytopenia Associated to Systemic Lupus Erythematosus

Decreased haptoglobin levels inversely correlated with pulmonary artery pressure in patients with pulmonary arterial hypertension

Lupus thrombocytopenia: pathogenesis and therapeutic implications

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