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Effect of in utero hydroxychloroquine exposure on the development of cutaneous neonatal lupus erythematosus

Barsalou, Julie; Costedoat-Chalumeau, Nathalie; Berhanu, Adey; Fors-Nieves, Cesar; Shah, Ummara; Brown, Patrick; Laskin, Carl A; Morel, Nathalie; Levesque, Kateri; Buyon, Jill P; Silverman, Earl D; Izmirly, Peter M
OBJECTIVE:Cutaneous neonatal lupus (cNL) occurs in possibly 5%-16% of anti-Ro±anti-La antibody-exposed infants. Data suggest in utero exposure to hydroxychloroquine (HCQ) may prevent cardiac NL. The aim was to assess whether in utero exposure to HCQ decreases the risk of cNL and/or delays onset. METHODS:A multicentre case-control study was performed with 122 cNL cases and 434 controls born to women with a rheumatological disease who had documentation of maternal anti-Ro±anti-La antibodies at pregnancy and confirmation of medication use and the child's outcome. A secondary analysis was performed on 262 cNL cases, irrespective of maternal diagnosis, to determine if HCQ delayed time to cNL onset. RESULTS:Twenty (16%) cNL cases were exposed to HCQ compared with 146 (34%) controls (OR 0.4 (95% CI 0.2 to 0.6); p<0.01). Exposure to HCQ was associated with a reduced risk of cNL; exposure to anti-La antibody and female gender were associated with an increased risk of cNL. Exposure to HCQ remained significantly associated with a reduced cNL risk in the analyses limited to mothers with systemic lupus erythematosus and those who developed rash ≤1 month. When analysing all 262 cNL cases, HCQ-exposed infants were older (6.0 (95% CI 5.7 to 6.3) weeks) at cNL onset versus HCQ-non-exposed infants (4.4 (95% CI 3.9 to 5.0) weeks), but the difference was not statistically significant (p=0.21). CONCLUSION/CONCLUSIONS:Exposure to HCQ was associated with a reduced risk of cNL. Among cNL cases, those exposed to HCQ tend to have later onset of rash. Both findings suggest a protective effect of HCQ on cNL.
PMID: 30297329
ISSN: 1468-2060
CID: 3334702

Mortality in Systemic Lupus Erythematosus: an Updated Review

Fors Nieves, Cesar E; Izmirly, Peter M
Both systemic lupus erythematosus (SLE) and its treatments can contribute to increased mortality rates. The focus of this review is recent studies on mortality and comorbidities during the last 5 years from around the world. The authors conducted a literature review, using PUBMED, for articles relating to SLE mortality with a specific focus on literature published within the last 5 years. Our analysis found that while mortality in SLE patients continues to improve, there are differences in survival based on ethnicity, socioeconomic status, age, and gender. The most common cause of mortality is cardiovascular disease, followed closely by infection and severe disease activity. To conclude, while there have been significant advances in the treatment of SLE and its associated comorbidities, increased mortality remains a major concern in patient management.
PMID: 26984805
ISSN: 1534-6307
CID: 2030522

Impact of in Utero Hydroxychloroquine Exposure on the Risk of Cutaneous Neonatal Lupus Erythematosus [Meeting Abstract]

Barsalou, Julie; Costedoat-Chalumeau, Nathalie; Berhanu, Adey; Fors-Nieves, Cesar; Shah, Ummara; Brown, Patrick; Laskin, Carl; Morel, Nathalie; Levesque, Kateri; Buyon, Jill P.; Silverman, Earl; Izmirly, Peter M.
ISSN: 2326-5191
CID: 3726162

Rapid aneurysm growth and rupture in systemic lupus erythematosus

Graffeo, Christopher S; Tanweer, Omar; Nieves, Cesar Fors; Belmont, H Michael; Izmirly, Peter M; Becske, Tibor; Huang, Paul P
BACKGROUND: Subarachnoid hemorrhage (SAH) due to intracranial aneurysm rupture is a major neurosurgical emergency associated with significant morbidity and mortality. Rapid aneurysm growth is associated with rupture. Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder whose complications can include cerebral vasculitis and vasculopathy. Intracranial aneurysms are not known to occur more frequently in SLE patients than the general population; however, aneurysm growth rates have not been studied in SLE. CASE DESCRIPTION: We present a 43-year-old female with SLE on prednisone, hydroxychloroquine, and azathioprine with moderate disease activity who presented with severe, acute-onset headache and was found to have Hunt and Hess grade II SAH due to rupture of an 8 mm saccular anterior communicating artery (ACoA) aneurysm. The patient developed severe vasospasm, re-ruptured, and was taken for angiography and embolization, which was challenging due to a high degree of vasospasm and arterial stenosis. Review of imaging from less than 2 years prior demonstrated a normal ACoA complex without evidence of an aneurysm. CONCLUSION: We review the literature and discuss the risk factors and pathophysiology of rapid aneurysm growth and rupture, as well as the pathologic vascular changes associated with SLE. Although SLE patients do not develop intracranial aneurysm at an increased rate, these changes may predispose them to higher incidence of growth and rupture. This possibility-coupled with increased morbidity and mortality of SAH in SLE-suggests that SAH should be considered in SLE patients presenting with headache, and advocates for more aggressive treatment of SLE patients with unruptured aneurysms.
PMID: 25657862
ISSN: 2152-7806
CID: 1456852

Impact of in Utero Hydroxychloroquine Exposure on Age of Onset of Cutaneous Neonatal Lupus [Meeting Abstract]

Barsalou, Julie; Costedoat-Chalumeau, Nathalie; Fors-Nieves, Cesar; Shah, Ummara; Brown, Patrick; Laskin, Carl; Morel, Nathalie; Levesque, Kateri; Buyon, Jill P; Silverman, Earl; Izmirly, Peter M
ISSN: 2326-5205
CID: 2029732