Publications by authors named "Gaelle Guettrot-Imbert"

17 Publications

  • Page 1 of 1

Evaluation of the severe preeclampsia classification criterion for antiphospholipid syndrome in a study of 40 patients.

Arthritis Res Ther 2021 05 4;23(1):134. Epub 2021 May 4.

AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'Ile de France, Paris, France.

Background: The criteria for antiphospholipid syndrome (APS) include severe preeclampsia and/or placental insufficiency leading to preterm delivery before 34 weeks of gestation, but this APS manifestation has been rarely studied. Thus, we report a series of severe preeclampsia occurred in patients with APS.

Methods: We retrospectively analysed data of women with APS (Sydney criteria) who experienced severe preeclampsia with delivery before 34 weeks' gestation between 2000 and 2017 at five French internal medicine departments and one Italian rheumatology unit.

Results: The 40 women had a mean age of 30.5 ± 4.6 years at their first episode of preeclampsia; 21 were nulligravid (52.5%), 12 (30%) had already been diagnosed with APS, and 21 (52.5%) had a triple-positive antiphospholipid (aPL) antibody test. Preeclampsia occurred at a median gestational age of 25.5 weeks (IQR 23-29). It was associated with HELLP in 18 cases (45%), eclampsia in 6 (15%), placental abruption in 3 (7.5%), catastrophic APS in 3 (7.5%), and foetal and neonatal death in 11 and 15 cases. Overall, 14 (35%) children survived, born at a median gestational age of 31 weeks. Among other APS criteria, 16 women (40%) experienced at least one thrombosis, 17 (42.5%) an intrauterine foetal death, and 19 (47.5%) at least one episode of HELLP during follow-up (median 5 years, IQR = 2-8). None had three or more consecutive miscarriages. Notably, 12 women (30%) had systemic lupus erythematosus.

Conclusions: Severe preeclampsia led to high mortality in the offspring. Almost half of these women experienced other APS features, but not three consecutive miscarriages.
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http://dx.doi.org/10.1186/s13075-021-02518-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8094564PMC
May 2021

SLE-DAS in the First Trimester of Gestation Predicts Maternal Lupus Flares Later in Pregnancy.

Front Pharmacol 2021 16;12:660123. Epub 2021 Apr 16.

Rheumatology Unit, Department of Medicine, DIMED, University of Padova, Padova, Italy.

Systemic Lupus Erythematosus (SLE) mainly occurs during childbearing age. Remission or low disease activity state (LDAS) before conception are recommended by experts to achieve a favourable lupus pregnancy outcome but little is known on the best way to evaluate remission or activity status during pregnancy. We tested SLE-disease activity score (SLE-DAS) in the first trimester as predictor of maternal flares and obstetrical complications in 2nd and 3rd trimester in a cohort of SLE pregnant women. Inclusion criteria were: 1) women ≥ 18 years; 2) affected with SLE (SLICC 2012); 3) enrolled in two referral centers (Italy and France) 4) with an ongoing singleton pregnancy at 12 weeks (only one pregnancy per patient). Disease activity was assessed at first trimester of pregnancy, using SLE-pregnancy disease activity index (SLEPDAI) and retrospectively applying SLE-DAS. Maternal lupus flares at 2nd and 3rd trimester were defined by the SELENA-SLEDAI Flare Index (SFI). Adverse pregnancy outcome (APO) included: fetal and neonatal death, placental insufficiency with premature delivery <37 weeks, and small for gestational age (SGA) (≤3rd percentile). We included 158 pregnant patients affected with SLE. At first trimester the median SLEPDAI (IQR) was 2 (0-4) and the median SLE-DAS (IQR) 1.32 (0.37-2.08). At least one flare occurred in 25 (15.8%) women during the 2nd and 3rd trimester. APO occurred in 19 (12.0%) patients. A significant correlation between SLE-DAS and SLEPDAI was found in this cohort (Spearman's = 0.97, Figure 1). At multivariate analysis, both SLE-DAS and SLEPDAI predicted maternal flares (adjOR = 1.2; 95% CI = 1.0-1.3, = 0.02; adjOR 1.3, 95% CI = 1.1-1.6 per unit increase, = 0.01, respectively). SLE-DAS and SLEPDAI were associated with APO at univariate analysis ( = 0.02). SLE-DAS was highly correlated with SLEPDAI and its use in the first trimester predicted maternal flares in the 2nd and 3rd trimester, making SLE-DAS a reliable instrument to measure SLE activity during pregnancy.
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http://dx.doi.org/10.3389/fphar.2021.660123DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8085518PMC
April 2021

Risk factors for hydroxychloroquine retinopathy in systemic lupus erythematosus: a case-control study with hydroxychloroquine blood-level analysis.

Rheumatology (Oxford) 2020 12;59(12):3807-3816

Department of Internal Medicine, Centre de Référence Maladies Auto-Immunes et Systémiques Rares d'Ile de France, Cochin Hospital, APHP.

Objective: HCQ is an essential medication in SLE, proven to lengthen survival and reduce flares. Its use, however, is limited by its rare but severe ophthalmological complications. Here, we aimed to analyse factors associated with HCQ retinopathy including HCQ blood levels.

Methods: This case-control study compared SLE patients with and without HCQ retinopathy, defined by abnormal results for at least two of the following ophthalmological tests: automated visual fields, spectral-domain optical coherence tomography (SD-OCT), multifocal electroretinogram (mfERG) and fundus autofluorescence. We compared clinical and laboratory findings to assess risk factors for HCQ retinopathy.

Results: The study included 23 patients with confirmed retinopathy (cases) and 547 controls. In the univariate analysis, age (P < 0.001), height (P = 0.045), creatinine clearance (P < 0.001), haemoglobin concentration (P = 0.01), duration of HCQ intake, (P < 0.001), higher cumulative HCQ dose (P < 0.001) and geographical origin (West Indies and sub-Saharan Africa) (P = 0.007) were associated with the risk of retinopathy, while HCQ blood levels were not. In the multivariate analysis, only cumulative dose (P = 0.016), duration of intake (P = 0.039), creatinine clearance (P = 0.002) and geographical origin (P < 0.0001, odds ratio 8.7) remained significantly associated with retinopathy.

Conclusion: SLE patients on HCQ should be closely monitored for retinopathy, especially those from the West Indies or sub-Saharan Africa, or with renal insufficiency, longer HCQ intake or a high cumulative dose. Although reducing the daily dose of HCQ in patients with persistently high HCQ blood levels seems logical, these concentrations were not associated with retinopathy in this study with controls adherent to treatment.
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http://dx.doi.org/10.1093/rheumatology/keaa157DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8186841PMC
December 2020

Intrauterine fetal deaths related to antiphospholipid syndrome: a descriptive study of 65 women.

Arthritis Res Ther 2018 Nov 6;20(1):249. Epub 2018 Nov 6.

AP-HP, Cochin Hospital, Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île de France, 27 Rue du Faubourg Saint Jacques, 75014, Paris, France.

Objective: Although one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD.

Methods: We retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016.

Results: The study included 65 women. Their median age at the index IUFD was 29 years (IQR 26-33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile. IUFD occurred at a median gestational age of 24 weeks (IQR 18-27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus. Overall, including during the follow-up period of 4 years (IQR 2-9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages.

Conclusion: IUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the "3 consecutive early miscarriages" criterion was met only once. With treatment, most of the women successfully had at least one live birth.
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http://dx.doi.org/10.1186/s13075-018-1745-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6235231PMC
November 2018

Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus.

Int J Cardiol 2017 Dec 7;248:263-269. Epub 2017 Aug 7.

AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France. Electronic address:

Background: Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear.

Methods: We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies.

Results: Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM.

Conclusion: Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.
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http://dx.doi.org/10.1016/j.ijcard.2017.07.100DOI Listing
December 2017

Fertilization in 37 Women with Systemic Lupus Erythematosus or Antiphospholipid Syndrome: A Series of 97 Procedures.

J Rheumatol 2017 05 15;44(5):613-618. Epub 2017 Jan 15.

From the Centre de compétence de maladies auto-immunes et systémiques rares, Service de médecine interne, Hôpital Robert Debré, Centre Hospitalier Universitaire (CHU), Reims Cedex; Service de médecine interne, CHU, Nantes; Université René Descartes Paris V, Centre de référence maladies auto-immunes et systémiques rares, Service de médecine interne, Hôpital Cochin, AP-HP; Service de gynécologie-obstétrique, Hôpital Cochin, AP-HP; Service de gynécologie-obstétrique, Groupe Hospitalier Pitié Salpêtrière, AP-HP; Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B); INSERM, UMR_S 959, F-75013; CNRS, FRE3632, F-75005; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Paris, France.

Objective: To compile and assess data about complication and success rates for fertilization (IVF) of women with systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). To date, such data are sparse.

Methods: This retrospective study described women with SLE and/or APS who have had at least 1 IVF cycle.

Results: Thirty-seven women with SLE (n = 23, including 8 with antiphospholipid antibodies), SLE with APS (n = 4), or primary APS (n = 10) underwent 97 IVF procedures. For 43% of cases, the infertility was female in origin, for 19% male, 14% mixed, and 24% unexplained. No women had premature ovarian insufficiency because of cyclophosphamide. Median age at IVF was 34 years (range 26-46). The median number of IVF cycles was 2.6 (1-8). Patients were treated with hydroxychloroquine (72%), steroids (70%), azathioprine (3%), aspirin (92%), and/or low molecular weight heparin (62%). There were 27 (28%) pregnancies, 23 live births among 26 neonates (3 twin pregnancies), 2 miscarriages, and 2 terminations for trisomy 13 and 21. Six spontaneous pregnancies occurred during the followup. Finally, 26 women (70%) delivered at least 1 healthy child. Complications occurred in or after 8 IVF cycles (8%): SLE flares in 4 (polyarthritis in 3 and lupus enteritis in 1) and thromboembolic events in 4 others. One SLE flare was the first sign of previously undiagnosed SLE. Poor treatment adherence was obvious in 2 other flares and 2 thromboses. No ovarian hyperstimulation syndrome was reported.

Conclusion: These preliminary results confirm that IVF can be safely and successfully performed in women with SLE and/or APS.
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http://dx.doi.org/10.3899/jrheum.160462DOI Listing
May 2017

Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome.

Autoimmun Rev 2015 Dec 15;14(12):1154-60. Epub 2015 Aug 15.

AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. Electronic address:

Background: Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.

Methods: Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies.

Results: 214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p<0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p<0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB.

Conclusion: In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.
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http://dx.doi.org/10.1016/j.autrev.2015.08.005DOI Listing
December 2015

Hydroxychloroquine: a multifaceted treatment in lupus.

Presse Med 2014 Jun 19;43(6 Pt 2):e167-80. Epub 2014 May 19.

Université de Clermont-Ferrand, CHU de Clermont-Ferrand, hôpital Gabriel-Montpied, service de médecine interne, 63003 Clermont-Ferrand, France.

The efficacy of antimalarials, especially hydroxychloroquine (HCQ), in preventing systemic lupus erythematosus (SLE) flares is well demonstrated. However, many studies show that the percentage of SLE patients treated with HCQ remains low. By blocking the toll-like receptor 7 and 9 in plasmacytoid dendritic cells, HCQ inhibits interferon-alpha production which plays a crucial role in SLE pathogenesis. In addition to reducing damage accrual in SLE patients, HCQ appears to protect against the occurrence of diabetes, thrombotic events, and dyslipidemia. As a consequence, some studies have suggested that HCQ, which is inexpensive, has a protective effect on survival in SLE patients. Thanks to the pharmacokinetic properties of HCQ (long half-life) and to the availability of its blood assay, very low or undetectable blood HCQ concentrations are a valuable marker of non-adherence to treatment, thus adding a new benefit to HCQ prescriptions. The main side effect of HCQ is retinal toxicity. This complication is very rare, but may be potentially severe, thus requiring regular screening. Retinal toxicity remains the only absolute contra-indication of HCQ in adult SLE patients. Other contra-indications are few and rare. During pregnancy and breast-feeding, HCQ continuation is not only allowed but recommended. In conclusion, the risk/benefit ratio of HCQ is excellent. Many now believe that all SLE patients should be offered this treatment.
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http://dx.doi.org/10.1016/j.lpm.2014.03.007DOI Listing
June 2014

Efficacy of Il-1β blockade in refractory aseptic abscesses syndrome.

Mod Rheumatol 2014 Jan;24(1):217-9

Service de Médecine Interne, Hôpital Gabriel Montpied, CHU de Clermont-Ferrand , 58 rue Montalembert, 63003 Clermont-Ferrand , France.

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http://dx.doi.org/10.3109/14397595.2013.852835DOI Listing
January 2014

Adherence to treatment in systemic lupus erythematosus patients.

Best Pract Res Clin Rheumatol 2013 Jun;27(3):329-40

Université René Descartes Paris V, 75005 Paris, France; AP-HP, Centre de référence maladies auto-immunes et systémiques rares, Service de médecine interne Pôle médecine, Hôpital Cochin, 27 rue du Faubourg Saint Jacques, 75679 Paris cedex 14, France. Electronic address:

Adherence is defined as "the extent to which a person's behaviour coincides with medical or health advice." Poor adherence to therapeutic regimens is a common and expensive problem in patients with chronic diseases including systemic lupus erythematosus (SLE) and is associated with a higher risk of flares, morbidity, hospitalisations and poor renal outcome. Non-adherence to the treatment is multifactorial for most patients and varies according to unintentional or intentional patterns. The rates of non-adherence in SLE patients range from 3% to 76% depending on the assessment methods, which are all subject to limitations. Indeed, poor adherence to therapeutic regimens is difficult to evaluate. Two studies have shown that undetectable blood hydroxychloroquine (HCQ) concentration may be a simple, objective and reliable marker of non-adherence in SLE patients. The accurate diagnosis of non-adherence may prevent one from incorrectly interpreting disease manifestations as a lack of response. It may then avoid an unnecessary or even dangerous treatment escalation.
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http://dx.doi.org/10.1016/j.berh.2013.07.001DOI Listing
June 2013

Study of anti-Müllerian hormone and its relation to the subsequent probability of pregnancy in 112 patients with systemic lupus erythematosus, exposed or not to cyclophosphamide.

J Clin Endocrinol Metab 2013 Sep 5;98(9):3785-92. Epub 2013 Jul 5.

University Pierre and Marie Curie (UPMC), Université Paris 6, Paris, France.

Context: Cyclophosphamide is used for renal and major extrarenal involvement in systemic lupus erythematosus (SLE) and is associated with a risk of premature ovarian failure. There are no data available about the relation between anti-Müllerian hormone (AMH) serum levels and the probability of subsequent pregnancy in SLE patients.

Objective: We analyzed AMH levels and the probability of pregnancy in SLE women exposed to cyclophosphamide.

Design And Setting: We conducted a matched cohort study in referral centers for SLE.

Patients: Fifty-six cyclophosphamide-exposed SLE women younger than 40 years of age and 56 control SLE women matched for age within 6 months participated in the study.

Main Outcome Measures: AMH was measured in samples from the PLUS study (ClinicalTrials.gov no. NCT00413361). All patients were interviewed in May 2012 regarding their obstetric status.

Results: The mean age ± SD of the 112 patients was 31.6 ± 5.8 years. The mean AMH level was low (1.21 ± 1.01 ng/mL) and was significantly lower in patients exposed to cyclophosphamide (P = .03) and in patients older than 30 years (P = .02). During a median follow-up (interval between sampling and the interview) period of 4.2 (range, 2.5-4.8) years, 38 patients sought to become pregnant, and 32 (84.2%) succeeded. In the univariate analysis, the risk of failure was associated with cumulative cyclophosphamide dose (P = .007) and older age (P = .02), but not with AMH.

Conclusion: We confirmed that AMH levels are low in SLE patients and decrease significantly with age and cyclophosphamide exposure. Nonetheless, the risk of failure to conceive was low and was predicted by cyclophosphamide exposure and age, but not by AMH levels.
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http://dx.doi.org/10.1210/jc.2013-1235DOI Listing
September 2013

[IgG4 related disease].

Presse Med 2012 Jul 7;41(7-8):682-94. Epub 2011 Dec 7.

CHU G.-Montpied, service de médecine interne, Clermont-Ferrand cedex 1, France.

IgG4 related disease (IgG4 RD) was first reported as autoimmune pancreatitis then it was established as a systemic disorder characterised by high blood level of IgG4 and fibrosis with rich plasmocytes IgG4+ in almost all organs. IgG4 RD is very sensitive to corticosteroid therapy. IgG4 RD has a high prevalence in eastern countries. Numerous articles on this topic are published and new diagnostic criteria are regularly established. The autoimmune or allergic mechanism of IgG4 RD is still a matter of debate. Interestingly, IgG4 subclass of antibody has anti-inflammatory features. IgG4 RD is not yet very well characterised in western countries. Whether IgG4 is involved in IgG4 RD, pathophysiology is to be defined. IgG4 RD spontaneously regresses in some cases so indications of treatment are not already well clear.
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http://dx.doi.org/10.1016/j.lpm.2011.10.016DOI Listing
July 2012

F-18 FDG-PET/CT in aseptic abscesses with recurrent febrile abdominal pain.

Scand J Gastroenterol 2011 May 30;46(5):577-82. Epub 2010 Nov 30.

Service de Médecine Interne, Hopital Gabriel Montpied, Centre Hospitalier Universitaire, Université d'Auvergne, Clermont-Ferrand, France.

Objective: Mendelian and complex autoinflammatory disorders frequently manifest as recurrent abdominal pain and fever. Diagnosis may be difficult and scant data are available about the interest of 2-deoxy-2-[18F]fluoro-D-glucose-positron emission tomography/computed tomography (FDG-PET/CT) in such conditions, particularly aseptic abscesses (AA).

Material And Methods: We analyzed five cases of AA in which FDG-PET/CT was performed at diagnosis (n = 2) and after a suspected relapse (n = 5). Follow-up FDG-PET/CT was performed in two patients 9 days and 6 weeks after the initiation of oral corticosteroids.

Results: FDG-PET/CT showed intense uptake foci in the abdominal lymph nodes (n = 4), liver (n = 2) and spleen (n = 4) before treatment. A marked metabolic response was observed while patients were being treated. In a relapsing patient with abdominal pain but no raised CRP, although CT scan was unchanged, abnormal uptake of FDG was observed. By contrast, some lesions previously observed on CT scan displayed no fixation on new FDG-PET/CT and were suggestive of sequelae in three patients.

Conclusion: Although nonspecific, FDG-PET/CT may be an interesting tool for the diagnosis and management of recurrent and febrile abdominal pain in AA. At the time of relapse, it can differentiate between a sequela of previous flares and a new localization. It can be used for whole-body screening to look for other asymptomatic disease localizations.
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http://dx.doi.org/10.3109/00365521.2010.539254DOI Listing
May 2011

A new presentation of neonatal lupus: 5 cases of isolated mild endocardial fibroelastosis associated with maternal Anti-SSA/Ro and Anti-SSB/La antibodies.

J Rheumatol 2011 Feb 15;38(2):378-86. Epub 2010 Nov 15.

AP-HP, Service de Médecine Interne, Centre de Référence National pour le Lupus Systémique et le Syndrome des Antiphospholipides, Centre Hospitalier Universitaire Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France.

Objective: Maternal anti-SSA/Ro or anti-SSB/La antibodies are associated with neonatal lupus erythematosus syndrome (NLES), especially congenital heart block (CHB), which may be associated with severe endocardial fibroelastosis (EFE) and dilated cardiomyopathy (DCM). A few reports have described severe EFE without CHB associated with anti-SSA/Ro antibodies, with a poor prognosis. EFE has also been observed in biopsies of DCM that had been considered idiopathic. These points, considered in association with 5 unusual cases of mild EFE, led us to consider the relationship between underrecognized cases of isolated autoantibody-associated EFE and DCM that had been considered idiopathic.

Methods: We analyzed 5 cases of EFE diagnosed in utero (n = 4) or after birth (n = 1). In 3 cases, maternal antibody status was discovered because of the EFE diagnosis.

Results: Endomyocardial hyperechogenicity predominated in the left atrium (n = 3) and mitral annulus (n = 3). No left-heart dysfunction was observed. Two mothers were treated with betamethasone. One mother chose to have a therapeutic abortion, and EFE was confirmed at autopsy. Electrocardiograms at birth (n = 4) did not show CHB. Other manifestations of NLES were present in all cases. One child had right ventricular hypoplasia and underwent a partial cavopulmonary anastomosis. At last followup (4-7 yrs), the other 3 children had normal heart function, and echocardiography showed a normal heart (n = 2) or mild persistent EFE (n = 1).

Conclusion: Middle-term prognosis of isolated autoantibody-associated EFE may be better than previously reported, although the longterm prognosis remains unknown. We hypothesize that a fetal insult can lead to DCM.
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http://dx.doi.org/10.3899/jrheum.100317DOI Listing
February 2011

Atypical dementia.

Lancet 2010 Aug;376(9741):656

Centre de Référence National pour le Lupus Systémique et le Syndrome des Antiphospholipides, Service de Médecine Interne, Université Pierre et Marie Curie-Paris 6, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié-Salpêtrière, Paris, France.

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http://dx.doi.org/10.1016/S0140-6736(10)60877-XDOI Listing
August 2010