Pilar Santo

Hospital Universitari de Bellvitge, l'Hospitalet de Llobregat, Catalonia, Spain

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Publications (10)31.83 Total impact

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    ABSTRACT: Background PAH is a rare complication of SLE and the prevalence varies among the different studies and diagnostic method employed. The clinical course of PAH is asymptomatic until advanced stages and therefore has poor outcome (1). Objectives The aim of this study is to estimate the prevalence of PAH in a cohort of SLE patients and evaluate if Raynaud’s phenomenon (RP) and certain antibodies constitute risk factors for developing PAH. Methods Asymptomatic SLE patients (who fulfilled ACR criteria for SLE) were recruited in a rheumatology unit of a hospital in Barcelona, Spain. We assessed clinical features of SLE, presence of antiphospholipid syndrome, interstitial lung disease, pulmonary embolism, autoantibody profile and history of RP. Transthoracic echocardiography (TTE) was performed to all SLE patients to estimate the pulmonary artery pressures. PAH was defined as systolic pulmonary artery pressure (sPAP) ≥36 mmHg according to current guidelines (2). Results 49 SLE patients were evaluated with TTE and none had PAH. We separated two groups according to sPAP ≤30 and sPAP between ≥31 to 35 mmHg; not finding any differences between the clinical features of the 2 groups. Conclusions PAH is a serious complication with very low prevalence in SLE patients. In our SLE cohort none of our patients developed PAH. Also, we failed to prove any association with RP in the group with higher sPAP. Finally, with our study we may conclude that routine screening with TTE in asymptomatic SLE patients is not justified due to the very low prevalence of PAH in our SLE population. Disclosure of Interest None Declared
    Annals of the Rheumatic Diseases 01/2014; 71(Suppl 3):678-678. DOI:10.1136/annrheumdis-2012-eular.690 · 10.38 Impact Factor
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    ABSTRACT: Background Nailfold capillaroscopy (NC) is a noninvasive technique that allows to scan the microcirculation. Tortuosities and isolated hemorrhages have been described in the NC of patients with primary Sjögren’s syndrome (PSS). In patients with Raynaud’s phenomenon (RP) associated with PSS decreased capillary density, greater presence of hemorrhage, enlarged loops and even megacapillaries have been reported. Objectives 1) To identify specific findings in patients with PSS defined according to the American-European criteria of 2002. 2) To assess capillaroscopic differences in patients with PSS with or without RP. 3) To analise if the positive of anti-Ro and/or anti-La confers a distinct profile in capillaroscopy pattern. 4) To describe whether patients with labial biopsy findings characteristic of PSS have differentiated features in microvascular examination. Methods The multicenter NC group of the Catalan Rheumatology Society (CapiCAT group), conducted 150 NC in patients with Sjögren’s syndrome, 136 of them were diagnosed of PSS. Mean age was 57.6 years (±12.7), 130 (96%) were women. The mean time since PSS diagnosis was 7.9 years (±6.7). Demographic variables, presence of RP, symptoms of PSS, ANA, reumathoid factor, anti-Ro, anti-La, anti-CCP, salivary scintigraphy, labial biopsy, treatment and outcome of NC according to CapiCAT group consensus (capillary density, capillary length, tortuosities, capillary diameter - including enlarges loops and megacapilaries -, branching, capillary organisation, pericapillary hemorrhages, capillary thrombosis, areas of capillary loss, subpapillary venous plexus and capillaroscopic pattern) were collected. Results RP was present in 32% of PSS, keratoconjunctivitis sicca in 91% (125/136) of patients (p<0.001), oral xerosis in 93% (127/136), skin or genital xerosis in 53% (72/136). Extraglandular involvement (arthritis, pneumonitis, blood disorders, peripheral neuropathy or CNS involvement) was associated in 47% (65/136). 75% of PSS were anti-Ro + (102/136) and 40% (54/136) anti-La +. In patients with positive anti-Ro and/or anti-La NC was normal in 53% of cases and nonspecific in 36% (p NS). Lip biopsy was characteristic of PSS in 45/50 lip biopsies. There were no difference in the findings of NC. NC in patients with PSS were normal in 51% of cases and nonspecific in 34%. Scleroderma pattern was reported in 14 patients. RP associated with PSS had nonspecific capillaroscopy in 40% of cases (p=0.1). The amount of pericapillary hemorrhages (p=0.06) and capillary thrombosis (p=0.2) were not increased in our patients, but more enlarged loops were detected in 48% (18/37) of cases. Conclusions 1) Patients with positive anti-Ro and/or anti-La have not a distinct profile in capillaroscopy pattern. 2) Patients with labial biopsy findings characteristic of PSS have not differential features in microvascular examination. 3) In our sample, patients with RP associated with PSS had more enlarged loops, but neither pericapillary hemorrhagesnor capillary thrombosis were observed, as reported in the literature. Disclosure of Interest None Declared
    Annals of the Rheumatic Diseases 01/2014; 71(Suppl 3):553-553. DOI:10.1136/annrheumdis-2012-eular.3188 · 10.38 Impact Factor
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    ABSTRACT: In March 2008, FRAX, developed by Kanis and collaborators in the University of Sheffield and supported by the World Health Organization, became available online to calculate absolute risk of osteoporotic fracture in the next 10 years. To analyze the risk of fracture calculated by FRAX and its determinants in the patients sent to a densitometry unit for bone mineral density (BMD) testing. All the patients submitted by Primary Care to the Densitometry Unit for BMD testing underwent a self administered questionnaire to assess the clinical risk factors included in FRAX and a bone densitometry of lumbar spine and proximal femur with a DXA densitometer Hologic QDR 4500. They were classified as having a normal BMD, osteopenia or osteoporosis along with the recommendations of the International Society for Clinical Densitometry. As the reference population to calculate the T and Z scores, we used the one from the NHANES III study for femoral neck and total hip and the one from the Study of the Spanish Population for total spine. With the data of the questionnaire, we calculated, by FRAX, the absolute risk in the next ten years of having a major fracture (MFR) or a hip fracture (HFR). Both risks were calculated with or without the inclusion in the algorithm of BMD: MFR+, MFR-, HFR+ and HFR-. The results were recorded in an Access 2003 database and analyzed with the statistical package SPSS 15.0 for Windows. We analyzed the data from 853 women with a mean age of 61.9 (8.9) years and a mean body mass index of 27.0 (4.2)kg/m(2). Mean BMD at lumbar spine was 0.873 (0.127)g/cm(2); at femoral neck, 0.704 (0.105)g/cm(2); and at total hip, 0.817 (0.107)g/cm(2). Twenty percent of the patients had a normal BMD, 55% had osteopenia and 25%, osteoporosis. Yet excluding age and body mass index, the number of fracture risk factors seems low: 31% of the patients had no risk of fracture; 40%, had one; 22%, two; 6%, three; 1%, four; and one patient had five. Mean MFR+ was 5.4 (4.8)%; mean MFR-, 6.3 (5.5)%; mean HFR+, 1.5 (2.9)%; and HFR-, 2.1 (3.3)%. When BMD was included in the algorithm for the calculation of the risk of fracture, the risk was statistically lower (p<0.001), especially in patients with better BMD. The risk of fracture calculated by FRAX in the patients sent to a densitometry unit for bone BMD testing seems low and, probably, a better selection of the patients would detect a higher risk of fracture population. When the fracture risk is calculated with the introduction of BMD in the algorithm, it is lower than without including BMD.
    05/2012; 8(4):179-83. DOI:10.1016/j.reuma.2012.02.003
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    ABSTRACT: BACKGROUND AND OBJECTIVE: To assess the effect of the application in routine clinical practice of a proposal of thresholds for the indication of bone densitometry in Spanish postmenopausal women. PATIENTS AND METHODS: We determined the risk of major fracture (RMF) by FRAX(®) of the patients referred to a bone densitometry unit from Primary Care who were untreated. We calculated how many scans would have been avoided if they had been performed only to women≥65 years with a RMF<10% or women<65 years with a RMF≥3.6%. RESULTS: We included 643 women with a mean age of 61 (9) years. Twenty-three percent had a normal bone mineral density, 56% had osteopenia, and 21% osteoporosis. The RMF was 5.9 (5.5)%. Eighty of 217 (37%) bone scans in women≥65 years and 273 of 426 (64%) in women<65 years would have been avoided. As a whole, 55% of the scans would have been avoided. The sensitivity of the threshold of 3.6% of RMF for the diagnosis of osteoporosis was 51%, specificity 68%, positive predictive value 20%, and negative predictive value 20%. CONCLUSIONS: The application of the proposed thresholds for the indication of bone densitometry in Spanish postmenopausal women, based on age and risk of fracture calculated by FRAX(®) would result in a significant decrease of the activity of the bone densitometry unit.
    Medicina Clínica 05/2012; 140(10). DOI:10.1016/j.medcli.2012.03.008 · 1.25 Impact Factor
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    ABSTRACT: To describe the clinical, laboratory and radiological features, treatment and prognosis of patients with adult onset Still's disease (AOSD). Specific clinical features were retrospectively recorded in 41 patients fulfilling the Yamaguchi criteria. Patients were reviewed in two academic hospitals with a referral area of 700,000-1,000,000 inhabitants. Laboratory tests including haemogram, ferritin, biochemistry and autoimmunity were reviewed. Radiological studies, treatment and ACR functional class were determined. Forty-one patients with AOSD were identified, 25 of whom were female. Mean age at diagnosis: 38.19 years (range 17-68). Feverish polyarthritis was the most common clinical presentation. Acute phase reactants were invariably high in all patients. Serum ferritin levels were elevated in 86% of patients. Anti-cyclic citrullinated peptide antibodies (anti-CCP antibodies) were negative in all patients except one. The course of the disease was monocyclic in 44% of the patients, polycyclic in 26%, and chronic articular in 30%. ACR class was as follows: 29 (72.5%) class I, 7 (17.5%) class II, 2 (5%) class III and 2 (5%) class IV. As for the treatment received, aspirin or NSAIDs controlled the disease in eight patients (19.5%) and high-dose corticosteroids (0.5-1 mg/kg/day) in 32 (78%). Almost half of the patients (49%) required an additional diseasemodifying agent, usually methotrexate. Finally, in seven of them (17%) a biological treatment with TNF-α or specially anti-IL-1 had to be added to control the disease. The clinical and laboratory findings were similar to previous studies. Anti-CCP antibodies were almost always negative. A monocyclic course was associated with a good prognosis. Most of the patients were in ACR functional class I and II. Biological agents were required in 7 patients (17%).
    Clinical and experimental rheumatology 03/2011; 29(2):331-6. · 2.97 Impact Factor
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    ABSTRACT: Blue digit syndrome (or sign) is a cutaneous manifestation of multiple diseases that produce acute or subacute ischemic compromise in one or more fingers or toes. The most frequent cause of this syndrome is a reduction in arterial blood flow due to compromise or occlusion of small peripheral vessels, with preservation of the distal pulses. The reduction in blood flow may be caused by a variety of pathogenic mechanisms including thrombosis, embolism, severe vasoconstriction, or inflammatory or non-inflammatory lesions of the vascular wall. The finger or toe affected by ischemia turns blue or violet and may develop necrosis.Independently of the cause, blue digit syndrome is a medical emergency requiring rapid diagnosis and specific treatment, given the risk of progression to irreversible necrosis.
    Seminarios de la Fundación Española de Reumatología 01/2011; 12(1):2–9. DOI:10.1016/j.semreu.2010.04.002
  • Iván Castellví · Pilar Santo
    Seminarios de la Fundación Española de Reumatología 11/2010;
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    ABSTRACT: La maladie de Castleman (MC) est un syndrome lymphoprolifératif rare qui peut se présenter sous deux formes : la forme localisée et la forme multicentrique. La MC multicentrique comporte habituellement des signes généraux, une polyadénopathie superficielle, une hépatosplénomégalie et des signes biologiques inflammatoires. Une éruption cutanée et des arthrites peuvent aussi être des manifestations révélatrices. Dans ces cas, la MC multicentrique peut ressembler à une maladie de Still de l’adulte (MSA), notamment quand les arthrites précèdent les autres manifestations. Nous présentons ici l’observation d’un patient pour lequel un diagnostic de MSA avait été suspecté initialement, mais chez lequel 18 mois plus tard le diagnostic de MC multicentrique était porté devant l’apparition d’adénopathies axillaires de volume rapidement croissant. Malgré sa rareté, le diagnostic de MC multicentrique doit être gardé à l’esprit parmi les diagnostics différentiels chez un patient ayant une suspicion de MSA.
    Revue du Rhumatisme 05/2009; 76(5):471-474. DOI:10.1016/j.rhum.2008.10.017
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    ABSTRACT: Castleman's disease is a rare lymphoproliferative disorder having two types of presentation: the localized and the multicentric form. Multicentric Castleman's disease (MCD) typically presents with constitutional symptoms, generalized peripheral lymphadenopathy, hepatosplenomegaly, and laboratory markers of inflammation. Rash and arthritis may also be initial complaints of this disease. In these cases, MCD can resemble adult-onset Still's disease (AOSD), especially if the arthritis precedes other manifestations. We describe a patient with initial clinical suspicion of AOSD. Eighteen months later evidence of MCD was ascertained when the patient developed insidiously growing axillary lymphadenopathies. Despite its rarity, MCD should be borne in mind in the differential diagnosis of patients with suspicion of AOSD.
    Joint, bone, spine: revue du rhumatisme 03/2009; 76(3):304-7. DOI:10.1016/j.jbspin.2008.10.005 · 3.22 Impact Factor
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    ABSTRACT: Lobular panniculitis, together with polyarthritis and intraosseous fat necrosis, may occasionally complicate pancreatic disease. This triad is known in the literature as the pancreatitis, panniculitis, and polyarthritis (PPP syndrome). We describe a case of the PPP syndrome and review the available literature to summarize the clinical characteristics of patients with this condition. A patient with the PPP syndrome, with evidence of extensive intraosseous fat necrosis in the joints involved revealed by magnetic resonance imaging, is described and the relevant literature based on a PubMed search from 1970 to February 2008 is reviewed. The keywords used were pancreatitis or pancreatic disease, panniculitis, arthritis, and intraosseous fat necrosis. Including our case, 25 well-documented patients with the PPP syndrome have been reported. Our patient had few abdominal symptoms despite high serum levels of pancreatic enzymes. In our review of the literature, almost 2/3 of patients had absent or mild abdominal symptoms, leading to misdiagnosis. The delay in diagnosis and specific treatment of the underlying pancreatitis worsens the prognosis of this condition, which has a mortality rate as high as 24%. In nearly 45% of the patients, the arthritis follows a chronic course with a poor response to nonsteroidal anti-inflammatory drugs and corticosteroids, and the rapid development of radiographic joint damage. Certain forms of pancreatic disease can very occasionally cause arthritis and panniculitis. Although uncommon, physicians should be alert to the possible presence of this syndrome for 2 reasons: first, unrecognized pancreatic disease can be fatal if not treated promptly; second, to avoid inappropriate and risky therapy to improve joint symptoms.
    Seminars in arthritis and rheumatism 01/2009; 39(5):417-23. DOI:10.1016/j.semarthrit.2008.10.001 · 3.63 Impact Factor