Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.

OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

Accelerated atherosclerosis in ANCA-associated vasculitis.

OBJECTIVES: Cardiovascular disease, including myocardial infarction and stroke, is a major cause of mortality in ANCA-associated vasculitis (AAV). Although AAV affects small vessels, an accelerated atherosclerosis not explained by traditional cardiovascular risk factors (CVRF) has been demonstrated. We aimed to investigate the association of atherosclerosis measured by carotid intima-media thickness (CIMT) and cerebral small vessel disease in AAV-patients. MATERIALS & METHODS: Twenty-three AAV-patients in complete remission were recruited. Carotid ultrasonography (US), transcranial Doppler (TCD), brain magnetic resonance imaging (MRI), and SPECT after intravenous administration of tracer 99mTc-HMPAO (dose: 720MBq) were performed. RESULTS: AAV-patients presented higher CIMT compared to normative population. Multivariate linear regression analysis demonstrated an association of higher CIMT with increased pulsatility index in middle cerebral artery (PI-MCA) (P=.011), higher lesion load on ARWMC scale (P=.011) and abnormal SPECT (P=.008). No association between higher CIMT and CVRF (diabetes or hypertension) was demonstrated. Increasing internal carotid artery pulsatility index (PI-ICA) was associated with decreasing mean flow velocity (MFV)-MCA (P=.038), increasing PI-MCA (P=.008) and increasing white matter lesions on MRI (P=.011). CONCLUSIONS: Our study adds weight to the presence of increased atherosclerosis in AAV-patients. The association observed between CIMT and PI-ICA with small vessel cerebral disease, points the possible association of easy to use carotid US in predicting microvascular brain injury.

[Report of the editors, 2011]. / Informe de los editores, 2011.

The editors of Revista Clínica Española (Rev Clin Esp) inform on their editorial activity during the last 12 months (November 2010 to October 2011): (a) Objectives and attainments during 2011, (b) editorial activity, and (c) objectives for 2012. In 2011 we have updated the editorial algorithm (revision by the responsible editor of all manuscripts sent to peer review and incorporated an «editorial coordinator¼), we have renovated two advise facilities (editorial and scientific committees), we have created a new section called «monthly e-image¼, and we have promoted Rev Clin Esp annual prizes. From the first January 2010 to 31(st) October 2011 we handled 422 manuscripts (42,2 manuscripts per month, higher than the 2010 figure of 40,4 manuscript/month). Overall we have accepted 26% (originals, 16%). We asked for 343 revisions and obtained 231 (67%). Seventy two percent of the reviewers sent their comments in less than two weeks. The mean time taken to accept or reject a given manuscript has been 26 days. The mean time taken since a manuscript is received to publication (october, novembrer and december issues) has dropped from 334 days in 2010 to 254 in 2011 (24% decrease). The collaboration with the working groups has reported about 2 published manuscripts per issue. Our objectives for 2012 are: (a) to improve the editorial process; (b) main article translation into English; (c) improve some sections (i.e. clinical conference); (d) estimulate working groups collaboration; and (e) improve continued medical education. Revista Clínica Española is an open forum for all internal medicine specialists. We all have the responsibility to make our journal, each day, better.

[2009 report from the editors]. / Informe de los editores: 2009.

The editors of the Rev Clin Esp present the editorial course of action of the journal over the past year. We have up-dated the design of the journal, its contents (sections) and computerized the editorial process. We processed 467 manuscripts and made an editorial decision on 402 of them between November 2008 and October 2009. A total of 92 manuscripts (23%) were accepted. Fifteen (13%) out of the 119 original articles for which the editorial process was completed were accepted. Our goal for the year 2010 is to make the journal available on the Internet for all those who are subscribers to the Rev Clin Esp as well as for internal medicine residents (for which they must be members of each regional society). Other objectives of the editorial team are to edit the E-cases, for the journal to be a continuing education tool and that the coordinators of the work groups develop a monographic number at least once every two years. These actions aim to increase the impact factor and the quality of the Rev Clin Esp, official publication of the Spanish Society of Internal Medicine and of Spanish-speaking internal medicine physicians.

First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study.

The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis.

Update in pulmonary arterial hypertension. / Actualización en hipertensión arterial pulmonar.

Pulmonary arterial hypertension is a rare and progressive disease that mainly affects the pulmonary arterioles (precapillary), regardless of the triggering aetiology. The prevalence of pulmonary hypertension and pulmonary arterial hypertension in Spain is estimated at 19.2 and 16 cases per million inhabitants, respectively. The diagnosis of pulmonary arterial hypertension is based on haemodynamic criteria (mean pulmonary artery pressure ≥25mmHg, pulmonary capillary wedge pressure ≤15mmHg and pulmonary vascular resistance >3 Wood units) and therefore requires the implementation of right cardiac catheterisation. Sequential therapy with a single drug has been used in clinical practice. However, recent European guidelines recommend combined initial therapy in some situations. This review conducts a critical update of our knowledge of this disease according to the latest guidelines and recommendations.

Report of the editors, 2014. / Informe de los Editores, 2014.

The editors of Revista Clínica Española(Rev Clin Esp) inform on their editorial activity during the last 12 months: (a) Objectives and attainments in 2014, (b) Editorial activity, 2014, and (c) 2013 impact factor. In 2014 we achieved the 5 planned objectives. We have published the 9 programmed issues and 103% of the planned papers according to the usual fixed sections. We emphasize the publication of 29 editorials, 21 of which are signed by prestigious foreign authors. From the first January to the 30th September 2014 we received 421 manuscripts (46.8 manuscripts per month), a slight lower figure to that obtained in 2013 (50.9 manuscripts per month). The acceptance rate of the 404 manuscripts whose editorial process has been concluded was 32.3% (originals, 22.4%). We asked for 315 revisions to 240 reviewers and we received 53.3% revisions in less than two weeks (10.4 days). The mean time to adopt an editorial decision for all manuscripts («accepted¼/«rejected¼) has been 18,3 (less than half than in 2009). For «originals¼ this figure has dropped from 56,6 days in 2009 to 26.6 days in 2014. The mean time elapsed from manuscript reception to its on-line publication was 103 days. In 2014 the collaboration with the working groups from the Internal Medicine Spanish Foundation (FEMI) has reported 11 published manuscripts. In July 2014 we were informed that the Journal Citation Reports gave Rev Clin Esp an Impact Factor of 1,314 (year 2013). This Impact Factor without self-citations would have been 0.705 (in 2009 the global impact factor was 0,584). With the Editorial Committee farewell we welcome the new editorial team and we sincerely thank the SEMI Steering Committee, our colleagues, journal officers, reviewers, readers and authors that since 2009 have trusted on our editorial work.

Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

[Diagnosis with helical CT and echocardiography of patients with suspected chronic thromboembolic pulmonary hypertension]. / Diagnóstico con TAC helicoidal y ecocardiografía de la hipertensión pulmonar por tromboembolismo pulmonar crónico.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a serious disease with a very high mortality after variable periods of time. Early diagnosis of CTEPH is critical because thromboendarterectomy can improve survival, hemodimamics and functional capacity. Based on our experience we have performed a review of diagnostic techniques. Echocardiography, pulmonary angiography, fiberoptic angioscopy, helical CT an MRI. For each diagnostic procedure we have analysed its accuracy, invasiveness and easiness of use. We conclude that currently the most useful techniques are echocardiography (to asses pulmonary hypertension), and helical CT which is a good alternative to pulmonary angiography and can detect very specific findings such as dilatation of central pulmonary arteries, eccentric localized thrombi and mosaic attenuation of the pulmonary parenchyma. Even more accurate findings can be expected by the use of multislice CT, which could allow to asses the degree of narrowing of the pulmonary vessels and bronchial circulation.

[Homocysteine. The cardiovascular risk factor of the next millennium?]. / La homocisteína. El factor de riesgo cardiovascular del próximo milenio?

Several epidemiologic studies have demonstrated that hyperhomocysteinemia is a risk factor for arteriosclerosis in coronary, cerebral, peripheral and aortic arteries. This risk is independent of other cardiovascular risk factors, and it is dose related. However, prospective studies show contradictory findings. Hyperhomocysteinemia is also associated with a higher risk of venous thrombosis to which other coagulation disorders, such as factor V Leiden, could contribute. Hyperhomocysteinemia can be due to genetic defects in the enzymes that control homocysteine metabolism, and also to other factors, mainly nutritional (deficiencies in vitamin B6, vitamin B12, or folic acid). Dietary supplements of these vitamins reduce plasma homocysteine levels. Randomized clinical trials are still needed to demonstrate that reducing plasma homocysteine levels will reduce the risk for cardiovascular disease.

[Report of the editors, 2013]. / Informe de los editores, 2013.

The editors of Revista Clínica Española(Rev Clin Esp) inform on their editorial activity during the last 12 months: (a) objectives and attainments, (b) editorial activity, and (c) objectives for 2014. In 2013 the most relevant modification concerning the editorial activity has been the translation into English of the 5 manuscripts with abstract contained in each issue (http://www.revclinesp.es/). From the first January to the 30th September 2013 we received 458 manuscripts (50.9 manuscripts per month), a similar figure to that obtained in 2012 (51.1 manuscripts per month). The acceptance rate of the 443 manuscripts whose editorial process has been concluded was 23.7% (originals, 11.8%). We asked for 253 revisions to 186 reviewers and we received 74.4% revisions in less than 2 weeks (10.9 days). The mean time to adopt an editorial decision for all manuscripts («accepted¼/«rejected¼) has been 20,3 (half than in 2009). For «originals¼ this figure has dropped from 56.6 days in 2009 to 22.5 days in 2013. The mean time elapsed from manuscript reception to its on-line publication was 94.8 days in 2013 (110.5 in 2012 and 155.8 in 2011). In 2013 the collaboration with the working groups from the Internal Medicine Spanish Foundation has reported 17 published manuscripts. In 2013 we were informed that the Journal Citation Reports excluded Rev Clin Esp from its impact factor journal list due to its elevated self-citations. We have taken a number of actions to reduce self-citations and we expect to be a minority in 2014. Some other data concerning the editorial policy are encouraging. In this sense, manuscript citation to Rev Clin Esp published articles has seen a substantial increase from 19% in 2008 to 29% in 2012. We work to achieve the digitalization of Rev Clin Esp from 1940 to 1999 (the journal is already digitalized since 2000). The continuous renewal of the journal sections and the working groups collaboration are necessary elements to make our journal, each day, better.

Editors' report, 2012.

The editors of Revista Clínica Española (Rev Clin Esp) inform on their editorial activity during the last 12 months: a) objectives and attainments; b) editorial activity; and c) objectives for 2013. In 2012 the most relevant modifications concerning the editorial activity have been: a) revision by the responsible editor of all manuscripts sent to peer review, and b) translation into English of five manuscripts with abstract of each issue. From the first January to the 30th September 2012 we handled 457 manuscripts (50 manuscripts per month) a figure 22.5% higher than in 2011 (40.8 manuscripts per month). We have accepted 27.2% of all manuscripts (originals, 13.0%). We asked for 295 revisions to 241 reviewers and we have received 66.1% of the revisions asked for in less than two weeks (mean time to receive a revision, 11.3 days). The mean time to adopt an editorial decision for all manuscripts («accept¼/«reject¼) has been 23.9 days (31.3 days in 2011; 35.4 in 2010). This figure has been 29.4 days for the original manuscripts (9.5 and 12.3 days less than in 2011 and 2010, respectively). The mean time taken since a manuscript is received to publication has dropped from 203 days in 2010, 168 in 2011 and 115 in 2012 (less than 3 months). The collaboration with the working groups has reported 19 published manuscripts in 2012. We hope that in 2013 both the English version and the digitalization of Rev Clin Esp from 1940 to 1999 (the journal is already digitalized since 2000) will become a reality. The renewal of the actual sections (i.e., clinical conference, an image for a diagnosis) pretends, with the collaboration of all, to make our journal, each day, better.

[Climate change: new diseases for a new climate?]. / Cambio climático: euronuevas enfermedades para un nuevo clima?

Since the beginning of the 20th century, there has been a progressive increase in the Earth's temperature. This warming of the Earth's surface is largely due to the so-called green house effect that can alter the natural behavior of some natural phenomena such as El Niño. Several articles on climate change have been published recently. Some authors have raised the question of whether these changes could affect human health or even lead to the reappearance of some eradicated diseases. This report focuses on the changes seen in health up to date and their probable causes. Some changes in energy emission levels have been proposed in order to restrain this trend.

[Interstitial cells of Cajal: another contribution to modern medicine]. / Las células intersticiales de Cajal: otra contribución a la medicina moderna.

The contributions of Santiago Ramon y Cajal to Modern medicine are innumerable. This large scientist and humanist, a man ahead of his time, spoke for the first time such concepts as angiogenesis, which represents one of the most innovative fields for oncology today, in addition to conducting experimental research on the degeneration and regeneration of the nervous system by which laid the foundation morphofunctional of neuroscience today. In this case, we wanted to remember the significance of the discovery of so-called <>, which have been involved in multiple processes and digestive diseases we collect, and especially his recent relationship with gastrointestinal stromal tumors with the therapeutic implications this could bring.

ANCA-associated vasculitis as paraneoplastic syndrome with colon cancer: a case report.

Coexistence of a vasculitis and a neoplastic disease is rare and the pathogenesis is unknown. Most of these associations refer to leukocytoclastic or poliarteritis nodosa (PAN)-type vasculitis and hematological malignancies. There are few reports of vasculitis in patients with solid tumours and there are also few reports of paraneoplastic ANCA-associated vasculitis. We report a case of p-ANCA-positive vasculitis with peripheral nerve involvement associated with a colon cancer. Vasculitis resolved after corticoid treatment and surgical removal of the tumour.

[Cost-effectiveness of computerized axial tomography in the diagnosis of traditional clinical picture of fever of unknown origin]. / Rentabilidad de la tomografía axial computarizada en el diagnóstico de la fiebre de origen desconocido clásica.

Diagnostic strategy in fever of unknown origin has been changing in recent decades. The objective of this study is to define the role of thoracoabdominal computerized axial tomography in the first diagnostic stage. A sample of 24 patients with classical fever of unknown origin from our hospital Internal Medicine department was selected between January 1995 and December 2002. Cost-effectiveness of diagnostic thoracoabdominal computerized axial tomography compared to that of abdominal echography was analyzed; the information obtained with both diagnostic techniques for the final diagnosis was also evaluated. Computerized axial tomography contributed data suggestive of the diagnosis in 10 of 24 patients (41.7%); abdominal echography only gave orienting information to the diagnosis in 2 cases (8%). In 9 (90%) of the 10 patients with computerized axial tomography specific alterations, a conclusive definitive diagnosis (with analysis of biological sample) was possible. Accordingly, the use of this technique is justified at the beginning of the study of the classical fever unknown origin in order to orient the clinician to resort to other more directed and specific diagnostic techniques and with higher diagnostic cost-effectiveness.

[Clinical-biological and radiological study of Wegener's granulomatosis in a university hospital]. / Estudio clínico-biológico y radiológico de la granulomatosis de Wegener en un hospital universitario.

UNLABELLED: Wegener's granulomatosis is a rare systemic vasculitis that fundamentally affects the kidney and respiratory tract. Although the histological findings are very characteristic, they do not occur in all cases, which makes diagnosis difficult. METHODS: The main clinical, biological and radiological characteristics of 13 cases of Wegener's granulomatosis diagnosed in a university hospital of the center of Spain are collected in this present paper. RESULTS: The majority of the patients were men in their 50's. Fever and general syndrome were usual presentations of the disease. Upper respiratory tract (10 cases) and lung involvement (9), followed by renal involvement (8), were the most common. Alveolar infiltrates and bilateral nodules were the most common findings in the X-ray and thoracic CT scan, although the latter permits a greater definition of the lesions. Measurement of antibodies against cytoplasma of the neutrophils (ANCA) was positive in 8 patients (6 cANCA and 2 pANCA). Renal biopsy was used most for the histologic diagnosis, where focal and segmental glomerulonephritis was the most common finding. The majority of the patients received steroids plus cyclophosphamide as induction treatment. CONCLUSIONS: Wegener's disease is a rare systemic vasculitis. The existence of hematuria or respiratory tract involvement in a patient with fever suggests the diagnosis. Conducting a CT scan and ANCAs measurement are useful tools for its diagnosis, since the histology is not definitive.