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).
Lung Diseases, Interstitial , Lung , Scleroderma, Diffuse , Scleroderma, Limited , Adult , Aged , Cause of Death , Chi-Square Distribution , Female , Heart Diseases/mortality , Heart Diseases/physiopathology , Humans , Hypertension, Pulmonary/mortality , Hypertension, Pulmonary/physiopathology , Logistic Models , Lung/diagnostic imaging , Lung/pathology , Lung/physiopathology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/mortality , Lung Diseases, Interstitial/physiopathology , Lung Diseases, Interstitial/therapy , Male , Microscopic Angioscopy , Middle Aged , Multivariate Analysis , Odds Ratio , Prevalence , Prognosis , Registries , Risk Factors , Scleroderma, Diffuse/diagnosis , Scleroderma, Diffuse/mortality , Scleroderma, Diffuse/physiopathology , Scleroderma, Diffuse/therapy , Scleroderma, Limited/diagnosis , Scleroderma, Limited/mortality , Scleroderma, Limited/physiopathology , Scleroderma, Limited/therapy , Severity of Illness Index , Skin/pathology , Spain/epidemiology , Tomography, X-Ray Computed , Vital Capacity
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Humans , Male , Adult , Crohn Disease/complications , Crohn Disease/diagnosis , Crohn Disease/physiopathology , Clobetasol/therapeutic use , Polymerase Chain Reaction/methods , Polymerase Chain Reaction/trends , Diagnosis, Differential , Infliximab/therapeutic use , Dermatitis, Atopic/complications , Dermatitis, Atopic/drug therapy , Eczema/complications , Eczema/drug therapy , Eczema/physiopathology , Adrenal Cortex Hormones/therapeutic use , Metronidazole/therapeutic use
Crohn Disease/complications , Dermatitis/etiology , Adalimumab/therapeutic use , Adult , Cellulitis/diagnosis , Crohn Disease/drug therapy , Crohn Disease/pathology , Dermatitis/diagnosis , Dermatitis/drug therapy , Dermatitis/pathology , Diagnosis, Differential , Drug Substitution , Giant Cells/pathology , Granuloma/diagnosis , Granuloma/etiology , Granuloma/pathology , Humans , Infliximab/therapeutic use , Male , Sarcoidosis/diagnosis , Thigh
Overgrowth syndromes are characterized by global or localized disproportionate growth associated with other anomalies, including vascular malformations and neurological and/or visceral disorders. CLOVES (Congenital Lipomatous asymmetric Overgrowth of the trunk with lymphatic, capillary, venous, and combined-type Vascular malformations, Epidermal naevi, Scoliosis/Skeletal and spinal anomalies) is an overgrowth syndrome caused by mosaic activating mutation in gene PIK3CA, which gives rise to abnormal PI3K-AKT-mTOR pathway activation. These mutations are responsible for the clinical manifestations of the syndrome, which include low- and high-flow vascular malformations, thoracic lipomatous hyperplasia, asymmetric growth, and visceral and neurological disorders. These common anomalies are illustrated with figures from two personal cases. Identification of the clinical and genetic characteristics of CLOVES syndrome is crucial for the differential diagnosis with other overgrowth syndromes, such as Proteus or Klippel-Trenaunay (K-T) syndromes, and for the therapeutic management of the different anomalies. In this context, a new entity comprising different syndromes with phenotypic mutations in PIK3CA has been proposed, designated PIK3CA-related overgrowth spectrum (PROS), with the aim of facilitating clinical management and establishing appropriate genetic study criteria.
Abnormalities, Multiple/genetics , Lipoma/pathology , Musculoskeletal Abnormalities/pathology , Nevus/pathology , Phosphatidylinositol 3-Kinases/genetics , Vascular Malformations/pathology , Abnormalities, Multiple/pathology , Class I Phosphatidylinositol 3-Kinases , Growth Disorders/pathology , Humans , Mutation , Syndrome
BACKGROUND AND OBJECTIVE: The aim of the present study was to compare the risk stratification obtained with the Framingham function, recommended by the National Cholesterol Education Program (ATP-III), and the risk charts of the Systematic Coronary Risk Evaluation (SCORE) program, in a cohort of subjects between 20 and 75 years (mean 59 years, 70% females) with metabolic syndrome. SUBJECTS AND METHOD: Participants were classified as low, moderate, or high risk by ATP-III and by SCORE of risk at 10 years, respectively. RESULTS: 8.3% of males and 7.4% of women from our study were classified as high risk in 10 years time by Framingham-DORICA. Regarding SCORE program 50% men and 29.6% of women were considered to have high coronary risk. CONCLUSIONS: Risk estimation with SCORE and Framingham function shows significant gender and quantitative differences. There is no concordance between Framingham and SCORE function to estimate cardiovascular risk on subjects included on the high-risk group.
Cardiovascular Diseases/epidemiology , Cardiovascular Diseases/etiology , Metabolic Syndrome/complications , Risk Assessment/methods , Adult , Aged , Algorithms , Female , Humans , Male , Middle Aged , Risk Factors , Spain/epidemiology , Young Adult
Basal cell carcinoma (BCC) is a malignant cutaneous neoplasm with a tendency to spread locally and with several clinical and histological subsets. We studied 82 patients with a clinical diagnosis of superficial BCC on different anatomical locations, to whom imiquimod 5% cream was administered on a low-frequency regime (three times a week for 4 weeks), and who were followed up 2 years after completion of treatment. Clinical clearance rate at 1 and 2 years were 89% and 85%, respectively. We conclude that imiquimod seems to be an appropriate therapeutic alternative for the treatment of superficial BCC in patients with associated comorbidities.
Aminoquinolines/therapeutic use , Antineoplastic Agents/therapeutic use , Carcinoma, Basal Cell/drug therapy , Skin Neoplasms/drug therapy , Administration, Topical , Adult , Aged , Aged, 80 and over , Aminoquinolines/administration & dosage , Antineoplastic Agents/administration & dosage , Female , Humans , Imiquimod , Male , Middle Aged
Immunoglobulin G/therapeutic use , Immunosuppressive Agents/therapeutic use , Psoriasis/drug therapy , Receptors, Tumor Necrosis Factor/therapeutic use , Adolescent , Child , Dose-Response Relationship, Drug , Etanercept , Female , Humans , Male , Treatment Outcome , Tumor Necrosis Factor-alpha/antagonists & inhibitors
