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MYCOPHENOLATE MOFETIL IN THE TREATMENT OF SJÖGREN SYNDROME
S36
Abstracts from 10th Congress of the European Federation of Internal Medicine/European Journal of Internal Medicine 22S (2011) S1–S112
Methods: 89 adolescents and young men aged 15-34 years (mean 20.7+/-4.1 yr, height 179.6+/-7.2 cm, weight 80.9+/-15.8 kg, BMI 25.0+/-4.4 kg/m2) with I-II stage prehypertension underwent 24-h. monitoring of ECG, BP and breathing (respiratory inductance plethysmography, Inkart, Russia). Results: One patient reported snoring and none - upper airway disease. 13.5% of pts had low weight (BMI<20 kg/m2), 34.8% - overweight (25-29 kg/m2) and 14.6% were obese (BMI=30-34 kg/m2). Patients were divided into 3 groups: under 5 A/H episodes/hour (AHI 3.0+/-1.0, n=25), with 5-9 (6.9+/-1.5, n=41) and with >10 episodes/hour (AHI 12.8+/-4.0, n=23). Height was significantly bigger in group I (182.5+/7.8 cm) than in the group II (177.8+/-7.9) and didn’t differ from group III. No differences were found in age, weight, BMI, HR, SBP at day (137.8+/-11.8, 137.5+/-12.4 and 138.4+/9.4 mm Hg) and DBP both at day and night, whereas SBP at night in group III was higher (125.4+/-8.4) than in group I (120.4+/-11.2 mm Hg, P=0.04). Power of LF band of HRV in group III was significantly bigger at day and HF at night than in group I. All pts in group III had night pauses up to 2 s. Weak significant correlation obtained between height and AHI (r= - 0.22). Conclusions: SAHS has been found in 71.9% of young men with prehypertension without snoring and was accompanied by elevation of HRV value: power of LF band at day and HF - at night. MYCOPHENOLATE MOFETIL IN THE TREATMENT OF SJÖGREN SYNDROME Sónia Gonçalves, Tiago Pereira, Sara Estrela, Ana Rita Cardoso, Cristina Gonçalves, Luís Jerónimo. Centro Hospitalar Médio Tejo – Tomar, Serviço de Medicina Introduction: Sjögren (SS) syndrome is a systemic rheumatic disease with a prevalence of 0,6-3,3% of the population, there is a clear tendency on appearing in females (14:1). In its physiopathology inflammatory phenomena intervene such as (T e B), cytokine and auto-antibodies. The dry complaints are frequent, however the symptomathology extra-gland, potentially more serious might be difficult to treat. Clinical case: Female, 45 years old with SS (xerostomia, Xerophthalmia, positive Schirmer test, anti-nuclear positive antibodies with anti-SSA and anti-SSB in it) with 10 years of evolution followed by Medicine and Rheumatology medical appointments. Was continuously medicated with hydroxychloroquine, and Azathioprine, however, through a marked and persistent disease (Leukopaenia with Azathioprine, vasculities lesions in the lower limbs, VS highly persistent, hypocomplementemia, polyclonal hypergammaglobulinemia and Rheumatoid factor in high titles) started a therapy with MMF showing a clinical and laboratorial improvement. Comments: The authors present a study case reinforcing the MMF as a possible therapeutic option in the manifestation of extra-gland of SS. ADULT COELIAC DISEASE González Vázquez L1, Fernández Villaverde A2, Rodríguez Pecci S1, Montero Tinnirello J1, Puerta Louro R1, Fernández Fernández F1, De la Fuente Aguado J1. 1Internal Medicine, POVISA Hospital. Vigo. Spain; 2 Gastroenterology, POVISA Hospital. Vigo. Spain Background: Coeliac disease (CD) is a chronic disease of the small intestine, which is caused by gluten intolerance, producing malababsorption of nutrients and vitamins. Clinical manifestations of adult CD are highly variable, including intestinal and extra-intestinal symptoms. Our objective is to describe the incidence and clinical manifestations of CD in adults. Methods: A retrospective study was carried out in patients, diagnosed of CD between January 1990 and December 2010. Diagnosis was based on serologic tests, HLA DQ2/DQ8, and duodenal biopsy. Results: 103 adult patients were diagnosed. Mean age: 33 (18-65) years and 77 (75%) were women. Ten (9,7%) had a first-degree family history and 16 (15,5%) had been previously diagnosed with another autoimmune disease. Clinical manifestations: diarrhea in 53(51,4%), abdominal pain in 39(37,8%), dyspepsia in 23(22,3%), loss of weight in 22(21,3%), neurologic symptoms in 3, and 6 had been diagnosed of dermatitis herpetiformis. Analytical results: slight increase of transaminases in 43(41,7%), ferropenic anaemia in 53(51,4%), hypoalbuminaemia in 19 (18,4%) and folic acid deficiency in 11(20%). Two patients did not show clinical improvement and type 1 resistance was documented. Population-based incidence of CD in adults had increased from 0,7-2/100.000 per year in the ninetys to 15/100.000 in the last year.
Conclusions: CD can be atypical in some cases. Patients with ferropenic anaemia and a negative response to treatment or those with an unexplained increase in transaminases, should be screening for CD. There is a maked increase in the incidence-rats of CD in adults over time. A CASE OF MEDULLARY NECROSIS Inês Gonzalez1, Rita Dutschmann1, Fernando Gomes2, Sofia Santos3. 1 Department of Medicine I of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal; 2Department of Oncology of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal; 3Department of Pathology of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal Medullary necrosis consists of medullary stroma and myeloid tissue necrosis. It’s a rare finding and its cause is almost always malignant. Frequent signs and symptoms are bone pain and fever and laboratory findings include anemia, thrombocytopenia, leucopenia and elevated LDH. Bone marrow histological evaluation is diagnostic and treatment and prognosis depend on the underlying cause. We report the case of a 70-year old caucasian male that was admitted to our Emergency Department with a 6-month history of weight loss, malaise, anorexia, fever and lumbosacral pain. Clinical evaluation revealed apyrexia, mucocutaneous pallor and hepatomegaly. Blood tests showed pancythopenia (Hb: 6,7 g/dL, MCV: 86 fL, MCH: 26,8 pg; leukocytes: 2800/mm3, neutrophils: 900/mm3; platelets: 62.000/mm3) and LDH: 1341 U/L. Bone marrow aspirate showed amorphous substance with no cells. Extensive medullary necrosis was revealed by osteomedullary biopsy. Abdominal ultrasound and body CT scan showed hepatomegaly and a liver biopsy revealed a follicular B-cell lymphoma. Metastatic lesions in the thoracic and lumbar spine were shown in spine MRI. The patient started treatment with Rituximab and Prednisolone. There was significant clinical and laboratory improvement. He was discharged 2 months after admission with continuous follow-up by Oncology and Internal Medicine. The treatment was later changed to cyclophosphamide + vincristine + prednisolone. Seven months after hospital discharge he developed a iatrogenic neutropenia and a severe, bilateral community acquired pneumonia and died. HEART FAILURE WITH PRESERVED EJECTION FRACTION: AN EARLY STAGE OF LEFT VENTRICULAR SYSTOLIC DYSFUNCTION? Páez-Rubio MI1, Carrasco-Sánchez FJ1, Escobar-Cervantes C2, Yebra-Yebra M3, Sánchez-Gómez N3, Santiago-Ruiz JL3, González-García A3, Manzano L3. 1 Department of Internal Medicine. Juan Ramón Jiménez Hospital. Huelva. Spain; 2 Cardiology department. Infanta Sofía Hospital. San Sebastián de los Reyes. Madrid; 3Department of Internal Medicine. Ramón y Cajal Hospital. Madrid Background: Some researches suggest that heart failure with preserved ejection fraction (HFPEF) might represent an early stage of heart failure with reduced ejection fraction (HFREF). However, there are no strong data regarding the natural history of this clinical syndrome. Aims of this study were: 1. To determine whether a cohort of patients diagnosed with HFPEF progress to systolic dysfunction. 2. To evaluate potential variables involve in systolic dysfunction progression. Methods:We enrolled 178 patients with HFPEF. Diagnosis of heart failure was confirmed based on current guidelines. A doppler-echocardiographic study was performed in all cases. The diagnosis of diastolic dysfunction required one of the following conditions to be satisfied: 1. Enlarged left atrium (LA) + brain natriuretic peptide (BNP) levels > 100 pg/ml or E / Ea ratio> 8; 2. Normal LA + BNP levels > 500 pg/ml or E / Ea ratio > 15. Patients with significant valvular heart disease or pericardial disease were excluded. Primary endpoint was progression to systolic dysfunction during the follow-up. We used a T-student or a U-Mann-Whitney test for quantitative variables and the statistical Ji-squared test with Fisher corrections for hypothesis testing. Results: Median follow-up was 24 months (16 to 36.5). The overage age was 80.5 ± 5.7 years. Main baseline features were: women (75.7%), hypertension (96%) and type-2 diabetes mellitus (43.4%). Twenty-five patients (14%) had confirmed coronary heart disease (CHD) and hypertensive cardiomyopathy was present in 61.3%. Mean baseline ejection fraction (EF1) was 64.6 ± 7.2. Only five patients (2.8%) progressed to systolic dysfunction during the follow-up (EF2 41.6 ± 3.8). Patients with highest BNP levels were associated with increased risk for progression to HFREF (p <0.0001). Low glomerular filtration rate did not reach statistical signification (p=0.08). Treatment with beta-blockers, digoxin and/or dihydropyridine calcium antagonists were associated with reduced risk of developing systolic dysfunction. EF reduction was associated with increased risk of mortality (p <0.05).
Abstracts from 10th Congress of the European Federation of Internal Medicine/European Journal of Internal Medicine 22S (2011) S1–S112
Methods: 89 adolescents and young men aged 15-34 years (mean 20.7+/-4.1 yr, height 179.6+/-7.2 cm, weight 80.9+/-15.8 kg, BMI 25.0+/-4.4 kg/m2) with I-II stage prehypertension underwent 24-h. monitoring of ECG, BP and breathing (respiratory inductance plethysmography, Inkart, Russia). Results: One patient reported snoring and none - upper airway disease. 13.5% of pts had low weight (BMI<20 kg/m2), 34.8% - overweight (25-29 kg/m2) and 14.6% were obese (BMI=30-34 kg/m2). Patients were divided into 3 groups: under 5 A/H episodes/hour (AHI 3.0+/-1.0, n=25), with 5-9 (6.9+/-1.5, n=41) and with >10 episodes/hour (AHI 12.8+/-4.0, n=23). Height was significantly bigger in group I (182.5+/7.8 cm) than in the group II (177.8+/-7.9) and didn’t differ from group III. No differences were found in age, weight, BMI, HR, SBP at day (137.8+/-11.8, 137.5+/-12.4 and 138.4+/9.4 mm Hg) and DBP both at day and night, whereas SBP at night in group III was higher (125.4+/-8.4) than in group I (120.4+/-11.2 mm Hg, P=0.04). Power of LF band of HRV in group III was significantly bigger at day and HF at night than in group I. All pts in group III had night pauses up to 2 s. Weak significant correlation obtained between height and AHI (r= - 0.22). Conclusions: SAHS has been found in 71.9% of young men with prehypertension without snoring and was accompanied by elevation of HRV value: power of LF band at day and HF - at night. MYCOPHENOLATE MOFETIL IN THE TREATMENT OF SJÖGREN SYNDROME Sónia Gonçalves, Tiago Pereira, Sara Estrela, Ana Rita Cardoso, Cristina Gonçalves, Luís Jerónimo. Centro Hospitalar Médio Tejo – Tomar, Serviço de Medicina Introduction: Sjögren (SS) syndrome is a systemic rheumatic disease with a prevalence of 0,6-3,3% of the population, there is a clear tendency on appearing in females (14:1). In its physiopathology inflammatory phenomena intervene such as (T e B), cytokine and auto-antibodies. The dry complaints are frequent, however the symptomathology extra-gland, potentially more serious might be difficult to treat. Clinical case: Female, 45 years old with SS (xerostomia, Xerophthalmia, positive Schirmer test, anti-nuclear positive antibodies with anti-SSA and anti-SSB in it) with 10 years of evolution followed by Medicine and Rheumatology medical appointments. Was continuously medicated with hydroxychloroquine, and Azathioprine, however, through a marked and persistent disease (Leukopaenia with Azathioprine, vasculities lesions in the lower limbs, VS highly persistent, hypocomplementemia, polyclonal hypergammaglobulinemia and Rheumatoid factor in high titles) started a therapy with MMF showing a clinical and laboratorial improvement. Comments: The authors present a study case reinforcing the MMF as a possible therapeutic option in the manifestation of extra-gland of SS. ADULT COELIAC DISEASE González Vázquez L1, Fernández Villaverde A2, Rodríguez Pecci S1, Montero Tinnirello J1, Puerta Louro R1, Fernández Fernández F1, De la Fuente Aguado J1. 1Internal Medicine, POVISA Hospital. Vigo. Spain; 2 Gastroenterology, POVISA Hospital. Vigo. Spain Background: Coeliac disease (CD) is a chronic disease of the small intestine, which is caused by gluten intolerance, producing malababsorption of nutrients and vitamins. Clinical manifestations of adult CD are highly variable, including intestinal and extra-intestinal symptoms. Our objective is to describe the incidence and clinical manifestations of CD in adults. Methods: A retrospective study was carried out in patients, diagnosed of CD between January 1990 and December 2010. Diagnosis was based on serologic tests, HLA DQ2/DQ8, and duodenal biopsy. Results: 103 adult patients were diagnosed. Mean age: 33 (18-65) years and 77 (75%) were women. Ten (9,7%) had a first-degree family history and 16 (15,5%) had been previously diagnosed with another autoimmune disease. Clinical manifestations: diarrhea in 53(51,4%), abdominal pain in 39(37,8%), dyspepsia in 23(22,3%), loss of weight in 22(21,3%), neurologic symptoms in 3, and 6 had been diagnosed of dermatitis herpetiformis. Analytical results: slight increase of transaminases in 43(41,7%), ferropenic anaemia in 53(51,4%), hypoalbuminaemia in 19 (18,4%) and folic acid deficiency in 11(20%). Two patients did not show clinical improvement and type 1 resistance was documented. Population-based incidence of CD in adults had increased from 0,7-2/100.000 per year in the ninetys to 15/100.000 in the last year.
Conclusions: CD can be atypical in some cases. Patients with ferropenic anaemia and a negative response to treatment or those with an unexplained increase in transaminases, should be screening for CD. There is a maked increase in the incidence-rats of CD in adults over time. A CASE OF MEDULLARY NECROSIS Inês Gonzalez1, Rita Dutschmann1, Fernando Gomes2, Sofia Santos3. 1 Department of Medicine I of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal; 2Department of Oncology of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal; 3Department of Pathology of Hospital Prof. Dr. Fernando da Fonseca, EPE., Amadora, Portugal Medullary necrosis consists of medullary stroma and myeloid tissue necrosis. It’s a rare finding and its cause is almost always malignant. Frequent signs and symptoms are bone pain and fever and laboratory findings include anemia, thrombocytopenia, leucopenia and elevated LDH. Bone marrow histological evaluation is diagnostic and treatment and prognosis depend on the underlying cause. We report the case of a 70-year old caucasian male that was admitted to our Emergency Department with a 6-month history of weight loss, malaise, anorexia, fever and lumbosacral pain. Clinical evaluation revealed apyrexia, mucocutaneous pallor and hepatomegaly. Blood tests showed pancythopenia (Hb: 6,7 g/dL, MCV: 86 fL, MCH: 26,8 pg; leukocytes: 2800/mm3, neutrophils: 900/mm3; platelets: 62.000/mm3) and LDH: 1341 U/L. Bone marrow aspirate showed amorphous substance with no cells. Extensive medullary necrosis was revealed by osteomedullary biopsy. Abdominal ultrasound and body CT scan showed hepatomegaly and a liver biopsy revealed a follicular B-cell lymphoma. Metastatic lesions in the thoracic and lumbar spine were shown in spine MRI. The patient started treatment with Rituximab and Prednisolone. There was significant clinical and laboratory improvement. He was discharged 2 months after admission with continuous follow-up by Oncology and Internal Medicine. The treatment was later changed to cyclophosphamide + vincristine + prednisolone. Seven months after hospital discharge he developed a iatrogenic neutropenia and a severe, bilateral community acquired pneumonia and died. HEART FAILURE WITH PRESERVED EJECTION FRACTION: AN EARLY STAGE OF LEFT VENTRICULAR SYSTOLIC DYSFUNCTION? Páez-Rubio MI1, Carrasco-Sánchez FJ1, Escobar-Cervantes C2, Yebra-Yebra M3, Sánchez-Gómez N3, Santiago-Ruiz JL3, González-García A3, Manzano L3. 1 Department of Internal Medicine. Juan Ramón Jiménez Hospital. Huelva. Spain; 2 Cardiology department. Infanta Sofía Hospital. San Sebastián de los Reyes. Madrid; 3Department of Internal Medicine. Ramón y Cajal Hospital. Madrid Background: Some researches suggest that heart failure with preserved ejection fraction (HFPEF) might represent an early stage of heart failure with reduced ejection fraction (HFREF). However, there are no strong data regarding the natural history of this clinical syndrome. Aims of this study were: 1. To determine whether a cohort of patients diagnosed with HFPEF progress to systolic dysfunction. 2. To evaluate potential variables involve in systolic dysfunction progression. Methods:We enrolled 178 patients with HFPEF. Diagnosis of heart failure was confirmed based on current guidelines. A doppler-echocardiographic study was performed in all cases. The diagnosis of diastolic dysfunction required one of the following conditions to be satisfied: 1. Enlarged left atrium (LA) + brain natriuretic peptide (BNP) levels > 100 pg/ml or E / Ea ratio> 8; 2. Normal LA + BNP levels > 500 pg/ml or E / Ea ratio > 15. Patients with significant valvular heart disease or pericardial disease were excluded. Primary endpoint was progression to systolic dysfunction during the follow-up. We used a T-student or a U-Mann-Whitney test for quantitative variables and the statistical Ji-squared test with Fisher corrections for hypothesis testing. Results: Median follow-up was 24 months (16 to 36.5). The overage age was 80.5 ± 5.7 years. Main baseline features were: women (75.7%), hypertension (96%) and type-2 diabetes mellitus (43.4%). Twenty-five patients (14%) had confirmed coronary heart disease (CHD) and hypertensive cardiomyopathy was present in 61.3%. Mean baseline ejection fraction (EF1) was 64.6 ± 7.2. Only five patients (2.8%) progressed to systolic dysfunction during the follow-up (EF2 41.6 ± 3.8). Patients with highest BNP levels were associated with increased risk for progression to HFREF (p <0.0001). Low glomerular filtration rate did not reach statistical signification (p=0.08). Treatment with beta-blockers, digoxin and/or dihydropyridine calcium antagonists were associated with reduced risk of developing systolic dysfunction. EF reduction was associated with increased risk of mortality (p <0.05).