TY - JOUR AU - Witczak, Birgit Nomeland AU - Hetlevik, Siri Opsahl AU - Sanner, Helga AU - Barth, Zoltán AU - Schwartz, Thomas AU - Flatø, Berit AU - Lilleby, Vibke AU - Sjaastad, Ivar TI - Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease : A Controlled Study JF - JOURNAL OF RHEUMATOLOGY J2 - J RHEUMATOL VL - 46 PY - 2019 IS - 7 SP - 739 EP - 747 PG - 9 SN - 0315-162X DO - 10.3899/jrheum.180526 UR - https://m2.mtmt.hu/api/publication/30602468 ID - 30602468 AB - To assess cardiac function in patients with juvenile mixed connective tissue disease (JMCTD) compared to matched controls, and to investigate possible associations between cardiac impairment and disease variables and cardiovascular risk factors.Fifty JMCTD patients (86% female) examined median 14.9 (6.6-23.0) years after disease onset were compared with 50 age- and sex-matched controls. Electrocardiogram and echocardiography [including e' as a marker for diastolic dysfunction and long-axis strain (LAS) and left ventricular (LV) ejection fraction (EF) as markers of systolic function] were performed. LV dysfunction (LVD) was defined as low EF, low LAS, or low e'. Right ventricular function was assessed with tricuspid annular plane systolic excursion (TAPSE). Cardiovascular risk factors and disease variables were assessed.LVD was found in 16% of patients and 4% of controls (p = 0.035). EF and LAS were lower in patients compared to controls (6% lower, p < 0.001, and 4% lower, p = 0.044, respectively). TAPSE was 8% lower in patients versus controls (p = 0.008). No patients had signs of pulmonary hypertension. Patients had longer corrected QT time than controls (p = 0.012). LVD was associated with higher levels of apolipoprotein B, higher disease activity measured by physician's global assessment, longer prednisolone treatment, and more organ damage assessed with the Myositis Damage Index.Patients with JMCTD had impaired left and right ventricular function compared to matched controls after median 15 years disease duration. High disease activity and longer treatment with prednisolone were factors associated with LVD. LA - English DB - MTMT ER - TY - JOUR AU - Barth, Zoltán AU - Schwartz, T AU - Flato, B AU - Aalokken, TM AU - Koller, Ákos AU - Lund, MB AU - Sjaastad, I AU - Sanner, H TI - The Association Between Nailfold Capillary Density and Pulmonary and Cardiac Involvement in Medium- to Long-standing Juvenile Dermatomyositis JF - ARTHRITIS CARE & RESEARCH J2 - ARTHRIT CARE RES VL - 71 PY - 2019 IS - 4 SP - 492 EP - 497 PG - 6 SN - 2151-464X DO - 10.1002/acr.23687 UR - https://m2.mtmt.hu/api/publication/3392111 ID - 3392111 AB - OBJECTIVE: To explore the associations between microvascular abnormalities assessed by nailfold capillaroscopy (NFC) and pulmonary and cardiac involvement in patients with juvenile dermatomyositis (DM) assessed after medium- to long-term follow-up. METHODS: Fifty-eight juvenile DM patients were examined mean 17.0 (SD 10.6) years after symptom onset. Nailfold capillary density (NCD) and neovascular pattern (defined as scleroderma active or late pattern) were analysed blinded to clinical data. Pulmonary involvement was assessed by pulmonary function tests (PFT) including spirometry, diffusion capacity for carbon monoxide (DLCO) and body plethysmography; also high-resolution computed tomography (HRCT) was performed. Cardiac involvement was assessed by electrocardiography, Holter monitoring (heart rate variability) and echocardiography. RESULTS: Patients with low NCD (<6 capillaries/mm) (n=21), compared to patients with normal NCD (>/= 6 capillaries/mm) (n=37), presented lower forced vital capacity (89.7 vs 98.5% of predicted), total lung capacity (87.8 vs 94.5% of predicted) and more often low DLCO (15/21 (71%) vs 14/37 (38%)); all p's<0.05. HRCT assessed airways disease was more frequent in the low NCD group (6/21 (30%) vs 3/37 (8%); p=0.034). No associations were found between i) NCD and cardiac parameters or ii) neovascular pattern and pulmonary or cardiac parameters. CONCLUSION: In juvenile DM patients, low NCD was associated with lung involvement, which was mostly subclinical. No significant associations with cardiac involvement were found. These results shed light on possible mechanisms underlying organ involvement, but further and preferably larger studies are needed to identify NCD as a potential biomarker for lung and cardiac involvement in juvenile DM. This article is protected by copyright. All rights reserved. LA - English DB - MTMT ER - TY - JOUR AU - Barth, Zoltán AU - Witczak, BN AU - Flato, B AU - Koller, Ákos AU - Sjaastad, I AU - Sanner, H TI - Assessment of Microvascular Abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyositis After Medium‐ to Long‐Term Followup JF - ARTHRITIS CARE & RESEARCH J2 - ARTHRIT CARE RES VL - 70 PY - 2018 IS - 5 SP - 768 EP - 776 PG - 9 SN - 2151-464X DO - 10.1002/acr.23338 UR - https://m2.mtmt.hu/api/publication/3255866 ID - 3255866 AB - OBJECTIVE: In juvenile dermatomyositis (JDM), microvascular abnormalities measured by nailfold capillaroscopy (NFC) are common early in disease course. We aimed to compare the presence of NFC abnormalities in patients with medium- to longstanding JDM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics. METHODS: Fifty-eight JDM patients clinically examined median 16.8 (2-38) years after disease onset were compared with matched controls. By NFC, we assessed nailfold capillary density (NCD), giant capillaries, scleroderma and neovascular pattern (defined as scleroderma active or late pattern). NFC was analyzed blinded to patient/control identity and disease characteristics. We measured disease activity and damage by validated tools, and patients were divided into active and inactive disease according to the PRINTO criteria. RESULTS: Compared to controls, patients had decreased NCD, mean 6.4/mm (2.1) vs 7.6/mm (0.8), (p=0.001); and showed more abnormalities in all other NFC parameters; 36% of patients vs. 4% of controls had NCD<6/mm, (p <0.001). Giant capillaries, scleroderma and neovascular pattern were found in 9%, 84% and 41% of patients, respectively. Patients with active disease (n=30) presented more frequently neovascular pattern than inactive patients (n=28) (p =0.041). Decreased NCD and neovascular pattern were associated with higher disease activity and impaired muscle function. CONCLUSION: After medium to long-term follow-up, JDM patients had decreased NCD and often neovascular pattern; both were associated with higher disease activity and impaired muscle function. This suggests that NFC can have a role as a biomarker for disease activity also in longstanding JDM. This article is protected by copyright. All rights reserved. LA - English DB - MTMT ER - TY - JOUR AU - Witczak, BN AU - Hetlevik, SO AU - Barth, Zoltán AU - Schwartz, T AU - Flato, B AU - Lilleby, V AU - Sjaastad, I TI - Impaired Cardiac Function in Juvenile Mixed Connective Tissue Disease Compared with Controls JF - ARTHRITIS & RHEUMATOLOGY J2 - ARTHRITIS RHEUMATOL VL - 69 PY - 2017 IS - Suppl. 10 PG - 6 SN - 2326-5191 UR - https://m2.mtmt.hu/api/publication/3290463 ID - 3290463 LA - English DB - MTMT ER - TY - JOUR AU - Barth, Zoltán AU - Schwartz, T AU - Flato, B AU - Koller, A AU - Samersaw-Lund, MB AU - Sjaastad, I AU - Sanner, H TI - Nail Fold Capillary Changes Are Associated with Pulmonary, but Not with Cardiac Involvement in Juvenile Dermatomyositis JF - ARTHRITIS & RHEUMATOLOGY J2 - ARTHRITIS RHEUMATOL VL - 69 PY - 2017 IS - Suppl. 10 PG - 2 SN - 2326-5191 UR - https://m2.mtmt.hu/api/publication/3290456 ID - 3290456 LA - English DB - MTMT ER - TY - JOUR AU - Barth, Zoltán AU - Witczak, BN AU - Schwartz, T AU - Gjesdal, K AU - Flato, B AU - Koller, Ákos AU - Sanner, H AU - Sjaastad, I TI - In juvenile dermatomyositis, heart rate variability is reduced, and associated with both cardiac dysfunction and markers of inflammation: a cross-sectional study median 13.5 years after symptom onset JF - RHEUMATOLOGY (UNITED KINGDOM) J2 - RHEUMATOLOGY VL - 55 PY - 2016 IS - 3 SP - 535 EP - 543 PG - 9 SN - 1462-0324 DO - 10.1093/rheumatology/kev376 UR - https://m2.mtmt.hu/api/publication/3044511 ID - 3044511 LA - English DB - MTMT ER - TY - JOUR AU - Barth, Zoltán AU - Witczak, B. N. AU - Schwartz, T. AU - Gjesdal, K. AU - Flato, B. AU - Koller, A. AU - Sjaastad, I. AU - Sanner, H. TI - EVALUATION OF THE HEART RATE VARIABILITY AND IT'S RELATIONSHIP WITH THE CARDIAC FUNCTION AND INFLAMMATION IN JUVENILE DERMATOMYOSITIS JF - ANNALS OF THE RHEUMATIC DISEASES J2 - ANN RHEUM DIS VL - 74 PY - 2015 SP - 622 EP - 622 PG - 1 SN - 0003-4967 DO - 10.1136/annrheumdis-2015-eular.1467 UR - https://m2.mtmt.hu/api/publication/32533660 ID - 32533660 LA - English DB - MTMT ER -