Zoltan Barth1, Thomas Schwartz2, Berit Flatø3, Trond M Aaløkken4, Akos Koller5, May B Lund6, Ivar Sjaastad2, Helga Sanner7. 1. Institute for Experimental Medical Research and K. G. Jebsen Center for Cardiac Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway, Institute for Translational Medicine, Medical School, University of Pécs, Pécs, Hungary, and Bjørknes University College, Oslo, Norway. 2. Institute for Experimental Medical Research and K. G. Jebsen Center for Cardiac Research, Oslo University Hospital-Ullevål and University of Oslo, Oslo, Norway. 3. Oslo University Hospital-Rikshospitalet and Institute for Clinical Medicine, University of Oslo, Oslo, Norway. 4. Oslo University Hospital-Rikshospitalet, Oslo, Norway. 5. Medical School, University of Pécs, Pécs, Hungary, and University of Physical Education, Budapest, Hungary. 6. Institute for Clinical Medicine, University of Oslo and Oslo University Hospital-Rikshospitalet, Oslo, Norway. 7. Bjørknes University College and Oslo University Hospital-Rikshospitalet, Oslo, Norway.
Abstract
OBJECTIVE: To explore the associations between microvascular abnormalities as assessed by nailfold capillaroscopy (NFC) and pulmonary and cardiac involvement in patients with juvenile dermatomyositis (DM) who are assessed after medium- to long-term follow-up. METHODS: Fifty-eight patients with juvenile DM were examined a mean ± SD of 17.0 ± 10.6 years after symptom onset. Nailfold capillary density (NCD) and a neovascular pattern (defined as an active or late scleroderma pattern) were analyzed, with blinding to clinical data. Pulmonary involvement was assessed by pulmonary function tests including spirometry, diffusing capacity for carbon monoxide (DLco), and body plethysmography. High-resolution computed tomography (HRCT) was also 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) had lower forced vital capacity (89.7% versus 98.5% predicted), total lung capacity (87.8% versus 94.5% predicted), and more often had low DLco values (15 [71%] of 21 patients versus 14 [38%] of 37 controls) (all P < 0.05). Use of HRCT to assess airway disease was more frequent in the group with low NCD (6 [30%] of 20 patients versus 3 [8%] of 36 patients in the normal NCD group; P = 0.034). No associations between NCD and cardiac parameters or between neovascular pattern and pulmonary or cardiac parameters were observed. CONCLUSION: In patients with juvenile DM, low NCD was associated with lung involvement, which was mostly subclinical. No significant associations with cardiac involvement were observed. 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.
OBJECTIVE: To explore the associations between microvascular abnormalities as assessed by nailfold capillaroscopy (NFC) and pulmonary and cardiac involvement in patients with juvenile dermatomyositis (DM) who are assessed after medium- to long-term follow-up. METHODS: Fifty-eight patients with juvenile DM were examined a mean ± SD of 17.0 ± 10.6 years after symptom onset. Nailfold capillary density (NCD) and a neovascular pattern (defined as an active or late scleroderma pattern) were analyzed, with blinding to clinical data. Pulmonary involvement was assessed by pulmonary function tests including spirometry, diffusing capacity for carbon monoxide (DLco), and body plethysmography. High-resolution computed tomography (HRCT) was also 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) had lower forced vital capacity (89.7% versus 98.5% predicted), total lung capacity (87.8% versus 94.5% predicted), and more often had low DLco values (15 [71%] of 21 patients versus 14 [38%] of 37 controls) (all P < 0.05). Use of HRCT to assess airway disease was more frequent in the group with low NCD (6 [30%] of 20 patients versus 3 [8%] of 36 patients in the normal NCD group; P = 0.034). No associations between NCD and cardiac parameters or between neovascular pattern and pulmonary or cardiac parameters were observed. CONCLUSION: In patients with juvenile DM, low NCD was associated with lung involvement, which was mostly subclinical. No significant associations with cardiac involvement were observed. 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.
Authors: Birgit Nomeland Witczak; Thomas Schwartz; Zoltan Barth; Eli Taraldsrud; May Brit Lund; Trond Mogens Aaløkken; Berit Flatø; Ivar Sjaastad; Helga Sanner Journal: Rheumatol Int Date: 2022-01-04 Impact factor: 3.580
Authors: Alexander G S Oldroyd; James B Lilleker; Tania Amin; Octavio Aragon; Katie Bechman; Verna Cuthbert; James Galloway; Patrick Gordon; William J Gregory; Harsha Gunawardena; Michael G Hanna; David Isenberg; John Jackman; Patrick D W Kiely; Polly Livermore; Pedro M Machado; Sue Maillard; Neil McHugh; Ruth Murphy; Clarissa Pilkington; Athiveeraramapandian Prabu; Phoebe Rushe; Stefan Spinty; Joanne Swan; Hasan Tahir; Sarah L Tansley; Paul Truepenny; Yvonne Truepenny; Kishore Warrier; Mark Yates; Charalampia Papadopoulou; Neil Martin; Liza McCann; Hector Chinoy Journal: Rheumatology (Oxford) Date: 2022-05-05 Impact factor: 7.046