OBJECTIVE: Our aim is to present the disease course, frequency of relapses and survival of juvenile and adult dermatomyositis (JDM/DM) patients. METHODS: Analysis was performed using data on 73 patients. The median follow-up for 38 JDM patients was 32 months and 78 months for 35 adult DM patients. RESULTS: 23/38 JDM patients (60%) had monophasic, 12/38 (31.6%) had polycyclic and 3/38 (7.9%) had chronic disease. Among children treated only with glucocorticoids, 12/20 (60%) had monophasic and 8/20 (40%) had polycyclic disease. 10/17 (58.8%) children, who required second-line immunosuppressive agents, had monophasic and 4/17 (23.5%) had polycyclic disease. 18/35 DM (51.4%) patients had monophasic, 13/35 (37.1%) had polycyclic, 1/35 (2.9%) had chronic disease and 3/35 (8.6%) had fulminant myositis. Among DM patients requiring only glucocorticoids, 12/20 (60%) were monophasic and 8/20 (40%) were polycyclic. In patients requiring second-line immunosuppressive agents, 6/15 patients (40%) had monophasic and 5/15 (33.3%) had polycyclic disease. Among patients with polycyclic disease, the risk of relapse was higher during first year than later in the disease course. None of the JDM patients have died, while 4 disease-specific deaths occurred in adult patients. There was no significant difference between the survival of JDM and DM patients. DISCUSSION: There was no correlation between relapse-free survival and the initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed for at least 2 years. Although we found a favourable survival rate, further investigations are needed to assess functional outcome.
OBJECTIVE: Our aim is to present the disease course, frequency of relapses and survival of juvenile and adult dermatomyositis (JDM/DM) patients. METHODS: Analysis was performed using data on 73 patients. The median follow-up for 38 JDM patients was 32 months and 78 months for 35 adult DMpatients. RESULTS: 23/38 JDM patients (60%) had monophasic, 12/38 (31.6%) had polycyclic and 3/38 (7.9%) had chronic disease. Among children treated only with glucocorticoids, 12/20 (60%) had monophasic and 8/20 (40%) had polycyclic disease. 10/17 (58.8%) children, who required second-line immunosuppressive agents, had monophasic and 4/17 (23.5%) had polycyclic disease. 18/35 DM (51.4%) patients had monophasic, 13/35 (37.1%) had polycyclic, 1/35 (2.9%) had chronic disease and 3/35 (8.6%) had fulminant myositis. Among DMpatients requiring only glucocorticoids, 12/20 (60%) were monophasic and 8/20 (40%) were polycyclic. In patients requiring second-line immunosuppressive agents, 6/15 patients (40%) had monophasic and 5/15 (33.3%) had polycyclic disease. Among patients with polycyclic disease, the risk of relapse was higher during first year than later in the disease course. None of the JDM patients have died, while 4 disease-specific deaths occurred in adult patients. There was no significant difference between the survival of JDM and DMpatients. DISCUSSION: There was no correlation between relapse-free survival and the initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed for at least 2 years. Although we found a favourable survival rate, further investigations are needed to assess functional outcome.
Authors: G Esther A Habers; Adam M Huber; Gulnara Mamyrova; Ira N Targoff; Terrance P O'Hanlon; Sharon Adams; Janardan P Pandey; Chantal Boonacker; Marco van Brussel; Frederick W Miller; Annet van Royen-Kerkhof; Lisa G Rider Journal: Arthritis Rheumatol Date: 2016-03 Impact factor: 10.995
Authors: Nicholas E Johnson; W David Arnold; Donald Hebert; Kelly Gwathmey; Mazen M Dimachkie; Richard J Barohn; April L McVey; Mamatha Pasnoor; Anthony A Amato; Michael P McDermott; John Kissel; Chad R Heatwole Journal: Neuromuscul Disord Date: 2015-05-07 Impact factor: 4.296
Authors: Anjali Patwardhan; Robert Rennebohm; Igor Dvorchik; Charles H Spencer Journal: Pediatr Rheumatol Online J Date: 2012-09-20 Impact factor: 3.054
Authors: C H Spencer; K Rouster-Stevens; H Gewanter; G Syverson; R Modica; K Schmidt; H Emery; C Wallace; S Grevich; K Nanda; Y D Zhao; S Shenoi; S Tarvin; S Hong; C Lindsley; J E Weiss; M Passo; K Ede; A Brown; K Ardalan; W Bernal; M L Stoll; B Lang; R Carrasco; C Agaiar; L Feller; H Bukulmez; R Vehe; H Kim; H Schmeling; D Gerstbacher; M Hoeltzel; B Eberhard; R Sundel; S Kim; A M Huber; A Patwardhan Journal: Pediatr Rheumatol Online J Date: 2017-06-13 Impact factor: 3.054