Juvenile dermatomyositis presenting as complete heart block in a 10-year-old girl (original) (raw)
Related papers
Acute Presentation of Juvenile Dermatomyositis with Subclinical Cardiac Involvement: A Rare Case
Journal of Clinical and Diagnostic Research, 2016
A seven-year-old boy presented with pruritic erythematous rashes over his face since past 6 week with complain of not being able to sit, stand or take turns in bed and severe myalgia since past 4 week. Bilateral flexion deformity at knee, ankle, elbow, hip, wrist and interphalangeal joint with dysphagia for solid food was present since 2 weeks. There was no history of similar complaints in past. There was no complain of any trauma, fever, photosensitivity, change in voice or vision, breathlessness, abnormal behaviour and altered urinary or bowel habit. The child was born of non-consanguineous marriage and family history was not significant.
Cardiac dysfunction in juvenile dermatomyositis: a case-control study
Annals of the Rheumatic Diseases, 2011
Objective To compare cardiac function in patients with juvenile dermatomyositis (JDM) with matched controls, and examine associations between pathological electrocardiography (ECG), echocardiographic abnormalities and disease variables in patients with JDM. Methods A total of 59 patients with JDM, examined a median 16.8 years (range 2-38 years) after disease onset, were compared with 59 age-matched and sex-matched controls. Echocardiography, including early diastolic transmitral fl ow/early diastolic tissue velocity (E/E') as a marker for diastolic dysfunction, and 12-channel ECG were performed and analysed blinded to patient information. Disease activity and damage were assessed by clinical examination at follow-up and chart review. Results E/E' was elevated (>9.5) in 13 (22%) patients versus 0 controls (p<0.001). In all, 10 patients presented with pathological ECG compared to 4 controls (p=0.054). Previous or current hypertension was found in 12 patients versus 0 controls (p<0.001). Among the patients, pathological ECG was found in 6/13 patients with versus 4/44 without elevated E/E' (p=0.002); and systolic blood pressure was correspondingly 132±24 mm Hg versus 112±18 mm Hg in the groups (p=0.012). E/E' correlated with cumulative organ damage assessed at follow-up (r sp 0.41, p=0.001) and disease activity at 1 year (r sp 0.56, p<0.001), which also predicted pathological E/E' after controlling for age and gender. During disease course, 12% of patients with JDM developed pericarditis. Conclusion Only patients with JDM and no controls had subclinical left ventricular diastolic dysfunction; the patients with elevated E/E' also had high prevalence of pathological ECG and hypertension. High disease activity 1-year post diagnosis predicted high E/E' at follow-up. The fi ndings suggest that subclinical heart disease is related to the systemic nature of JDM.
ECHOCARDIOGRAPHIC EVALUATION OF CARDIAC DYSFUNCTION IN JUVENILE DERMATOMYOSITIS PATIENTS
Background:-Cardiac involvement is common in adult polymyositis (PM) and dermatomyositis(DM). Similarly, pediatric patients with juvenile dermatomyositis (JDM) can be affected. Echocardiography is a valuable tool to assess cardiac dysfunction and to detect subtle signs of cardiac damage in these patients. Objective:-The aim of this study was to assess left ventricular diastolic and systolic function in patients with JDM by echocardiography and to determine whether cardiac function and/or pathological ECG correlated with disease activity parameters. Subjects and methods:-We evaluated 30 patients fulfilling the criteria for dermatomyositis including 7 males, and 23 females (6-27 years age) and compared them to thirty age and sex matched healthy control subjects (5.5-28 years age). Disease activity was measured by disease activity score (DAS) for JDM. Cumulative organ damage was measured by the Myositis Damage Index (MDI). A 12-channelel ectrocardiography (ECG) and echocardiographic assessment of conventional systolic and diastolic LV functions as well as tissue Doppler imaging (TDI). Long axis strain and E' were used to reflect systolic and diastolic function respectively. Results:-Our results showed significant difference between patients and controls as regard pathological ECG findings (26.6% in patients vs 3.3% in controls) and it correlated with disease duration. Echocardiography of patients with juvenile JDM showed significant difference between patients and controls as regard long axis strain, E', and E/E'.There were no significant difference between patients and controls as regard other left ventricle dimensions and ejection fraction. Our results showed significant correlation between echocardiographic findings of systolic and diastolic dysfunction (long axis strain and E'), and clinical parameters including patient age, disease duration, DAS, MDI, prednisolone dose , and not with systolic blood pressure and CHAQ/HAQ. Conclusion:-Patients with JDM showed subclinical systolic and diastolic cardiac dysfunction detected by echocardiography and this correlated well with disease activity.
Rheumatology International
This study aimed at exploring the association between detectable cardiac and pulmonary involvement in long-term juvenile dermatomyositis (JDM) and to assess if patients with cardiac and pulmonary involvement differ with regard to clinical characteristics. 57 JDM patients were examined mean 17.3 (10.5) years after disease onset; this included clinical examination, myositis specific/associated autoantibodies (immunoblot), echocardiography, pulmonary function tests and high-resolution computed tomography. Cardiac involvement was defined as diastolic and/or systolic left ventricular dysfunction and pulmonary involvement as low diffusing capacity for carbon monoxide, low total lung capacity and/or high-resolution computed tomography abnormalities. Patients were stratified into the following four groups: (i) no organ involvement, (ii) pulmonary only, (iii) cardiac only, and (iv) co-existing pulmonary and cardiac involvement. Mean age was 25.7 (12.4) years and 37% were males. One patient h...
Annals of the Rheumatic Diseases, 2013
Objective To compare systolic cardiac function in patients with juvenile dermatomyositis ( JDM) with matched controls and examine associations between systolic and diastolic cardiac function and disease variables. Methods Fifty-nine patients, examined at follow-up, median 16.8 years (2-38 years) after disease onset, were compared with 59 age-matched and sex-matched controls. Echocardiography was performed and analysed blinded to patient information. We used mitral annulus displacement to assess the relative long-axis shortening of the left ventricle (long-axis strain) and early diastolic tissue velocity (e 0 ), as markers for systolic and diastolic function, respectively. Disease activity and organ damage were assessed at follow-up by clinical examination and retrospectively by chart review. Results Long-axis strain was reduced in patients compared with controls (16.6% (2.5) vs 17.7% (2.0), mean (SD), p=0.001), whereas no difference was seen between patients with active and inactive disease. Disease duration correlated with systolic and diastolic function (r sp =−0.50 and r sp =−0.73, both p<0.001) and so did Myositis Damage Index (MDI) 1 year (r sp =−0.36 and r sp =−0.46) and MDI at follow-up (r sp =−0.33 and r sp =−0.60), all p<0.01. High early disease activity score (DAS) in skin (DAS skin 1 year), but not in muscle, predicted systolic (standardised β=−0.28, p=0.011, R 2 =48%) and diastolic dysfunction (β=−0.36, p<0.001, R 2 =72%) at follow-up. Conclusions Long-axis strain was reduced in JDM patients compared with controls, suggesting systolic dysfunction. Impaired systolic and diastolic function was predicted by DAS skin 1 year. This indicates a common pathway to two different cardiac manifestations in JDM, perhaps with similar pathogenesis as skin affection.
Dermatomyositis and supraventricular tachycardia
International Archives of Medicine, 2008
Background: Dermatomyositis is an idiopathic inflammatory myopathy, often associated with an underlying malignancy. Its prevalence rate is approximately one per 100,000 in the general population, and is even rarer without evidence of a cancer. Dermatomyositis rarely involves myocardial muscle fibers, but has shown to be associated with cardiac arrhythmias.
A dermatopathic Juvenile Dermatomyositis; An Unexpected Case in Childhood
Iranian Journal of Child Neurology, 2020
Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents with skin rashes along with muscle weakness. We report a case of JDM in a 10- year-old girl with no skin manifestations presenting with progressive muscle weakness and fatigue. Further laboratory investigations, along with a muscle biopsy, confirmed the diagnosis of adermatopathic JDM. The patient was treated with intravenous immunoglobulin, corticosteroids, methotrexate, hydroxychloroquine, pamidronate, and rituximab. Following treatment, patients’ symptoms subsided, and she gained normal muscular strength over a year.
Successful treatment of cardiac involvement in dermatomyositis with rituximab
Joint Bone Spine, 2008
Polymyositis and dermatomyositis are autoimmune inflammatory myopathies characterized by muscle weakness and inflammation. Current recommended therapy includes corticosteroids as mainstay treatment in addition to immunosuppressant. We present herein a 25 year-old female with dermatomyositis and cardiac involvement resistant to disease modifying anti-rheumatic drugs and anti-tumor necrosis factor-a. She was treated with anti-CD20 monoclonal antibody, rituximab. The patient demonstrated a remarkable clinical and laboratory response. B-cell depletion therapy with rituximab may be a viable option in patients with dermatomyositis and heart disease.
Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids
The Journal of Pediatrics, 2010
Objective-To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids. Study design-A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic and ancillary treatments were recorded. Results-Patients in the no corticosteroid group were followed for a median of 2.8 years (range 2.1-9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (MTX) (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than one year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients developed calcinosis (at 1 and 9 years of disease); however, no patient developed joint contractures, muscle atrophy, lipodystrophy, or functional limitations. Conclusion-Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as MTX and IVIG may be prescribed to effectively treat JDM and prevent complications.