Acute Presentation of Juvenile Dermatomyositis with Subclinical Cardiac Involvement: A Rare Case (original) (raw)
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Juvenile dermatomyositis presenting as complete heart block in a 10-year-old girl
Paediatrics and International Child Health, 2020
Juvenile dermatomyositis (JDM) is an auto-immune inflammatory condition associated with cardiac disorders including conduction abnormalities and myocardial dysfunction. The time of presentation of cardiac abnormalities can range from disease onset to after long-term followup, emphasising the importance of screening for cardiac involvement in JDM. A previously healthy 10-year-old girl presented with syncope, fatigue and weakness associated with a heliotrope rash. JDM was diagnosed based on the clinical, laboratory and imaging findings. An ECG demonstrated complete heart block (CHB). All symptoms resolved following treatment with parenteral corticosteroids. In JDM, it is important to investigate for cardiac manifestations and in CHB to consider administering corticosteroids.
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.
Juvenile dermatomyositis case report from the albert royer national
Juvenile dermatomyositis (JD) is a rare multi-systemic inflammatory idiopathic disease characterised by primary cutaneous and muscular involvement. There is a paucity of literature concerning children. We report the case of a 7-year-old girl. The diagnosis was made following hospitalisation for respiratory distress with a fever of 39°C and deterioration in general condition with general muscle wasting and relative functional impotence. Examination revealed flaccid quadriplegia, significant muscular amyotrophy and ankylosis with pain on mobilisation of the joints. Hypochromic macules associated with Gottron papules were also found. Biochemistry revealed elevated muscle enzymes and LDH. EMG revealed myogenic patterns and muscle biopsy revealed inflammatory infiltrates in the endomysium and connective tissue. Treatment included prednisone, methotrexate and folic acid. The short- and medium-term outcome was favourable. Death occurred 20 months later at home from an unclear cause.
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.
Juvenile Dermatomyositis in Iranian Children; a Case Series Report
Annals of Paediatric Rheumatology, 2012
Objective: Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children, distinguished by proximal muscle weakness and a characteristic rash. Underlying pathology in this disorder is infiltration of inflammatory cells in microscopic vessels of muscles and skin resulting in vascular inflammation. Extramuscular manifestations include joint contracture, dysphagia, cardiac disturbances, pulmonary symptom and subcutaneous calcifications. The study describes the clinical features and outcomes of juvenile dermatomyositis (JDM) in Iranian children and compares these with findings reported by similar studies from other parts of the world.
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...
Actuality of juvenile dermatomyositis
Joint Bone Spine, 2011
Juvenile dermatomyositis is a rare disorder, but remains the most commonly occurring chronic inflammatory myopathy among children. Other than the proximal muscles and skin, which are routinely affected, vasculopathy may affect other viscera and can be multisystemic. A redefinition of the diagnostic criteria is currently underway and is likely to lead to other clinical signs and to sensitive and non-invasive examinations such as MRI. The impact of juvenile dermatomyositis on health and quality of life remains significant despite systemic corticosteroid therapy and immunosuppressor treatment, which have considerably improved the prognosis. Numerous predictors for favourable and pejorative evolution have been identified. The standardisation and the generalisation of clinical assessment tools will make it possible to carry out the clinical trials required to determine the relevance of the new therapeutic options available for children.
A rare complication of generalized edema in juvenile dermatomyositis: a report of one case
Brain & Development, 2004
Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by inflammation of the muscle, connective tissue, skin, gastrointestinal tract and small nerves. Periorbital and facial edema may also be associated. Although localized edema is a common feature of juvenile dermatomyositis, generalized edema has been reported rarely. In this article, we report a 14-year-old boy with juvenile dermatomyositis presenting with generalized edema. Of the diagnostic criteria of JDM, severe symmetric weakness of the proximal musculature, characteristic cutaneous changes, elevated serum muscle enzymes and myopathic electromyographic abnormalities were observed. Magnetic resonance imaging (MRI) of the lower extremities and pelvis showed marked diffuse edema in the subcutaneous tissue, muscles and myofascia. We suggest that MRI findings, which are not among the diagnostic criteria, may also be included in the diagnostic criteria of JDM. To the best of our knowledge, this is the 19th case of JDM reported for generalized edema in the English literature. q
Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy
Current Treatment Options in Rheumatology, 2021
Purpose of reviewTo identify clues to disease activity and discuss therapy options.Recent findingsThe diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavail...
Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood
The Lancet, 2008
Juvenile dermatomyositis, the most common infl ammatory myopathy of childhood, is a rare systemic autoimmune vasculopathy that is characterised by weakness in proximal muscles and pathognomonic skin rashes. The length of time before the initiation of treatment aff ects presenting symptoms, laboratory measures, and pathophysiology. It also aff ects disease outcomes, including the development of pathological calcifi cations, which are associated with increased morbidity. Both genetic and environmental risk factors seem to have a role in the cause of juvenile dermatomyositis; HLA B8-DRB1*0301 ancestral haplotype is a strong immunogenetic risk factor, and antecedent infections and birth seasonality suggest that environmental stimuli might increase risk. Activation of dendritic cells with upregulation of genes induced by type-1 interferon (α) in muscle and peripheral blood seems to be central to disease pathogenesis. Treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents. Disease outcome, if treatment is initiated early, is generally good. Randomised controlled trials are needed to defi ne the most eff ective treatments.