Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study

Hetlevik, Siri Opsahl; Flatø, Berit; Rygg, Marite; Nordal, Ellen; Brunborg, Cathrine; Hetland, Helene; Lilleby, Vibke

doi:10.1136/annrheumdis-2016-209522

Cited by 41 publications

(30 citation statements)

References 27 publications

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“…Hetlevik et al [19] reported presence of fever at the onset of the disease in 29% of children, arthritis in 94% of children, presence of rheumatoid factor in 64%, edema in hands in 77%, weakening of muscles in over 40%, sclerodactyly in 10%, lung function impairment in 58% of children. 15% of children had remission, active disease was diagnosed in 67%.…”

Section: Resultsmentioning

confidence: 99%

Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

Rutkowska-Sak¹,

Gietka²

2019

Reumatologia

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Objectives: Mixed connective tissue disease is a rare systemic connective tissue disease of developmental age and it includes the features of arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus and systemic sclerosis, with presence of anti-ribonucleoprotein antibodies (anti-RNP) in serum. Early diagnosis of the disease is difficult but essential in preventing development of systemic complications, which are often irreversible. International literature does not report many studies on large cohorts of children with this disease. The aim of this retrospective study was to define clinical characteristics and long-term results of treatment of the disease in 60 children with mixed connective tissue disease hospitalized in the period between 1978 and 2018. The diagnosis was established on the basis of Kasukawa's criteria. Material and methods: It was a group of 60 children (46 girls and 16 boys) aged 10.5 on average (4-16.5). When assessing general symptoms at the onset of the disease according to Kasukawa's criteria, the highest number, over 80% of children, demonstrated symptoms suggesting SLE, about 40% suggesting DM and about 25% suggesting SSC. In the period of observation the number of children with clinical symptoms suggesting SSC increased. The most common clinical symptoms included Raynaud syndrome, arthritis and myositis and the most common irregularities in the test results included presence of anti-RNP antibodies and rheumatoid factor and hematological symptoms such as leukopenia/thrombocytopenia. Restrictive lung function impairment was demonstrated by 20% of children. Treatment most often included combined therapy (glucocorticosteroids + methotrexate/azathioprine). Results: In 70% of the patients stable improvement was observed. Remission concerned 7% of the patients, frequent exacerbations were found in almost 20% of patients, and 2 children (3.5%) died. Conclusions: The long term observations of patients in developementeal age with mixed connective tissue disease revealed that the majority of them had domination of SLE symptoms, only in 7% achieved remission and 70% remained in stable improvement. Serious infections with septic state were the cause of death in two cases.

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Section: Resultsmentioning

confidence: 99%

Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

Rutkowska-Sak¹,

Gietka²

2019

Reumatologia

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“…The main strength of our study was that it was the first to compare juvenile MCTD patients with a control group. It was also the largest study to assess HRCT and PFT systematically in all patients, and the cohort is believed to be representative of juvenile MCTD patients living in Norway, as described in more detail previously (17). Progression of pulmonary manifestations in juvenile MCTD patients has not previously been reported, and we had a long follow-up of 16.2 years from the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease: A Controlled Study

Witczak

Hetlevik²,

Sanner³

et al. 2019

J Rheumatol

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Objective.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.Methods.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.Results.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.Conclusion.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.

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“…Patients were identified through multiple acquisition routes and a nationwide approach, identification and inclusion have been described in detail previously (17). Sixty two patients were initially identified, of these patients three were deceased, three did not wish to participate, one did not respond to our enquiries, and three had developed clinical SLE and were excluded.…”

Section: Patients and Controlsmentioning

confidence: 99%

Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease

et al. 2018

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Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study

Abstract: Most patients with JMCTD had active disease and organ damage after a mean follow-up of 16.2 years. Active disease was associated with higher anti-ribonucleoprotein antibody levels and positive RF. The presence of RF at diagnosis predicted persistent disease activity.

Cited by 41 publications

References 27 publications

Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

Clinical features and outcome of mixed connective tissue disease in developmental age – observational study from one center

Effect on Cardiac Function of Longstanding Juvenile-onset Mixed Connective Tissue Disease: A Controlled Study

Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease

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