Connective Tissue Disorders in Childhood: Are They All the Same?

Navallas, María; Clemente, Emilio J. Inarejos; Iglesias, E.; Rebollo-Polo, Mónica; Antón, Jordi; Navarro, Oscar M.

doi:10.1148/rg.2019180078

Cited by 12 publications

(14 citation statements)

References 64 publications

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“…The musculoskeletal (MSK) system is commonly affected in patients with SLE [ 180 , 181 , 182 , 183 ], and involvement can vary from mild arthralgia to a deforming arthropathy in rare cases [ 180 , 183 , 184 ]. However, arthritis in children with JSLE is most commonly polyarticular and almost always non-deforming and non-erosive [ 180 ].…”

Section: Arthritis Biomarkersmentioning

confidence: 99%

Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus

Greenan-Barrett

Doolan

Shah

et al. 2021

IJMS

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Juvenile systemic lupus erythematosus (JSLE) is characterised by onset before 18 years of age and more severe disease phenotype, increased morbidity and mortality compared to adult-onset SLE. Management strategies in JSLE rely heavily on evidence derived from adult-onset SLE studies; therefore, identifying biomarkers associated with the disease pathogenesis and reflecting particularities of JSLE clinical phenotype holds promise for better patient management and improved outcomes. This narrative review summarises the evidence related to various traditional and novel biomarkers that have shown a promising role in identifying and predicting specific organ involvement in JSLE and appraises the evidence regarding their clinical utility, focusing in particular on renal biomarkers, while also emphasising the research into cardiovascular, haematological, neurological, skin and joint disease-related JSLE biomarkers, as well as genetic biomarkers with potential clinical applications.

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Section: Arthritis Biomarkersmentioning

confidence: 99%

Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus

Greenan-Barrett

Doolan

Shah

et al. 2021

IJMS

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“…Connective tissue diseases (CTD) encompass a group of heterogeneous systemic inflammatory diseases characterised by the presence of circulating autoantibodies and autoimmune-mediated multiorgan system involvement [1]. These diseases are rare in children [2].…”

Section: Paediatric Rheumatological Diseasesmentioning

confidence: 99%

“…Clinical presentations of autoimmune CTD are extremely variable and can be associated with many systemic complications including pulmonary complications [1]. It is difficult to assess the true prevalence of pulmonary complications in CTD as they are often influenced by the acuity, chronicity and complexity of the causative CTD.…”

Section: Paediatric Rheumatological Diseasesmentioning

confidence: 99%

Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?

Ramphul

Gallagher

Warrier

et al. 2020

Breathe

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Systemic connective tissue diseases (CTDs) are characterised by the presence of autoantibodies and multiorgan involvement. Although CTDs are rare in children, they are associated with pulmonary complications, which have a high morbidity and mortality rate. The exact pathophysiology remains unclear. The pleuropulmonary complications in CTD are diverse in their manifestations and are often complex to diagnose and manage.The most common CTDs are discussed. These include juvenile systemic lupus erythematosus, juvenile dermatomyositis, juvenile systemic sclerosis, Sjögren's syndrome and mixed connective tissue disease. We describe the clinical features of the pleuropulmonary complications, focusing on their screening, diagnosis and monitoring. Treatment strategies are also discussed, highlighting the factors and interventions that influence the outcome of lung disease in CTD and pulmonary complications of treatment.Early detection and prompt treatment in a multidisciplinary team setting, including respiratory and rheumatology paediatricians and radiologists, is paramount in achieving the best possible outcomes for these patients.

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“…The identification of CTD is frequently delayed and identifying the exact nature of CTD can be challenging 4 . In SLE, about half of patients have some form of pulmonary immersion throughout the progression of the disease [5][6][7][8] .…”

Section: Introductionmentioning

confidence: 99%

Association of KL-6 Levels with Interstitial Lung Disease in Connective Tissue Diseased Children, case control study

Elbehedy

Domouky

El-Shafaey

et al. 2020

Preprint

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It was aimed to evaluate the correlation between serum KL-6 levels with both presence of interstitial lung disease (ILD) and its severity in connective tissue diseased children, directing to early detection and better management of interstitial lung disease in CTD children. This study included 40 CTD patients divided into with and without ILD as evaluated mainly by high-resolution computed tomography (HRCT), and spirometric pulmonary function test as well as 20 normal controls to detect the expression level of KL-6 by ELISA. KL-6 levels were compared among the different study groups. In comparison of all groups of the study, KL-6 levels were related positively with both presence and severity of ILD at a cut-off of 63.4 U/ml, with sensitivity of 95.2%, specificity of 89.7%, positive predictive value of 83.26% and negative predictive value of 97.2% for ILD in CTD patients. Moreover, there is a significant inverse correlation found between serum KL-6 levels and parameter of spirometry. In conclusion, Serum KL-6 offers high sensitivity and specificity for the diagnosis of ILD in CTD children and is inversely correlated with pulmonary function deterioration. Accordingly, serum KL-6 may represent a promising biomarker for prediction and monitoring of ILD severity.

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Connective Tissue Disorders in Childhood: Are They All the Same?

Cited by 12 publications

References 64 publications

Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus

Biomarkers Associated with Organ-Specific Involvement in Juvenile Systemic Lupus Erythematosus

Why is a paediatric respiratory specialist integral to the paediatric rheumatology clinic?

Association of KL-6 Levels with Interstitial Lung Disease in Connective Tissue Diseased Children, case control study

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