Juvenile Dermatomyositis and Scleroderma
Özet
Dermatomyositis is an autoimmune inflammatory disease affecting the skin and skeletal muscles. It develops as a result of an interaction of genetic, environmental and immunological factors. Cardiomyopathy, myocarditis, arrhythmias may develop.
Scleroderma is a disease characterized by sclerosis of the skin; it is divided into two groups as systemic sclerosis and localized scleroderma. It is thought that genetic, autoimmune and metabolic factors play a role in its etiology. The clinical finding is limited to the skin, subcutaneous and muscle tissues in localized scleroderma; visceral involvement and vascular damage are seen in systemic scleroderma. Reynaud phenomenon is the first finding in most patients. Cardiac effects develop as a result of cardiac fibrosis; reflected in the clinic as pericarditis, myocardial fibrosis, myocarditis, arrhythmias. Pulmonary hypertension is an important finding of systemic sclerosis and may occur as a result of different pathophysiological causes: precapillary (pulmonary vascular disease), intrapulmonary (interstitial lung disease), postcapillary (myocardial dysfunction). Pulmonary hypertension in systemic sclerosis should be monitored and treated by considering these different causes.
Cardiac abnormalities in patients with dermatomyositis and scleroderma are significant cause of morbidity and mortality. Cardiac screening should be performed from the moment of diagnosis and a multidisciplinary approach to treatment should be adopted.
Dermatomiyozit, deri ve iskelet kaslarını etkileyen otoimmün iltihaplı bir hastalıktır. Genetik, çevresel ve immünolojik faktörlerin bir etkileşimi sonucu gelişir. Kardiyomiyopati, miyokardit, aritmiler gibi durumlar gelişebilir.
Skleroderma cildin sklerozisi ile karakterize bir hastalık olup; sistemik sklerozis ve lokalize skleroderma olmak üzere iki gruba ayrılır. Etiyolojisinde genetik, otoimmün ve metabolik faktörlerin rol oynadığı düşünülmektedir. Lokalize sklerodermada klinik cilt, cilt altı ve kas dokular ile sınırlıyken; sistemik sklerodermada visseral tutulum ve vasküler hasar görülmektedir. Hastaların çoğunda Reynaud fenomeni ilk bulgu olarak görülmektedir. Kardiyak etkileri kardiyak fibrozis sonucu gelişmekle birlikte perikardit, miyokardiyal fibrozis, miyokardit, aritmiler olarak kliniğe yansımaktadır. Pulmoner hipertansiyon sistemik sklerozun önemli bir bulgusudur. Pulmoner hipertansiyon farklı fizyopatolojik nedenlerin sonucu olarak ortaya çıkabilmektedir: prekapiller (pulmoner vasküler hastalık), intrapulmoner (interstisyel akciğer hastalığı), postkapiller (miyokardiyal disfonksiyonu). Sistemik sklerozda pulmoner hipertansiyonun bahsedilen farklı nedenleri göz önünde bulundurularak takip ve tedavisi yapılmalıdır.
Dermatomiyozit ve skleroderma hastalarında kardiyak anormallikler, hastaların önemli bir morbidite ve mortalite kaynağıdır. Bu nedenle, tanı anından itibaren kalp taramaları yapılmalı ve tedaviye yönelik çok disiplinli bir yaklaşım benimsenmelidir.
Referanslar
Wedderburn LR, Rider LG. Juvenile Dermatomyositis: New Developments in Pathogenesis, Assessment and Treatment. Best Practice and Research Clinical Rheumatology. 2009; 23(5):665-678. doi:10.1016/j.berh.2009.07.007
Gara S, Jamil RT. Muse ME et al. Juvenile Dermatomyositis. StatPearls Publishing. 2023(1)
Cantez S, Gros GJ, MacLusky I et al. Cardiac findings in children with juvenile dermatomyositis at disease presentation. Pediatric Rheumatology. 2017; 15:54. doi: 10.1186/s12969-017-0182-0
Schwartz T, Diederichsen LP, Lundberg IE, et al. Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies. Rheumatic and Musculoskeletal Diseases. Open 2016; 2:e000291. doi: 10.1136/rmdopen-2016- 000291
Deveza LMA, Miossi R, Carlos de Souza FH. Electrocardiographic changes in dermatomyositis and polymyositis. Revista Brasileira De Reumatologia. 2016; 56(2):95–100. doi: 10.1016/j.rbre.2014.08.012
Barrón-Calvillo EE, García-Romero MT. Early-onset juvenile dermatomyositis: A report of two cases and review of the literature. 2022. doi: 10.1111/pde/14930
Torok KS. Pediatric Scleroderma –Systemic and Localized Forms. Pediatric Clinics of North America. 2012; 59(2):381–405. doi: 10.1016/j.pcl.2012.03.011
Zulian F, Martini G. Systemic sclerosis in children. Future Rheumatol. 2007: 2(1);51-60. doi:10.2217/17460816.2.1.51
Zulian F, Culpo R, Sperotto F et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Annals of the Rheumatic Diseases. 2019; 78:1019–1024. doi:10.1136/annrheumdis-2018-214697
Qudsiya Z, Waseem M. Dermatomyositis. StatPearls Publishing. 2022(8)
Herrick AL. Evidence-based management of Raynaud’s phenomenon. Therapeutic Advances in Musculoskeletal Disease. 2017; 9(12):317–329. doi: 10.1177/1759720x17740074
Mondal S, Barman P, Vignesh P. Cardiovascular abnormalities in juvenile dermatomyositis: a scoping review fort he clinical rheumatologists. Frontiers in Medicine. 2022; 9. doi: 10.3389/fmed.2022.827539
Lundberg IE. The heart in dermatomyositis and polymyositis. Rheumatology. 2006; 45:iv18-iv21. doi: 10.1093/rheumatology/kel311
Peter A, Balogh A, Csanadi Z et al. Subclinical systolic and diastolic myocardial dysfunction in polyphasic polymyositis/ dermatomyositis: a 2-year longitudinal study. Arthritis Research and Therapy. 2022; 24:219. doi:10.1186/s13075-022-02906-7
Deveza LMA, Miossi R, Carlos de Souza FH. Electrocardiographic changes in dermatomyositis and polymyositis. Revista Brasileira De Reumatologia. 2016; 56(2):95–100. doi: 10.1016/j.rbre.2014.08.012
AbdElAl IT, Ali AA. Echocardiographic evaluation of cardiac dysfunction in juvenile dermatomyositis patients. International Journal of Advanced Research. 2016; 4(6);841-846. doi: 10.21474/IJAR01
Apitz C, Girschick H. Systemic sclerosis-associated pulmonary arterial hypertension in children. Cardiovascular Diagnosis and Therapy. 2021; 11(4):1137-1143. doi:10.21037/cdt-20-901
Penmetsa GK, Sapra A. Morphea. StatPearls Publishing. 2023(2)
Mater HAV, Rabinovich CE. Scleroderma and Raynaud phenomenon. Nelson Textbook of Pediatrics. Kliegman RM. Philadelphia. Elsevier. 2016; 1186-1190.
Ergüven M, Akarlar Katıöz Y, Pelit M et al. Çocukluk döneminde skleroderma. Göztepe Tıp Dergisi. 2003; 18:110-112.
Careta MF. Localized scleroderma: clinical spectrum and therapeutic update. Anais Brasilleiros de Dermatologia. 2015; 90(1):62-73. doi: 10.1590/abd1806-4841.20152890
Rodríguez-Salgado P, García-Romero MT. Morphea: a practical review of its diagnosis, classification and treatment. Gaceta Medica De Mexico. 2019; 155:483-491. doi: 10.24875/GMM.M20000336
Quartier P, Bonnet D, Fournet JC et al. Severe Cardiac Involvement in Children with Systemic Sclerosis and Myositis. The Journal of Rheumatology. 2002: 29:1767–73.
Sevimli İ., Öztürk P, Mülayım MK et al. Late Onset Linear Sclorederma. Turkish Journal of Family medicine and Primary Care. 2018; 12(2): 153-155. doi:10.21763/tjfmpc.432556
Bielous-Wilk A, Poreba M, Staniszewska-Marszalek E et al. Electrocardiographic Evaluation in Patients with Systemic Scleroderma and without Clinically Evident Heart Disease. Annals of Noninvasive Electrocardiol. 2009; 14(3):251-257
Avouac J, Huscher D, Furst DE, et al. Expert consensus for performing right heart catheterisation for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis. Annals of the Rheumatic Diseases. 2014; 73:191–197. doi:10.1136/annrheumdis-2012-202567
