Araştırma Makalesi
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The Clinical, Laboratory, and Imaging Features of Children with Juvenile Dermatomyositis: A Single Center Experience

Yıl 2023, Cilt: 10 Sayı: 2, 76 - 82, 30.06.2023
https://doi.org/10.34087/cbusbed.1185544

Öz

Objective: Juvenile dermatomyositis (JDM) is a rare disease that accounts for the majority of idiopathic inflammatory myositis, typically affecting muscle and skin. Our study aims to evaluate pediatric patients' demographic, clinical, laboratory, and imaging characteristics and treatments followed up with the diagnosis of JDM in a tertiary pediatric rheumatology center.
Materials and Methods: The patients' medical records followed up with the diagnosis of JDM for at least 6 months between 2015- 2021 and were evaluated retrospectively.
Results: Six female and six male patients were included in the study. At the time of diagnosis, 5 patients had mild, 3 patients had moderate and 4 patients had severe clinical findings. During the follow-up, the clinical course was monocyclic in 2 patients, chronic persistent in 7 patients, and polycyclic in 3 patients. All patients had skin and muscle involvement at the time of diagnosis. At least one muscle enzyme elevation was found in 9 patients, CK elevation in 7 patients, LDH elevation in 7 patients, and AST elevation in 5 patients. Calcinosis was observed in 5 patients, joint contracture in 2 patients, and lipodystrophy in 1 patient during the follow-up period. High-dose methylprednisolone was given to 9 patients for 3 days as initial treatment. Seven (58.3) patients received IVIG.
Conclusion: Managing JDM is difficult because of patients are followed by experienced multidisciplinary pediatric rheumatology centers. Documenting existing patient data and presenting multidisciplinary data will be a guide for physicians who have undertaken the management of these patients.

Kaynakça

  • 1. Clarissa A. Pilkington BMF, Watchareewan Sontichai: Juvenile Dermatomyositis and Other Inflammatory Muscle Diseases. In: Textbook of Pediatric Rheumatology. edn. Edited by Ross E. Petty RML, Carol B. Lindsley, Lucy R. Wedderburn, Elizabeth D. Mellins, Robert C. Fuhlbrigge; 2021: 360-375.
  • 2. McCann L, Juggins A, Maillard S, Wedderburn L, Davidson J, Murray K, Pilkington CJR: The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the first 5 yr. 2006, 45(10):1255-1260.
  • 3. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC et al: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis and rheumatism 2013, 65(1):1-11.
  • 4. Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H, Carrasco R, Huber AM, Feldman BM, Reed AM: Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry. Arthritis care & research 2014, 66(3):404-410.
  • 5. Guseinova D, Consolaro A, Trail L, Ferrari C, Pistorio A, Ruperto N, Buoncompagni A, Pilkington C, Maillard S, Oliveira SK et al: Comparison of clinical features and drug therapies among European and Latin American patients with juvenile dermatomyositis. Clinical and experimental rheumatology 2011, 29(1):117-124.
  • 6. Bohan A, Peter JB: Polymyositis and dermatomyositis (first of two parts). The New England journal of medicine 1975, 292(7):344-347.
  • 7. Brown VE, Pilkington CA, Feldman BM, Davidson JE: An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford, England) 2006, 45(8):990-993.
  • 8. Bitnum S, Daeschner CW, Jr., Travis LB, Dodge WF, Hopps HC: DERMATOMYOSITIS. The Journal of pediatrics 1964, 64:101-131.
  • 9. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, Zemel LS, Wallace CA, Ballinger SH, Passo MH et al: Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis and rheumatism 2004, 50(5):1595-1603.
  • 10. Mathiesen PR, Zak M, Herlin T, Nielsen SM: Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients. Clinical and experimental rheumatology 2010, 28(5):782-789.
  • 11. Mathiesen P, Hegaard H, Herlin T, Zak M, Pedersen FK, Nielsen S: Long-term outcome in patients with juvenile dermatomyositis: a cross-sectional follow-up study. Scandinavian journal of rheumatology 2012, 41(1):50-58.
  • 12. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ, Pilkington CA: The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford, England) 2006, 45(10):1255-1260.
  • 13. Okong'o LO, Esser M, Wilmshurst J, Scott C: Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric rheumatology online journal 2016, 14(1):60.
  • 14. Malek A, Raeeskarami SR, Ziaee V, Aghighi Y, Moradinejad MH: Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis. Clinical rheumatology 2014, 33(8):1113-1118.
  • 15. Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD: Clinical features and disease course of patients with juvenile dermatomyositis. International journal of rheumatic diseases 2013, 16(5):561-567.
  • 16. Sato JO, Sallum AM, Ferriani VP, Marini R, Sacchetti SB, Okuda EM, Carvalho JF, Pereira RM, Len CA, Terreri MT et al: A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases. Clinical and experimental rheumatology 2009, 27(6):1031-1038.
  • 17. Wedderburn LR, Varsani H, Li CK, Newton KR, Amato AA, Banwell B, Bove KE, Corse AM, Emslie-Smith A, Harding B et al: International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis: a tool for potential use in clinical trials. Arthritis and rheumatism 2007, 57(7):1192-1201.
  • 18. Stevens BE, Torok KS, Li SC, Hershey N, Curran M, Higgins GC, Moore KF, Egla Rabinovich C, Dodson S, Stevens AM: Clinical Characteristics and Factors Associated With Disability and Impaired Quality of Life in Children With Juvenile Systemic Sclerosis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis care & research 2018, 70(12):1806-1813.
  • 19. Al-Mayouf SM, AlMutiari N, Muzaffer M, Shehata R, Al-Wahadneh A, Abdwani R, Al-Abrawi S, Abu-Shukair M, El-Habahbeh Z, Alsonbul A: Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children. Rheumatology international 2017, 37(9):1513-1517.
  • 20. Stringer E, Singh-Grewal D, Feldman BM: Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis and rheumatism 2008, 58(11):3585-3592.
  • 21. Taborda AL, Azevedo P, Isenberg DA: Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study. Clinical and experimental rheumatology 2014, 32(2):188-193.
  • 22. Tansley SL, Simou S, Shaddick G, Betteridge ZE, Almeida B, Gunawardena H, Thomson W, Beresford MW, Midgley A, Muntoni F et al: Autoantibodies in juvenile-onset myositis: Their diagnostic value and associated clinical phenotype in a large UK cohort. Journal of autoimmunity 2017, 84:55-64.
  • 23. Tansley SL, Betteridge ZE, Shaddick G, Gunawardena H, Arnold K, Wedderburn LR, McHugh NJ: Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology (Oxford, England) 2014, 53(12):2204-2208.
  • 24. Bellutti Enders F, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM, Lahdenne P, Magnusson B, Nistala K, Ozen S et al: Consensus-based recommendations for the management of juvenile dermatomyositis. Annals of the rheumatic diseases 2017, 76(2):329-340.
  • 25. Giancane G, Lavarello C, Pistorio A, Oliveira SK, Zulian F, Cuttica R, Fischbach M, Magnusson B, Pastore S, Marini R et al: The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients. Pediatric rheumatology online journal 2019, 17(1):24.
  • 26. Lam CG, Manlhiot C, Pullenayegum EM, Feldman BM: Efficacy of intravenous Ig therapy in juvenile dermatomyositis. Annals of the rheumatic diseases 2011, 70(12):2089-2094.

Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi

Yıl 2023, Cilt: 10 Sayı: 2, 76 - 82, 30.06.2023
https://doi.org/10.34087/cbusbed.1185544

Öz

Giriş ve Amaç: Juvenil dermatomiyozit (JDM) idiyopatik inflamatuar miyozitlerin büyük çoğunluğunu oluşturan, kas ve cilt tutulumu ile seyreden nadir görülen bir otoimmün miyopatidir. Çalışmamızın amacı merkezimizde JDM tanısı ile takip edilen çocuk hastaların demografik, klinik, laboratuvar, görüntüleme özelliklerinin ve tedavilerinin değerlendirilmesidir.
Gereç ve Yöntemler: Kliniğimizce 2015-2021 tarihleri arasında JDM tanısı ile en az 6 ay takip edilen hastaların verileri geriye yönelik olarak hasta tıbbi kayıtları incelenerek değerlendirildi.
Bulgular: Çalışmaya 6 kız 6 erkek hasta dahil edildi. Başvuru sırasında 5 hasta hafif, 3 hasta orta, 4 hasta ağır klinik bulgular ile tanı aldı. Takip süresince klinik seyir 2 hastada monosiklik, 7 hastada kronik persistan ve 3 hastada polisiklikti. Tüm hastaların tanı sırasında cilt ve kas bulguları mevcuttu, laboratuvar olarak 9 hastada en az bir kas enzimi yüksekliği, 7 hastada CK yüksekliği, 7 hastada LDH yüksekliği ve 5 hastada AST yüksekliği vardı. Takip süresince 5 hastada kalsinozis, 2 hastada eklem kontraktürü ve 1 hastada lipodistrofi gelişimi gözlendi. Başlangıç tedavisi olarak 9 hastaya yoğun yüksek doz metilprednizolon 3 gün süre ile verildi. Takipte yedi (58,3) hastaya IVIG tedavisi verildi.
Sonuç: JDM tedavisi ve takibi zor olan, multidisipliner deneyimli çocuk romatoloji merkezlerince yönetilen bir hastalıktır. Mevcut hasta verilerinin dökümente edilmesi ve multidisipliner verilerin sunulması bu hastaları takip eden hekimler için yol gösterici olacaktır.

Kaynakça

  • 1. Clarissa A. Pilkington BMF, Watchareewan Sontichai: Juvenile Dermatomyositis and Other Inflammatory Muscle Diseases. In: Textbook of Pediatric Rheumatology. edn. Edited by Ross E. Petty RML, Carol B. Lindsley, Lucy R. Wedderburn, Elizabeth D. Mellins, Robert C. Fuhlbrigge; 2021: 360-375.
  • 2. McCann L, Juggins A, Maillard S, Wedderburn L, Davidson J, Murray K, Pilkington CJR: The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the first 5 yr. 2006, 45(10):1255-1260.
  • 3. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC et al: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis and rheumatism 2013, 65(1):1-11.
  • 4. Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H, Carrasco R, Huber AM, Feldman BM, Reed AM: Clinical characteristics of children with juvenile dermatomyositis: the Childhood Arthritis and Rheumatology Research Alliance Registry. Arthritis care & research 2014, 66(3):404-410.
  • 5. Guseinova D, Consolaro A, Trail L, Ferrari C, Pistorio A, Ruperto N, Buoncompagni A, Pilkington C, Maillard S, Oliveira SK et al: Comparison of clinical features and drug therapies among European and Latin American patients with juvenile dermatomyositis. Clinical and experimental rheumatology 2011, 29(1):117-124.
  • 6. Bohan A, Peter JB: Polymyositis and dermatomyositis (first of two parts). The New England journal of medicine 1975, 292(7):344-347.
  • 7. Brown VE, Pilkington CA, Feldman BM, Davidson JE: An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Rheumatology (Oxford, England) 2006, 45(8):990-993.
  • 8. Bitnum S, Daeschner CW, Jr., Travis LB, Dodge WF, Hopps HC: DERMATOMYOSITIS. The Journal of pediatrics 1964, 64:101-131.
  • 9. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD, Zemel LS, Wallace CA, Ballinger SH, Passo MH et al: Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis and rheumatism 2004, 50(5):1595-1603.
  • 10. Mathiesen PR, Zak M, Herlin T, Nielsen SM: Clinical features and outcome in a Danish cohort of juvenile dermatomyositis patients. Clinical and experimental rheumatology 2010, 28(5):782-789.
  • 11. Mathiesen P, Hegaard H, Herlin T, Zak M, Pedersen FK, Nielsen S: Long-term outcome in patients with juvenile dermatomyositis: a cross-sectional follow-up study. Scandinavian journal of rheumatology 2012, 41(1):50-58.
  • 12. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ, Pilkington CA: The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford, England) 2006, 45(10):1255-1260.
  • 13. Okong'o LO, Esser M, Wilmshurst J, Scott C: Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric rheumatology online journal 2016, 14(1):60.
  • 14. Malek A, Raeeskarami SR, Ziaee V, Aghighi Y, Moradinejad MH: Clinical course and outcomes of Iranian children with juvenile dermatomyositis and polymyositis. Clinical rheumatology 2014, 33(8):1113-1118.
  • 15. Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD: Clinical features and disease course of patients with juvenile dermatomyositis. International journal of rheumatic diseases 2013, 16(5):561-567.
  • 16. Sato JO, Sallum AM, Ferriani VP, Marini R, Sacchetti SB, Okuda EM, Carvalho JF, Pereira RM, Len CA, Terreri MT et al: A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases. Clinical and experimental rheumatology 2009, 27(6):1031-1038.
  • 17. Wedderburn LR, Varsani H, Li CK, Newton KR, Amato AA, Banwell B, Bove KE, Corse AM, Emslie-Smith A, Harding B et al: International consensus on a proposed score system for muscle biopsy evaluation in patients with juvenile dermatomyositis: a tool for potential use in clinical trials. Arthritis and rheumatism 2007, 57(7):1192-1201.
  • 18. Stevens BE, Torok KS, Li SC, Hershey N, Curran M, Higgins GC, Moore KF, Egla Rabinovich C, Dodson S, Stevens AM: Clinical Characteristics and Factors Associated With Disability and Impaired Quality of Life in Children With Juvenile Systemic Sclerosis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis care & research 2018, 70(12):1806-1813.
  • 19. Al-Mayouf SM, AlMutiari N, Muzaffer M, Shehata R, Al-Wahadneh A, Abdwani R, Al-Abrawi S, Abu-Shukair M, El-Habahbeh Z, Alsonbul A: Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children. Rheumatology international 2017, 37(9):1513-1517.
  • 20. Stringer E, Singh-Grewal D, Feldman BM: Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features. Arthritis and rheumatism 2008, 58(11):3585-3592.
  • 21. Taborda AL, Azevedo P, Isenberg DA: Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study. Clinical and experimental rheumatology 2014, 32(2):188-193.
  • 22. Tansley SL, Simou S, Shaddick G, Betteridge ZE, Almeida B, Gunawardena H, Thomson W, Beresford MW, Midgley A, Muntoni F et al: Autoantibodies in juvenile-onset myositis: Their diagnostic value and associated clinical phenotype in a large UK cohort. Journal of autoimmunity 2017, 84:55-64.
  • 23. Tansley SL, Betteridge ZE, Shaddick G, Gunawardena H, Arnold K, Wedderburn LR, McHugh NJ: Calcinosis in juvenile dermatomyositis is influenced by both anti-NXP2 autoantibody status and age at disease onset. Rheumatology (Oxford, England) 2014, 53(12):2204-2208.
  • 24. Bellutti Enders F, Bader-Meunier B, Baildam E, Constantin T, Dolezalova P, Feldman BM, Lahdenne P, Magnusson B, Nistala K, Ozen S et al: Consensus-based recommendations for the management of juvenile dermatomyositis. Annals of the rheumatic diseases 2017, 76(2):329-340.
  • 25. Giancane G, Lavarello C, Pistorio A, Oliveira SK, Zulian F, Cuttica R, Fischbach M, Magnusson B, Pastore S, Marini R et al: The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients. Pediatric rheumatology online journal 2019, 17(1):24.
  • 26. Lam CG, Manlhiot C, Pullenayegum EM, Feldman BM: Efficacy of intravenous Ig therapy in juvenile dermatomyositis. Annals of the rheumatic diseases 2011, 70(12):2089-2094.
Toplam 26 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Romatoloji ve Artrit
Bölüm Araştırma Makalesi
Yazarlar

Figen Çakmak 0000-0002-1667-2480

Fatma Gül Demirkan 0000-0001-9950-2489

Nuray Aktay Ayaz 0000-0003-3594-7387

Yayımlanma Tarihi 30 Haziran 2023
Yayımlandığı Sayı Yıl 2023 Cilt: 10 Sayı: 2

Kaynak Göster

APA Çakmak, F., Demirkan, F. G., & Aktay Ayaz, N. (2023). Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi, 10(2), 76-82. https://doi.org/10.34087/cbusbed.1185544
AMA Çakmak F, Demirkan FG, Aktay Ayaz N. Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi. CBU-SBED. Haziran 2023;10(2):76-82. doi:10.34087/cbusbed.1185544
Chicago Çakmak, Figen, Fatma Gül Demirkan, ve Nuray Aktay Ayaz. “Juvenil Dermatomiyozit Tanısı Ile Takipli Çocuk Hastaların Klinik, Laboratuvar Ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 10, sy. 2 (Haziran 2023): 76-82. https://doi.org/10.34087/cbusbed.1185544.
EndNote Çakmak F, Demirkan FG, Aktay Ayaz N (01 Haziran 2023) Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 10 2 76–82.
IEEE F. Çakmak, F. G. Demirkan, ve N. Aktay Ayaz, “Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi”, CBU-SBED, c. 10, sy. 2, ss. 76–82, 2023, doi: 10.34087/cbusbed.1185544.
ISNAD Çakmak, Figen vd. “Juvenil Dermatomiyozit Tanısı Ile Takipli Çocuk Hastaların Klinik, Laboratuvar Ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 10/2 (Haziran 2023), 76-82. https://doi.org/10.34087/cbusbed.1185544.
JAMA Çakmak F, Demirkan FG, Aktay Ayaz N. Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi. CBU-SBED. 2023;10:76–82.
MLA Çakmak, Figen vd. “Juvenil Dermatomiyozit Tanısı Ile Takipli Çocuk Hastaların Klinik, Laboratuvar Ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi, c. 10, sy. 2, 2023, ss. 76-82, doi:10.34087/cbusbed.1185544.
Vancouver Çakmak F, Demirkan FG, Aktay Ayaz N. Juvenil Dermatomiyozit Tanısı ile Takipli Çocuk Hastaların Klinik, Laboratuvar ve Görüntüleme Bulgularının Değerlendirilmesi: Tek Merkez Deneyimi. CBU-SBED. 2023;10(2):76-82.