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SKLERODERMA'NIN EŞLİK ETTİĞİ DERMATOMİYOZİT'TE NORMOTANSİF RENAL KRİZ

NORMOTENSİVE RENAL CRISIS IN SCLERODERMA OVERLAPPED DERMATOMYOSITIS

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 1996

Key Words:

Keywords (Original Language):

Author Name
Abstract (2. Language): 
Dermatomyositis (DM) is the nonsuppurative inflammation of the skeletal muscle with dermal rashes. One third of the cases have an overlapping connective tissue disease or malignancy may be accompanying in 10% of them. Scleroderma (SCL); is a connective tissue disease of unknown aetiology, may be seen alone or with overlap syndromes, characterized by systemic fibrosis. A serious and life-threatening complication of SCL is "Scleroderma Renal Crisis" which is characterized by accelerated-malignant hypertension. Normotensive renal crisis may be seen in glucocorticoid-treated SCL patients. Normotensive SCL renal crisis is a syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, pulmonary hemorrhage, recent prior corticosteroid use and poor outcome. Here we report a case of 22-year old scleroderma-overlapped dermatomyositis whose diagnosis established on Bohan-Peter criteria and treated with corticosteroids. She developed acute renal failure (ARF) in the fifth week of the treatment. She was given high dose captopril after the diagnosis of SCL kidney established by renal biopsy. Although she was maintained on haemodialysis five times, she had a fatal outcome on the fourteenth day of normotensive ARF.
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Abstract (Original Language): 
Dermatomiyozit (DM) etyolojisi bilinmeyen ve deri döküntüleri ile seyreden, iskelet kasının nonsüpüratif inflamasyonudur. DM olgularının ip'üne diğer kollajen doku hastalıkları (Overlap Sendromları), 1110'una da maligniteler eşlik eder. Skleroderma (SCL) etyolojisi bilinmeyen, tek başına ya da overlap sendromları içinde yer alan, sistemik fibrozis ile seyreden bir bağ dokusu hastalığıdır. Yaşamı tehdit eden en önemli komplikasyonlanndan birisi akselere-malign hipertansiyon ile karakterize olan "Skleroderma Renal Krizi"dir. Glukokortikoid uygulanan SCL hastalarında normotansif renal krize de rastlanmaktadır. Normotansif SCL renal krizi; mikroanjiyopatik hemolitik anemi, trombositopeni, pulmoner hemoraji, glukokortikoid kullanımı öyküsü ve kötü prognoz ile karakterize bir sendromdur. Burada; Bohan-Peter kriterlerine göre DM tanısı konarak glukokortikoid uygulamasına başlanan, tedavinin beşinci haftasında akut böbrek yetmezliği (ABY) gelişen, kapalı böbrek biyopsisi ile SCL böbreği tanısı konarak yüksek doz kaptopril tedavisine alınan, beş kez hemodiyaliz uygulanan ve ABY'nin 14'üncü gününde kaybedilen 22 yaşında bir olgu sunulmuştur.
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  • 2
88-91
  • Turkish

REFERENCES

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