Erdheim-Chester disease multisystemic manifestations and long term survival with corticosteroid therapy

TERTEMİZ K. C., SEVİNÇ C., Tuna B., Karatosun V. A., YILMAZ E., Celik A.

Respiratory Medicine CME, cilt.1, sa.2, ss.126-130, 2008 (Scopus) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 1 Sayı: 2
  • Basım Tarihi: 2008
  • Doi Numarası: 10.1016/j.rmedc.2008.02.004
  • Dergi Adı: Respiratory Medicine CME
  • Derginin Tarandığı İndeksler: Scopus
  • Sayfa Sayıları: ss.126-130
  • Anahtar Kelimeler: Diabetes insipidus, Histiocytosis, Interstitial lung disease, Pneumothorax
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytic disorder of unknown cause characterized by heterogeneous systemic manifestations. The histiocytic infiltration can affect bones, orbits, heart, kidneys, lungs, mediastinum, liver, spleen and central nervous system. Corticosteroids are the first-line treatment. The 3-year survival rate for patients with ECD is approximately 50%. We report the case of a 31 year old male ECD patient that have pulmonary fibrosis, bone involvement and diabetes insipidus. He was treated with systemic and inhaled corticosteroids and has remained stable for 53 months after this rare type of treatment. © 2008 Elsevier Ltd. All rights reserved.