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

SummaryErdheim–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.