title: Long-term clinical course of idiopathic pulmonary haemosiderosis with rheumatoid arthritis.
abstract: Idiopathic pulmonary haemosiderosis (IPH) is a rare cause of diffuse alveolar haemorrhage during childhood, and its precise pathophysiology and long-term clinical course remain unclear. A 31-year-old man was diagnosed with IPH at four years of age and had recurrent episodes of haemoptysis. The patient's symptoms responded well to steroids. However, pulmonary fibrosis and the cystic region in the lung progressively worsened. At age 27, the patient developed polyarthritis with positive anti-cyclic citrullinated peptide antibodies. The patient also developed hand synovitis, which was diagnosed with ultrasonography. These results indicate complications from rheumatoid arthritis. The patient's dyspnoea gradually worsened, and at the age of 31, he developed pneumothorax and an acute exacerbation of IPH. The clinical course from ages 4 to 31 included progressive chronic respiratory failure because of pulmonary fibrosis, acute exacerbations, complications with rheumatoid arthritis, and deliberation regarding lung transplantation. The development of rheumatoid arthritis after the onset of IPH supports the theory of an autoimmune mechanism of IPH.
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Shunsuke
authors: S
authors: Sugimoto
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Jiro
authors: J
authors: Terada
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Akira
authors: A
authors: Naito
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Rintaro
authors: R
authors: Nishimura
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Kenji
authors: K
authors: Tsushima
authors: Department of Respirology, Graduate School of Medicine Chiba University Chiba Japan.
authors: Koichiro
authors: K
authors: Tatsumi
keywords: Anti‐cyclic citrullinated peptide antibodies; idiopathic pulmonary haemosiderosis; pneumothorax; rheumatoid arthritis