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.

Juvenile idiopathic arthritis presenting as diffuse alveolar hemorrhage at onset: A case series

“…In addition, some studies have shown that if ImPH is treated effectively in the early stages, the severity of JIA can be reduced, and even the occurrence of JIA can be avoided. This fully affirms the importance of early diagnosis and timely treatment of ImPH 20‐22 …”

“…This fully affirms the importance of early diagnosis and timely treatment of ImPH. [20][21][22] To our knowledge, this is the first report of case series about JIA presenting as DAH at onset. Considering the limitations of the small numbers of cases in our study, there may have been some selection bias.…”