Accumulation of periostin in acute exacerbation of familial idiopathic pulmonary fibrosis

Periostin, an extracellular matrix protein, has been identified as a novel biomarker of idiopathic interstitial pneumonia. Periostin is a mediator of chronic allergic diseases, and is involved in tissue remodeling observed in allergic inflammation in bronchial asthma, scleroderma, and atopic dermatitis (1). Periostin is highly expressed in idiopathic pulmonary fibrosis (IPF), and increased serum periostin correlates with the prognosis of IPF (2). Acute exacerbation (AE) of IPF (AE-IPF) is a life-threating event, and the prognosis of IPF is considerably dependent on the occurrence of AE (3). However, the association between periostin and AE-IPF has not been investigated. Herein, we have reported our findings in an autopsied patient with an AE of familial IPF (AE-FIPF) and revealed significant expression of periostin in the affected areas of the lungs. This is the first case report describing expression of periostin in AE-IPF