Accumulation of periostin in acute exacerbation of familial idiopathic pulmonary fibrosis

Abstract

Periostin, an extracellular matrix molecule, is associated with idiopathic pulmonary fibrosis (IPF). It is known that the frequency of familial IPF (FIPF) ranges from 0.5% to 2.2% among IPF cases. However, the relationship between periostin and FIPF has not been previously described. We report the first case of periostin accumulation in the lungs of a patient with an acute exacerbation of FIPF. A 72-year-old woman, diagnosed with FIPF, had been followed up for 5 years. The patient experienced increased dyspnea within a 1-month period and was referred to our hospital. The patient was hypoxic, and chest computed tomography showed rapidly expanding bilateral reticular shadows. Despite pulse-steroid and intravenous-cyclophosphamide therapy, the patient died 25 days after admission. On admission, serum periostin levels were not significantly elevated, while serum fibrotic marker levels were elevated. Immunohistochemical analysis of the lungs on autopsy showed marked accumulation of periostin in the active fibrotic lesions, whereas intact and burned-out areas did not show significant expression of periostin. This case might provide insight into the role of periostin in acute exacerbation of IPF.

Keywords: Familial idiopathic pulmonary fibrosis (FIPF); acute exacerbation (AE); idiopathic interstitial pneumonia; periostin.

Figure 1

Chest radiographs and computed tomography images showed symmetrical reticular and ground glass opacities. The radiological features progressed slowly over 5 years from the age of 72 years (A: 72 years; B: 77 years), but then rapidly deteriorated over a month (B: 54 days and C: 25 days prior to death, respectively).

Figure 2

Immunohistochemistry of periostin in the lung. Periostin accumulation is seen in the present case (left panel), while no staining of periostin is observed in the control lung obtained from a patient with early lung cancer (middle panel). Positive staining of periostin is seen in the asthmatic lung obtained from a patient with early lung cancer complicated with bronchial asthma (right panel). AE, acute exacerbation; IPF, idiopathic pulmonary fibrosis.

Figure 3

Periostin accumulation in sub-acute lesion after acute exacerbation of idiopathic pulmonary fibrosis. Periostin accumulation was observed in acute, sub-acute, and chronic inflammatory/fibrotic lesion of autopsy lung after acute exacerbation of idiopathic pulmonary fibrosis. Arrow indicates bronchiolar metaplasia and arrowheads indicates smooth muscle proliferation.