Successful Treatment of Infection-Triggered Acute Exacerbation of Idiopathic Pulmonary Fibrosis with Corticosteroids Combined with Macrolides
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial pneumonia (IP) with poor prognosis. Acute exacerbation (AE) of IPF (AE-IPF) has a substantial and sometimes fatal impact on prognosis. An effective pharmaceutical treatment for AE-IPF is lacking. Macrolides (MACs) have an anti-bacterial activity and anti-inflammatory effects, and IPF treatment with these agents has been recently reported. This report describes a case of infection-triggered AE-IPF treated with corticosteroids (CSs) combined with MACs. A 61-year-old male patient suffering from IPF previously treated with methyl-prednisolone (mPSL) (8 mg/day) was admitted because of fever, dry cough, and dyspnea. Reticular opacities (RO) on chest roentgenogram and ground-glass opacities (GGO) on high-resolution computed tomography (HRCT) exacerbated. The patient was diagnosed with influenza A and influenza A-triggered AE-IPF and was treated with peramivir and mPSL (1 g/day) for 3 days. RO on chest roentgenogram further exacerbated, prompting the addition of erythromycin (EM), in consideration of its anti-inflammatory effects. Thereafter, the patient was successfully treated with mPSL or PSL combined with EM. During the clinical course, he experienced cytomegalovirus (CMV)-induced IP and/or CMV-triggered AE-IPF, being successfully treated with ganciclovir and mPSL or PSL combined with EM. Thereafter, the patient was treated with CSs combined with EM or clarithromycin. Approximately 3 months after initiating EM, RO on chest roentgenogram and GGO on HRCT considerably improved. This case shows that treatment with CSs combined with MACs may be effective in some cases of infection-triggered AE-IPF.
References
Kawamura K, Ichikado K, Yasuda Y, Anan K, Suga M. Azithromycin for idiopathic acute exacerbation of idiopathic pulmonary fibrosis: a retrospective single-center study. BMC Pulm Med 2017;17:94.
Jouneau S, Kerjouan M, Caulet-Maugendre S, Guillot S, Meunier C, Desrues B, Delaval P. Clarithromycin stops lung function decline in airway-centered interstitial fibrosis. Respiration 2013;85:156-159.
Ohe M, Hashino S. A case of idiopathic pulmonary fibrosis treated with clarithromycin. Eastern J Med 2016; 19:240-243.
Kuse N, Abe S, Hayashi H, Kamio K, Saito Y, Usuki J, Azuma A, Kudoh S, Gemma A. Long-term efficacy of macrolide treatment in idiopathic pulmonary fibrosis: a retrospective analysis. Sarcoidosis Vasc Diffuse Lung Dis 2016;33:242-246.
Kohno N. Serum marker KL-6/MUC1 for the diagnosis and management of interstitial pneumonitis. J Med Invest 1999;46:151-158.
Kishaba T. Acute exacerbation of idiopathic pulmonary fibrosis. Medicina (Kaunas). 2019 Mar 16;55(3). pii: E70. doi: 10.3390/medicina55030070.
Lee SH, Yeo Y, Kim TH, Lee HL, Lee JH, Park YB, et al. Korean guideline for diagnosis and management of interstitial lung diseases: Part 2. Idiopathic pulmonary fibrosis. Tuberc Respir Dis 2019;82:102-117.
Tomioka H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep 2017;5:e00215.
Cai M, Bonella F, Dai H, Sarria R, Guzman J, Costabel U. Macrolides inhibit cytokine production by alveolar macrophages in bronchiolitis obliterans organizing pneumonia. Immunobiology 2013;218:930-937.
Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 2005;128:3310-3315.