Abstract

BackgroundInterstitial lung diseases (ILDs) are disorders of the lung parenchyma. The pathogenesis, clinical manifestations, and prognosis of ILDs vary depending on the underlying disease. The onset of most ILDs is insidious, but they may also present subacutely or require hospitalization for management. ILDs that may present subacutely include acute interstitial pneumonia, connective tissue disease-associated ILDs, cryptogenic organizing pneumonia, acute eosinophilic pneumonia, drug-induced ILDs, and acute exacerbation of idiopathic pulmonary fibrosis. Prognosis and response to therapy depend on the type of underlying ILD being managed.DiscussionThis opinion piece discusses approaches to differentiating ILDs in the hospitalized patient, emphasizing the role of bronchoscopy and surgical lung biopsy. We then consider pharmacologic treatments and the use of mechanical ventilation in hospitalized patients with ILD. Finally, lung transplantation and palliative care as treatment modalities are considered.SummaryThe diagnosis of ILD in hospitalized patients requires input from multiple disciplines. The prognosis of ILDs presenting acutely vary depending on the underlying ILD. Patients with advanced ILD or acute exacerbation of idiopathic pulmonary fibrosis have poor outcomes. The mainstay treatment in these patients is supportive care, and mechanical ventilation should only be used in these patients as a bridge to lung transplantation.

Highlights

  • Interstitial lung diseases (ILDs) are disorders of the lung parenchyma

  • The prognosis of ILDs presenting acutely vary depending on the underlying ILD

  • Depending on the CTD, most radiographic and histopathologic subtypes of ILD, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), diffuse alveolar damage (DAD), organizing pneumonia (OP), bronchiolitis, cystic lung disease, and diffuse alveolar hemorrhage have been reported in patients with CTD

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Summary

Introduction

Interstitial lung diseases (ILDs) are disorders of the lung parenchyma. The pathogenesis, clinical manifestations, and prognosis of ILDs vary depending on the underlying disease. Examples of ILDs that usually require hospitalization include acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), acute eosinophilic pneumonia (AEP), rapidly progressive or acute exacerbation Depending on the CTD, most radiographic and histopathologic subtypes of ILD, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), DAD, organizing pneumonia (OP), bronchiolitis, cystic lung disease, and diffuse alveolar hemorrhage have been reported in patients with CTD.

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