Tuberc Respir Dis.  2019 Oct;82(4):277-284. 10.4046/trd.2018.0092.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 3. Idiopathic Nonspecific Interstitial Pneumonia

Affiliations
  • 1Division of Pulmonology and Allergy, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University School of Medicine, Seoul, Korea.
  • 2Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary's Hospital, The Catholic University School of Medicine, Bucheon, Korea. kyh30med@catholic.ac.kr
  • 3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ulsan University Hospital, Ulsan University College of Medicine, Ulsan, Korea.
  • 4Department of Pulmonary and Critical Care Medicine, Chungnam National University Hospital, Daejeon, Korea.

Abstract

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.

Keyword

Lung Diseases, Interstitial; Idiopathic Interstitial Pneumonias; Guideline

MeSH Terms

Azathioprine
Bronchiectasis
Cough
Cyclophosphamide
Cyclosporine
Diagnosis*
Dyspnea
Female
Fibrosis
Humans
Idiopathic Interstitial Pneumonias
Idiopathic Pulmonary Fibrosis
Immunosuppressive Agents
Inflammation
Lung
Lung Diseases, Interstitial*
Pathology
Prognosis
Survival Rate
Traction
Azathioprine
Cyclophosphamide
Cyclosporine
Immunosuppressive Agents
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