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Semin Respir Crit Care Med 2016; 37(03): 378-394
DOI: 10.1055/s-0036-1583176
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?

Sara Tomassetti
1   Department of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy
,
Jay H. Ryu
2   Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota
,
Sara Piciucchi
3   Department of Radiology, GB Morgagni Hospital, Forlì, Italy
,
Marco Chilosi
4   Department of Pathology, University of Verona, Verona, Italy
,
Venerino Poletti
1   Department of Diseases of the Thorax, GB Morgagni Hospital, Forlì, Italy
› Author Affiliations
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Publication History

Publication Date:
27 May 2016 (online)

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Abstract

We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. This hypothesis warrants further studies. Currently, two major radiologic–pathologic different profiles have been well established. First, the “inflammatory type” characterized by prominent lymphocytic inflammation both on biopsy and bronchoalveolar lavage (BAL), and high-resolution computed tomography (HRCT) with mixed NSIP/organizing pneumonia pattern that tends to have a better response to corticosteroid and immunosuppressive treatment. Second, the “highly fibrotic” subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into “full-blown idiopathic pulmonary fibrosis.” The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients.

Keywords

interstitial lung diseases - idiopathic nonspecific interstitial pneumonia - cyclophosphamide - rituximab
 

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