non-specific interstitial pneumonia

non-specific interstitial pneumonia

An idiopathic interstitial pneumonia with diffuse inflammation, which is divided into the more common fibrotic NSIP with prominent fibrosis and cellular NSIP.

Clinical findings
Patients present with chronic or subacute cough and dyspnea, and are 7 to 10 years younger than those with idiopathic pulmonary fibrosis.
 
Imaging, high-resolution CT
Ground glass and fibrosis in fibrotic NSIP; ground glass changes in cellular NSIP. Given the clinical and radiographic overlaps between fibrotic NSIP and idiopathic interstitial pneumonia (IPF), surgical lung biopsy is often required to distinguish these two.

DiffDx
Collagen vascular disease, drug reaction, hypersensitivity.

Prognosis
Much better for NSIP than for IPF; most patients survive 7 to 10 years.

Management
Immunosuppression with oral corticosteroids and cytotoxic immunosuppressive agents are the primary therapy. Type and duration of therapy are guided by disease activity and degree of inflammation on biopsy and ground glass on HRCT. Pathologic NSIP is not a unique pattern and can often be seen in connective tissue disease or hypersensitivity pneumonitis; a thorough investigation for these should be undertaken to rule out these alternative diagnoses.
References in periodicals archive ?
Using whole-genome data and classifiers trained by histopathology "truth," researchers developed a classifier that could distinguish this pattern with high specificity (92 percent), suggesting its ability to identify and distinguish IPF from other ILDs, including non-specific interstitial pneumonia, emphysema and organizing pneumonia.
We hope that through this study, we will be able to provide the first clinical evidence of an effective treatment option to PH patients with different types of IIPs, for example non-specific interstitial pneumonia and unclassifiable forms of pulmonary fibrosis.

Full browser ?