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 ?
The histological patterns of CADM-ILD vary and include desquamative interstitial pneumonitis, usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), and diffuse alveolar damage (DAD) (5).
12) Another study from Lucknow (India) reported IPF, Cryptogenic Organising Pneumonia (COP), Non-Specific Interstitial Pneumonia (NSIP) and Sarcoidosis in 48.
5%), hypersensitivity pneumonitis (15%), non-specific interstitial pneumonias (10%), Connective Tissue Disorder (C.
5%), Hypersensitivity pneumonitis (15%), Non-specific interstitial pneumonias (10%) and Connective tissue disorder associated with ILDs in 7.
High serum concentrations of surfactant protein A in usual interstitial pneumonia compared with non-specific interstitial pneumonia," Thorax, vol.
These changes were also compatible with the Scleroderma Lung Study and non-specific interstitial pneumonia pattern reported in previous studies by Ooi et al.
Almost all of the patients had lung fibrosis, with almost half having non-specific interstitial pneumonia pattern in HRCT.
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.
In several statistical analyses, the classifiers consistently differentiated IPF from multiple ILDs, including non-specific interstitial pneumonia (NSIP) and hypersensitivity pneumonitis (HP).
2 Histopatologic diagnoses 10 Non-specific interstitial pneumonia (NSIP) 3 30 Lymphocytic interstitial pneumonia (LIP) 2 20 Idiopathic pulmonary fibrosis (IPF) 2 20 Usual intestitial pneumonia (UIP) 1 10 Desquamative interstitial pneumonia (DIP) 1 10 Chronic infantile pneumonia (CPI) 1 10 Therapies administered 10 Systemic steroid 5 50 Systemic steroid + hydroxychloroquine 2 20 Systemic steroid + cyclophosphamide 2 20 Systemic steroid + azathiopyrine 1 10 Table 4.
Patients with the bland-looking fibrosis that is characteristic of usual interstitial pneumonia have a markedly worse prognosis than patients who have non-specific interstitial pneumonia and other inflammatory processes.
Interstitial lung disease, pulmonary vascular disease, aspiration pneumonia and pleurisy are common among which Interstitial Lung Disease is most common with an usual Interstitial Pneumonia (UIP) or Non-Specific Interstitial Pneumonia Pattern (NSIP).

Full browser ?