non-specific interstitial pneumonia

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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 ?
13 cases (18.1%) were diagnosed as NSIP, 4 cases (5.5%) as UIP, 2 cases (2.8%) as organizing pneumonia.
In total, 32 patients with NSIP were selected according to the pathology with compatible clinical and radiological findings.
These findings are consistent with NSIP. Axial HRCT study (a) and sagittal reconstruction (b).
We also found that the prevalence of clubbing in patients with radiological NSIP pattern, RA-ILD, and SSc-ILD was 20.8%, 6.7%, and 25%, respectively.
The remaining 47 patients were retrospectively diagnosed with idiopathic interstitial pneumonias based on multiple disciplinary discussions, as per international consensus criteria [24], and consisted of 23 with IPF/UIP, 17 with NSIP, and 7 with COP.
NSIP: The presence of ground glass opacities, irregular linear or reticular infiltrates and associated with traction bronchiectasis with most commonly bilateral and symmetrical distribution.
Also, in our series, diagnostic rates are low in fibrotic ILD, such as UIP and NSIP. In non-fibrotic interstitial diseases, diagnostic rate was about 50% for Desquamative Interstitial Pneumonia (DIP) and bronchiolitis.
As performing these two techniques in a patient at the same time point could present some ethical limitations, we rather evaluated the yield of a sequential approach in which a SLB is performed after TBLCs when this latter technique is inconclusive or provides an unspecific diagnosis (including a histological pattern of nonspecific interstitial pneumonia NSIP).
Among all the ILD patterns, usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) are the most frequent, accounting for 40-60% and 11-30% of cases, respectively [28].
We present a 74-year-old male whom we evaluated for a suspected nonspecific interstitial pneumonia (NSIP) under ibrutinib (560 mg QD).
Systemic sclerosis-ILD is typically a nonspecific interstitial pneumonia (NSIP) pattern with groundglass opacities, interstitial pulmonary fibrosis, and honeycomb cystic changes in the lower lung zones [5, 6, 23].
Postac przewlekla AZPP histopatologicznie charakteryzuje sie obrazem srodmiazszowego wloknienia pluc mogacym przypominac typ wlokniejacy NSIP, UIP lub kryptogenne organizujace sie zapalenie pluc (cryptogenic organising pneumonia -COP) [33].