Non-specific interstitial pneumonia

Symptoms

Symptoms include cough, difficulty breathing, and fatigue.

Causes

It has been suggested that idiopathic nonspecific interstitial pneumonia has an autoimmune mechanism, and is a possible complication of undifferentiated connective tissue disease; however, not enough research has been done at this time to find a cause. Patients with NSIP will often have other unrelated lung diseases like COPD or emphysema, along with other auto-immune disorders.

Diagnosis

Diagnosis is made via a multi-disciplinary team review of patient history, imaging, lung function testing, and in some cases a surgical lung biopsy. While a lung biopsy is the gold standard, some clinicians opt against this due to the risks of the procedure.

Lung biopsies performed on patients with NSIP reveal two different disease patterns – cellular and fibrosing – which are associated with different prognoses. The cellular pattern displays chronic inflammation with minimal fibrosis. The fibrosing pattern displays interstitial fibrosis with various inflammation levels. Both patterns are uniform and lack the prominent fibroblastic foci that are found in other types of idiopathic interstitial pneumonia.

Treatment

The mainstay of treatment is corticosteroids such as prednisolone, with immunosuppressant medications such as azathioprine, and mycophenolate added in some cases. Some patients may require oxygen as their disease progresses.

Prognosis

The fibrosing pattern of NSIP has a five-year survival rate of 86% to 92%, while the cellular pattern of NSIP has a 100% five-year survival rate. Patients with NSIP (whether cellular or fibrosing), have a better prognosis than those with usual interstitial pneumonia (UIP).

References

References

1. (October 1998). "Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients.". American Journal of Respiratory and Critical Care Medicine.
2. (October 2007). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine.
3. (15 June 2008). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine.
4. (January 2000). "Idiopathic Nonspecific Interstitial Pneumonia: Prognostic Significance of Cellular and Fibrosing Patterns". The American Journal of Surgical Pathology.