Non-specific interstitial pneumonia (NSIP) is a form of

idiopathic interstitial pneumonia Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung diseases. These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptoge ...

Symptoms

Symptoms include

cough A cough is a sudden expulsion of air through the large breathing passages which can help clear them of fluids, irritants, foreign particles and Microorganism, microbes. As a protective reflex, coughing can be repetitive with the cough reflex fol ...

, difficulty breathing, and

fatigue Fatigue is a state of tiredness (which is not sleepiness), exhaustion or loss of energy. It is a signs and symptoms, symptom of any of various diseases; it is not a disease in itself. Fatigue (in the medical sense) is sometimes associated wit ...

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 Chronic obstructive pulmonary disease (COPD) is a type of progressive lung disease characterized by chronic respiratory symptoms and airflow limitation. GOLD defines COPD as a heterogeneous lung condition characterized by chronic respiratory s ...

emphysema Emphysema is any air-filled enlargement in the body's tissues. Most commonly emphysema refers to the permanent enlargement of air spaces (alveoli) in the lungs, and is also known as pulmonary emphysema. Emphysema is a lower respiratory tract di ...

, 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 Prednisolone is a corticosteroid, a steroid hormone used to treat certain types of allergies, inflammation, inflammatory conditions, autoimmune disorders, and cancers, Electrolyte imbalance, electrolyte imbalances and skin conditions. Some of ...

, with immunosuppressant medications such as

azathioprine Azathioprine, sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used for the treatment of rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus er ...

, and

mycophenolate Mycophenolic acid is an immunosuppressant medication used to prevent rejection following organ transplantation and to treat autoimmune conditions such as Crohn's disease and lupus. Specifically it is used following kidney, heart, and liver t ...

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 Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. The scarring ( pulmonary fibrosis) involves the pulmonary interstitium (the supporting framework of the lung). UIP is thus classifi ...

(UIP).

References

External links

{{Respiratory pathology Pneumonia