Non-specific interstitial pneumonia (NSIP) is a form of
idiopathic interstitial pneumonia.
Symptoms
Symptoms include
cough
A cough is a sudden expulsion of air through the large breathing passages that can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three ph ...
,
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
Undifferentiated connective tissue disease (UCTD) is a disease in which the body mistakenly attacks its own tissues. It is diagnosed when there is evidence of an existing autoimmune condition which does not meet the criteria for any specific auto ...
; 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 long-term respiratory symptoms and airflow limitation. The main symptoms include shortness of breath and a cough, which may or may not produce ...
or
emphysema, along with other
auto-immune disorders.
Diagnosis
A full clinical diagnosis can only be made from a
lung biopsy
A lung biopsy is an interventional procedure performed to diagnose lung pathology by obtaining a small piece of lung which is examined under a microscope. Beyond microscopic examination for cellular morphology and architecture, special stains and ...
of the tissue, fully best performed by a VATS, done by a cardio-thoracic surgeon. Some pulmonologists may first attempt a
bronchoscopy
Bronchoscopy is an endoscopic technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument (bronchoscope) is inserted into the airways, usually through the nose or mouth, or occasionally through a trac ...
; however, this frequently fails to give a full or correct diagnosis.
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
Commonly used drugs include
prednisone
Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and ad ...
,
imuran
Azathioprine (AZA), sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus erythematosus, ...
, and
cellcept. For active cases of pneumonia for these patients,
Levaquin is a commonly used antibiotic. Almost all patients with NSIP will be on oxygen.
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
External links
Pneumonia
{{respiratory-disease-stub