Treatment and prognosis of nonspecific interstitial pneumonia
- Kevin R Flaherty, MD, MS
Kevin R Flaherty, MD, MS
- Section Editor — Interstitial Lung Disease
- Associate Professor of Medicine
- University of Michigan Health System
Nonspecific interstitial pneumonia (NSIP) can be idiopathic or can be seen in association with connective tissue disease, HIV infection, a variety of drugs, and hypersensitivity pneumonitis. When idiopathic, NSIP is considered one of the idiopathic interstitial pneumonias (IIPs), which also include usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), acute interstitial pneumonia (AIP), and cryptogenic organizing pneumonia (COP) (table 1) . Idiopathic NSIP can present alone or in combination with one of the other IIPs.
The treatment and prognosis of NSIP will be reviewed here. The clinical manifestations, evaluation, and diagnosis of NSIP and the diagnosis and management of the other IIPs are discussed separately. (See "Causes, clinical manifestations, evaluation, and diagnosis of nonspecific interstitial pneumonia" and "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis" and "Treatment of idiopathic pulmonary fibrosis" and "Respiratory bronchiolitis-associated interstitial lung disease" and "Cryptogenic organizing pneumonia" and "Acute interstitial pneumonia (Hamman-Rich syndrome)".)
OVERVIEW OF TREATMENT
The treatment of NSIP depends on the cause, disease severity, and rate of progression. While the discussion in the sections that follow will emphasize the treatment for idiopathic NSIP, much of the support for treatment selections is based on experience in connective tissue associated NSIP.
●Drug or inhalational exposure – For patients with NSIP associated with a drug or inhalational exposure, the first step is to remove the inciting exposure. This alone may be adequate treatment. (See "Causes, clinical manifestations, evaluation, and diagnosis of nonspecific interstitial pneumonia", section on 'Definition and causes'.)
●Connective tissue disease – For patients with NSIP complicating a known connective tissue disease (eg, polymyositis/dermatomyositis, rheumatoid arthritis, systemic sclerosis), specific therapies for those diseases may guide treatment of the NSIP. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis" and "Overview of lung disease associated with rheumatoid arthritis" and "Interstitial lung disease associated with Sjögren's syndrome: Clinical manifestations, evaluation, and diagnosis" and "Overview of pulmonary complications of systemic sclerosis (scleroderma)" and "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis".)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- OVERVIEW OF TREATMENT
- Supportive care
- OBSERVATION FOR MILD STABLE DISEASE
- INITIAL THERAPY FOR MODERATE TO SEVERE DISEASE
- Pulse methylprednisolone
- Additional immunosuppressive drugs
- - Azathioprine
- - Mycophenolate mofetil
- REFRACTORY DISEASE
- Calcineurin inhibitors
- ONGOING MONITORING
- Acute exacerbations
- LUNG TRANSPLANTATION
- FUTURE DIRECTIONS
- SUMMARY AND RECOMMENDATIONS