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Nonspecific Interstitial Pneumonia

By

Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Last full review/revision Jun 2021| Content last modified Jun 2021
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Nonspecific interstitial pneumonia is an idiopathic interstitial pneumonia that occurs mainly in women, nonsmokers, and patients < 50 years. Patients have cough and dyspnea, which may be present for months to years. Diagnosis is with high-resolution CT and lung biopsy. Treatment is with corticosteroids and sometimes other immunosuppressive drugs.

Nonspecific interstitial pneumonia (NSIP) is an idiopathic interstitial pneumonia Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic... read more . It is much less common than idiopathic pulmonary fibrosis Idiopathic Pulmonary Fibrosis Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia, causes progressive pulmonary fibrosis. Symptoms and signs develop over months to years and include... read more Idiopathic Pulmonary Fibrosis (IPF). Most patients are women, are between the ages of 40 and 50, and have no known cause or association. However, a similar pathologic process can occur in patients with a connective tissue disorder (in particular, systemic sclerosis Systemic Sclerosis Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus... read more Systemic Sclerosis or autoimmune myositis Autoimmune Myositis Autoimmune myositis is characterized by inflammatory and degenerative changes in the muscles (polymyositis) or in the skin and muscles (dermatomyositis). Manifestations include symmetric weakness... read more Autoimmune Myositis ), in some forms of drug-induced pulmonary disease Drug-Induced Pulmonary Disease Drug-induced pulmonary disease is not a single disorder, but rather a common clinical problem in which a patient without previous pulmonary disease develops respiratory symptoms, chest x-ray... read more , and in patients with hypersensitivity pneumonitis Hypersensitivity Pneumonitis Hypersensitivity pneumonitis is a syndrome of cough, dyspnea, and fatigue caused by sensitization and subsequent hypersensitivity to environmental (frequently occupational or domestic) antigens... read more Hypersensitivity Pneumonitis .

Clinical presentation of NSIP is similar to that of IPF. Cough and dyspnea are present for months to years.

Constitutional symptoms are unusual, although a low-grade fever and malaise are possible.

Diagnosis of Nonspecific Interstitial Pneumonia

  • High-resolution CT (HRCT)

  • Surgical lung biopsy

The diagnosis of nonspecific interstitial pneumonia should be considered in patients with unexplained subacute or chronic cough and dyspnea. Diagnosis requires HRCT and always requires confirmation by surgical lung biopsy. NSIP is a diagnosis of exclusion that requires careful clinical review for possible alternative disorders, in particular connective tissue disorders, hypersensitivity pneumonitis, and drug toxicity.

Chest x-ray primarily shows lower-zone reticular opacities. Bilateral patchy opacities are also possible.

HRCT findings include bilateral patchy ground-glass attenuation, irregular lines, and bronchial dilation (traction bronchiectasis), generally with a lower lung zone distribution. Subpleural sparing is possible. Honeycombing is rare.

Bronchoalveolar lavage fluid has an increased percentage of lymphocytes in more than half of patients, but this finding is nonspecific.

Surgical lung biopsy is required for a diagnosis of NSIP. Histologically, most patients have some degree of fibrosis. The main feature of NSIP is temporally homogenous inflammation and fibrosis, as opposed to the heterogeneity in usual interstitial pneumonia. Although the changes are temporally uniform, the process may be patchy, with intervening areas of unaffected lung.

Treatment of Nonspecific Interstitial Pneumonia

  • Corticosteroids with or without immunosuppressive drugs

Many patients with nonspecific interstitial pneumonia respond to treatment with corticosteroids, with or without immunosuppressive drugs (eg, azathioprine, mycophenolate, cyclophosphamide).

Prognosis seems to depend most on the degree of fibrosis found during surgical lung biopsy. In patients with primarily cellular disease, almost all patients survive at least 10 years. However, with increasing fibrosis, survival worsens and in some series median survival for fibrotic NSIP is 3 to 5 years.

Key Points

  • Nonspecific interstitial pneumonia is uncommon; most patients are women, are between the ages of 40 and 50, and have no known risk.

  • Exclude connective tissue disorders (particularly systemic sclerosis and autoimmune myositis), drug-induced lung injury, and hypersensitivity pneumonitis and do surgical lung biopsy.

  • Treat with corticosteroids, with or without immunosuppressive drugs (eg, azathioprine, mycophenolate, cyclophosphamide).

  • Prognosis is worse if biopsy shows more fibrosis.

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