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  • Presents with nonspecific symptoms such as cough and dyspnea.
  • Associated most often with rheumatoid arthritis, scleroderma, primary Sjögren syndrome, dermatomyositis, polymyositis, and mixed connective tissue disease; rarely with systemic lupus erythematosus.
  • High-resolution computed tomography (HRCT) with thin-section images is the test of choice.
  • Nonspecific interstitial pneumonia is the most common histopathologic pattern.
  • Treatment with prednisone or immunomodulatory therapy (cyclophosphamide, mycophenolate mofetil, azathioprine) or both can be effective.

Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic rheumatic diseases, including rheumatoid arthritis, scleroderma, the inflammatory myopathies, primary Sjögren syndrome, and mixed connective tissue disease. Collectively, these conditions are called connective tissue disease–associated ILD. Symptoms are nonspecific and include cough and dyspnea. ILD can be a significant cause of morbidity and mortality in this patient population, and early diagnosis is essential to help prevent progression of symptoms and decline in pulmonary function.

ILDs are generally categorized based on the underlying histopathologic pattern seen on surgical lung biopsy (Table 64–1). Patients with rheumatic diseases can manifest many of the histopathologic patterns seen in idiopathic cases of ILD, including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), organizing pneumonia, and lymphocytic interstitial pneumonia (LIP) (Table 64–2).

Table 64–1. Key Histopathologic and HRCT Features of ILD Patterns.
Table 64–2. Histopathologic Patterns of ILD Found in the Rheumatic Diseases.

Although the diagnosis of connective tissue disease-associated ILD typically occurs after that of ...

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