Monitoring

Patient monitoring focuses on symptoms and pulmonary function tests, with or without high-resolution computed tomography every 3-6 months.[2]​ Assessments should preferably include evaluation of dyspnea, forced vital capacity, lung volumes, lung diffusion capacity test, arterial blood gasses, and cardiopulmonary exercise testing (with concomitant gas exchange analysis). However, it is not always possible to perform the full spectrum of tests, and practice may vary locally. 

Six-minute walk tests, which are simple and inexpensive to perform, can provide information on disease progression that correlates well with cardiopulmonary exercise testing.[158][159]​ Serial spirometry is also simple to perform and can provide useful prognostic information.[120][122][160][161]​ It is therefore reasonable to follow patients with serial spirometry and 6-minute walk tests every 6 months. More frequent measures may be appropriate in patients who experience a change in clinical status or new symptoms.

Monitoring pharmacotherapeutic response

Pharmacologic therapy requires monitoring for response and emergent adverse events. Progressive decline despite therapy or significant drug toxicity should result in the therapy being tapered and withdrawn. Subjective responses to treatment should not be used as the sole rationale for continuation of therapy (e.g., mild euphoria with corticosteroid therapy can be misleading). If moderate- to high-dose corticosteroid therapy is continued, prophylaxis against  Pneumocystis jirovecii infection (e.g., trimethoprim/sulfamethoxazole) and osteopenia (e.g., evaluate using bone densitometry and give calcium, vitamin D, and bisphosphonates, as appropriate) should be considered.[162]

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