Extraintestinal manifestations of inflammatory bowel disease (IBD) is a common clinical problem affecting up to half of all IBD patients; pulmonary disease, however, ranks among less common extraintestinal manifestations of IBD. Pulmonary disease in patients with IBD is most frequently drug induced due to treatment with sulfasalazine or mesalamine leading to eosinophilic pneumonia and fibrosing alveolitis or due to treatment with methotrexate leading to pneumonitis. Recently, various opportunistic infections have been shown to be a further important cause of pulmonary abnormalities in those IBD patients who are treated with immunosuppressants such as anti TNF-α monoclonal antibodies, methotrexate, azathioprine or calcineurin antagonists. In not drug related pulmonary disease a wide spectrum of disease entities ranging from small and large airway dysfunction to obstructive and interstitial lung disorders exist. Patients with lung disorders and inflammatory bowel disease should be evaluated for drug-induced lung disease and opportunistic infections prior to considering pulmonary disease as an extraintestinal manifestation of inflammatory bowel disease.

In the past decade, immunosuppressive drugs have come to play an integral role in the treatment of patients with inflammatory bowel disease. Cyclosporine, microemulsion cyclosporine, tacrolimus, and mycophenolate mofetil can be considered for the treatment of patients with refractory inflammatory Crohn's disease, fistulizing Crohn's disease, and severe ulcerative colitis. This article reviews the use of cyclosporine, tacrolimus, and mycophenolate mofetil in patients with inflammatory bowel disease, with emphasis on pharmacology, results in controlled clinical trials, and safety, and issues related to dosing and toxicity monitoring.

Extraintestinal manifestations of both Crohn's disease and ulcerative colitis (UC) have been well described, although pulmonary findings are often overlooked. We summarize the experience of more than 400 cases of pulmonary manifestations of inflammatory bowel disease (IBD). These manifestations will be categorized by disease mechanism into drug-induced disease, anatomic disease, over-lap syndromes, autoimmune disease, physiologic consequences of IBD, pulmonary function test abnormalities, and nonspecific lung disease. We intend to provide the clinician with a practical working update on the spectrum of pulmonary dysfunction associated with IBD.

Idiopathic pulmonary fibrosis (IPF) is an inflammatory disorder of the lungs of unknown etiology, with no effective treatment. Besides the recent finding of utility of mycophenolate mofetil (MMF) in a case of refractory interstitial lung disease associated with ulcerative colitis, I suggest that there are at least three other reasons to consider MMF for IPF. Previously, MMF has been found to be effective as salvage therapy in a number of diseases. MMF might work for IPF not only by white cell suppression, but also in vivo against proliferation of primary human pulmonary fibroblasts. There is one group of patients for whom, logically, MMF should be most strongly considered--those with a high likelihood of receiving a lung transplant. As MMF is often part of the post-transplant immunosuppressive regimen in these patients, logic would seem to dictate MMF should be considered for use before subjecting the patient to major surgery.