XXXX : current and future treatment options.

@entity82 @entity2167 @entity500 @entity3794 @entity786 @entity2361 @entity3131 @entity898 @entity2416 @entity288 @entity205 @entity1576 @entity3812 @entity401 @entity647
@entity2416 ( @entity2416 ) is a chronic condition of unknown etiology with an unfavorable outcome from progressively deteriorating respiratory function, leading ultimately to death from @entity1576 . It is characterized by sequential @entity647 resulting in progressive fixed tissue @entity82 , @entity2167 and loss of function. An excess of profibrotic cytokines and/or a deficiency in antifibrotic cytokines have been implicated in the pathological process as has excessive oxidation. @entity2416 is distinguished from other forms of @entity3794 by the presence of the specific histological pattern of usual @entity401 . Oral @entity288 are the usual treatment, but objective response rates are poor and good quality studies do not exist. Other therapies either alone or in combination with @entity288 are widely used, including @entity500 , @entity3812 , @entity786 and @entity2361 . There is a paucity of good quality information regarding the effectiveness of most noncorticosteroid immunosuppressive agents. Older studies of lesser methodological quality have shown benefits from these drugs, generally when added to @entity288 . Many were retrospective reviews or uncontrolled, nonrandomized, open-label, prospective studies and often included other histological patterns of disease which are now thought to respond better to immunosuppressive agents. The results of intervention with @entity3812 and @entity500 have been disappointing when assessed by good quality trials using modern diagnostic criteria. Modern high quality studies are lacking for several agents, notably @entity786 and @entity2361 . The older agents may yet prove to be effective but further good quality trials will be necessary to assess these agents adequately. Other new anti-inflammatory, antioxidant, antifibrotic or anticytokine compounds are largely untried or unreported. One trial using interferon-gamma-1b showed a significant improvement in pulmonary function but there are concerns regarding the generalizability of this study. @entity3131 , @entity205 and @entity898 may also prove to be of benefit but current studies are of insufficient quality to allow for any conclusions to be drawn. Currently there is no good evidence to support the routine use of oral @entity288 , @entity500 , @entity786 , @entity2361 , @entity3812 , @entity205 or any other immunosuppressive, antifibrotic or immunomodulatory agent in the management of @entity2416 . Interferon, @entity3131 and other new agents may be of benefit but further studies are required. Any recommendations for treatment must therefore be made on an individual and empiric basis. As some other forms of @entity3794 may respond better to immunosuppressive agents, it remains important to make an accurate diagnosis, by open lung biopsy if necessary.
