Immunomodulatory agents for idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

“…[27][28][29][30][31][32][33] However, pulmonary fibrosis has been demonstrated in animal models in the absence of inflammatory cells. 33,34 Furthermore, because inflammatory suppressive agents do not seem to be effective, it may be that IPF is not caused by inflammatory cells but that inflammatory cells are secondarily involved.…”

“…33,34 Furthermore, because inflammatory suppressive agents do not seem to be effective, it may be that IPF is not caused by inflammatory cells but that inflammatory cells are secondarily involved. [29][30][31][32][33] More recent research has demonstrated that alveolar epithelial cells, as a consequence of injury by an unknown cause, may be the source of a number of fibrogenic cy-tokines such as transforming growth factor-beta 1, [33][34][35][36][37][38] platelet-derived growth factor, 39 tumour necrosis factoralpha, 40,41 IL-1, 40 insulin-like growth factor1 42 and basic fibroblast growth factor. 43 Release of these cytokines may result in fibroblast proliferation and migration to various sites in the lung, followed by differentiation of the fibroblast phenotype.…”

“…4,[30][31][32] Although corticosteroids have been standard therapy for years, 30,32 no evidence of benefit in survival has been established. The use of corticosteroids is also associated with side effects and complications.…”

“…68 Despite the lack of evidence, a common therapy currently used for IPF is corticosteroids in conjunction with azathioprine. [30][31][32]69 The combination of prednisone (20 mg/d) and azathioprine (3 mg/kg daily but not to exceed 200 mg/d) was reported to tend to stabilize lung function, but no comment on mortality rates could be made since the duration of the study was only 1 year. 69 Before starting the combined therapy it may be wise to determine if the patient has latent tuberculosis by doing a tuberculin skin test.…”

Nintedanib: A Novel Therapeutic Approach for Idiopathic Pulmonary Fibrosis

“…Prevention of gastroesophageal reflux and recurrent microaspiration may slow disease progression. A number of other agents (eg, anticoagulation, 4 colchicine, 5 cyclophosphamide, 6 endothelin receptor antagonists, 7 interferon gamma-1b, 8 methotrexate, 9 cyclosporine, 10 penicillamine, 11 imatinib, 12 etanercept, 13 Nacetylcysteine, 14 prednisone/azathioprine/N-acetylcysteine combination 15,16 ) have been used in the past in either case series or clinical trials. The process of fibrosis is driven by innumerable growth factors and their downstream intracellular signaling pathways.…”