Imaging of macrophage-related lung diseases

Abstract

Macrophage-related pulmonary diseases are a heterogeneous group of disorders characterized by macrophage accumulation, activation or dysfunction. These conditions include smoking-related interstitial lung diseases, metabolic disorders such as Niemann-Pick or Gaucher disease, and rare primary lung tumors. High-resolution computed tomography abnormalities include pulmonary ground-glass opacification secondary to infiltration by macrophages, centrilobular nodules or interlobular septal thickening reflecting peribronchiolar or septal macrophage accumulation, respectively, emphysema caused by macrophage dysfunction, and honeycombing following macrophage-related lung matrix remodeling.

Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis?

“…However, Marten et al [26] reported that the coexistence of CLE with NSIP patients provided evidence for smoking as a pathogenetic factor in a subset of NSIP patients. Longitudinal studies both in the development of CLE and NSIP have demonstrated that CLE replaces macrophage-related respiratory bronchiolitis [27,28] and that macrophage accumulation of DIP may recede leaving residual fibrotic NSIP [26,29] . In a more recent study, Antoniou et al [30] reported coarser fibrosis and notable honeycombing in smokers with IPF and rheumatoid lung relative to non-smoking counterparts.…”

An integrated approach in the diagnosis of smoking-related interstitial lung diseases

“…Smoking cessation leads to radiological improvement and even complete recovery in early cellular and nodular cases of PLCH [89,91,92]. The disease may improve with smoking cessation alone; the 5-and 10-yr survival rate is 74.6% and 63.9%, respectively [93].…”

“…The mainstay of treatment in patients with DIP consists of corticosteroid therapy, which may cause stabilisation or improvement of the disease. Complete recovery of the disease has been described [59,89]. In ,25% of patients, however, progression of disease may be observed despite steroid therapy [11,59,89] and lung transplantation may be an option.…”

“…Complete recovery of the disease has been described [59,89]. In ,25% of patients, however, progression of disease may be observed despite steroid therapy [11,59,89] and lung transplantation may be an option. Recurrence has also been reported in patients who have received a lung transplant [90].…”

“…Spontaneous improvement has been observed in some patients with DIP, possibly related to smoke cessation. There is clinical deterioration in approximately two-thirds of patients with DIP who are untreated [11,89]. The mainstay of treatment in patients with DIP consists of corticosteroid therapy, which may cause stabilisation or improvement of the disease.…”