The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described.The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography.Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean¡SD): total lung capacity 88%¡17, forced vital capacity (FVC) 88%¡18, forced expiratory volume in one second (FEV1) 80%¡21 (% predicted), FEV1/FVC 69%¡13, carbon monoxide diffusion capacity of the lung 37%¡16 (% predicted), carbon monoxide transfer coefficient 46%¡19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1¡2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis.The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.

Phenotypically, ANCA-positive and ANCA-negative Churg-Strauss syndrome might differ. The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome.

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Results. The mean ؎ SD followup was 56.2 ؎ 31.7 months. Among the 72 patients studied, 93% achieved remission with CS therapy alone, and 35% relapsed, mainly during the first year of treatment. Among the 19 patients randomized to additional immunosuppression because of treatment failure or relapse, 5 of 10 receiving AZA and 7 of 9 receiving pulse CYC achieved remission, but the difference was not statistically significant. Survival rates in all patients at 1 and 5 years were 100% and 97%, respectively. At the end of followup, 79% of the patients whose disease was in remission required lowdose CS therapy, mainly to control respiratory disease. CS-related adverse events were observed in 31% of the 72 patients.Conclusion. In CSS patients with an FFS of 0, survival was excellent, confirming the predictive value of the FFS in this disease. First-line therapy with CS achieved remission in most patients, but relapses were common, and one-third of them required additional immunosuppressive therapy. AZA or pulse CYC was fairly effective in treating CS-resistant disease or major relapses. Over the long term, most patients continued to take oral CS, which might explain the high rate of CS-related adverse events.Churg-Strauss syndrome (CSS), which is also called allergic granulomatosis and angiitis, is characterized by necrotizing eosinophilic vasculitis affecting the small vessels and extravascular granulomas. The major clinical manifestations are asthma, hypereosinophilia, and extrapulmonary manifestations of systemic vasculitis. Antineutrophil cytoplasmic antibodies (ANCAs), ClinicalTrials.gov identifier: NCT00399399.

In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients were past smokers, 3 current smokers and 6 non-smokers. Lung function tests at diagnosis were as follows (% predicted): total lung capacity 73%+/-18, vital capacity 82%+/-23, forced expiratory volume in 1 s (FEV(1)) 88%+/-24, carbon monoxide diffusion capacity of the lung 49%+/-2 (% predicted). Bronchoalveolar lavage results showed an increased cellularity with increased neutrophils counts. High resolution computed tomography of the chest showed prominent fibrosis with some degree of ground-glass attenuation in all patients. These characteristics were similar to the control group. Microscopic polyangiitis (MPA) was a major complicating event in ANCA-positive patients, occurring in 7 patients (anti-myeloperoxidase specificity in 5 patients). Pulmonary fibrosis predated occurrence of MPA in 6 patients and was diagnosed concomitantly with MPA in 1 patient. During the follow-up, 10/17 patients died. The death was directly related to vasculitis in 3 patients. We conclude that patients with pulmonary fibrosis should be evaluated for the presence of ANCA. Patients with positive ANCA testing, particularly if anti-myeloperoxidase, should be carefully monitored to detect the occurrence of microscopic polyangiitis.

This study demonstrated that COPD patients produce greater quantities of MMP-12 than controls, which may be a critical step in the pathogenesis of COPD and emphysema.

Objective. Findings from the WEGENT trial and other short-term studies have suggested that azathioprine (AZA) or methotrexate (MTX) could effectively maintain remission of granulomatosis with polyangiitis (Wegener's) (GPA) or microscopic polyangiitis (MPA).This study was undertaken to examine whether differences in rates of relapse or adverse events would appear after discontinuation of these 2 maintenance regimens, when assessed over a longer followup period.Methods. Long-term outcomes in patients enrolled in the WEGENT trial were analyzed according to their

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×10 L at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3-6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively.In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity.