Acute exacerbation of interstitial lung diseases secondary to systemic rheumatic diseases: a prospective study and review of the literature

Abstract

Acute exacerbation (AE) is a possible manifestation of interstitial lung diseases (ILD) associated to very high mortality. It's defined as clinically significant respiratory deterioration with evidence of new widespread alveolar abnormalities on computed tomography scan. AE is better described in idiopathic pulmonary fibrosis (IPF) but also reported in ILD secondary to connective tissue diseases (CTD) and vasculitis. The main features and the real clinical impact of this severe complication in these patients are not well defined. Aim of our study was to prospectively investigate the incidence, clinical features and outcome of AE in a population of patients with ILD related to CTD and vasculitis. We consecutively enrolled all patients, with ILD secondary to rheumatic systemic diseases, referring to our multidisciplinary outpatient clinic for rare lung diseases. All patients were followed for at least 12 months (range, 12-36 months). At baseline, all patients underwent to a core set of laboratory investigations and periodically followed; data about demographic, disease onset, clinical, serological and therapeutic features were also recorded. AE occurred in 9/78 patients, with an incidence of 5.77/100 patients/year, and 5/9 patients died because of AE. The baseline value of DLCO was significantly associated to the risk of AE at Cox regression. In patients with ILD related to rheumatic systemic diseases AE can occur with an incidence similar to IPF. Rheumatologists should carefully consider this life-threatening complication as a possible natural course of all patients with ILD secondary to systemic rheumatic disease.

Acute Exacerbation of Interstitial Lung Disease in Adult Patients With Idiopathic Inflammatory Myopathies: A Retrospective Case-Control Study

“…For patients with IPF, 46% of deaths are secondary to AE and median survival period after AE is 3-4 months (49). And a high mortality rate (55.6%) was as well-seen in CTD patients with AE-ILD (14). In this study, the shortterm mortality rate of AE-ILD group was 39.1%.…”

“…And infection has been found to participate in the pathogenesis and progression of idiopathic pulmonary fibrosis (IPF) (13). As previously suggested, the occurrence of this clinical and radiological manifestation in a background of possible or inconsistent with UIP pattern was also considered diagnostic for AE in CTD patients (14,15). Cases manifested as UIP pattern were identified based on their radiologic appearance on HRCT: the presence of basal-dominant reticular opacities and predominantly basal and subpleural distribution of honeycomb lesions, with multiple equal-sized cystic lesions of 2-10 mm diameter with a thick wall (16).…”

Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Lights and Shadows

“…Acute exacerbation (AE) is a life-threatening condition defined as rapidly deteriorating respiratory symptoms within a 1-month period with newly developed bilateral ground-glass opacities and/or consolidations on chest CT scans, superimposed on a background pattern consistent with fibrosing ILD [204]. Other than IPF, AE can also complicate secondary forms of ILD, such as RA, CTD, and chronic hypersensitivity pneumonia [205][206][207][208][209][210][211].…”

“…In RA-ILD patients, older age at ILD diagnosis, UIP pattern, and MTX have been reported as the major risk factors for AE development [212]. AE of RA-ILD can occur at any time during the disease, and occasionally it can represent the onset manifestation of ILD [205][206][207][208][209][210][211]. It has a poor prognosis and high mortality, similar to AE in IPF.…”

“…Usually, corticosteroid therapy is empirically used, with or without immunosuppressive agents and antibiotics. [205][206][207][208][209][210][211][213][214][215].…”