Variable course of disease of rheumatoid arthritis-associated usual interstitial pneumonia compared to other subtypes

BMC Pulmonary Medicine, Jul 2016

Background In rheumatoid arthritis-associated interstitial lung disease (RA-ILD), occurring in 10 % of patients with patients with RA, usual interstitial pattern (UIP) has shown to associate with poor prognosis but more detailed data about the course of the disease in different subtypes is limited. Our aim was to compare the disease course of patients with RA-ILD categorized into either UIP or other types of ILDs. Methods Clinical and radiological information of 59 patients with RA-ILD were re-assessed and re-classified into UIP or non-UIP groups, followed by a between-group comparison of demographic data, lung function, survival, cause of death and comorbidities. Results The majority of patients (n = 35/59.3 %) showed a radiological UIP-like pattern in high resolution computed tomography. The median survival was 92 months (95 % CI 62.8–121.2) in the UIP-group and 137 months (95 % CI 31.0–243.0) in the non-UIP-group (p = 0.417). Differences in course of disease were found in the number of hospitalizations for respiratory reasons (mean 1.9 ± 2.6 in UIP vs. 0.5 ± 0.9 in non-UIP group, p = 0.004), the use of oxygen therapy (8/22.9 % UIP patients vs. 0 non-UIP patients, p = 0.016), number of deaths (23/65.7 % vs. 10/41.7 %, p = 0.046) and decline in diffusion capacity (56 ± 20.6 vs. 69 ± 20.2, p = 0.021). Dyspnea and inspiratory crackles were detected more often in the UIP group. RA-ILD was the most common primary cause of death (39.4 % of cases). Hypertension, coronary artery disease, chronic obstructive pulmonary disease, heart insufficiency, diabetes and asthma were common comorbidities. ILD preceded RA diagnosis in 13.6 % of patients. Conclusions The course of the disease in RA-UIP patients is different from the other RA-ILD subtypes. Several comorbidities associated commonly with RA-ILD, although ILD was the predominant primary cause of death.

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Variable course of disease of rheumatoid arthritis-associated usual interstitial pneumonia compared to other subtypes

Nurmi et al. BMC Pulmonary Medicine Variable course of disease of rheumatoid arthritis-associated usual interstitial pneumonia compared to other subtypes Hanna M. Nurmi 0 1 Minna K. Purokivi 1 Miia S. Kärkkäinen 0 5 Hannu-Pekka Kettunen 4 Tuomas A. Selander 3 Riitta L. Kaarteenaho 0 1 2 0 Division of Respiratory Medicine, Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences, University of Eastern Finland , POB 1627, 70211 Kuopio , Finland 1 Center of Medicine and Clinical Research, Division of Respiratory Medicine, Kuopio University Hospital , POB 100, 70029 KYS Kuopio , Finland 2 Respiratory Medicine, Internal Medicine Research Unit, Medical Research Center Oulu, Oulu University Hospital and University of Oulu , POB 20, 90029 Oulu , Finland 3 Science Services Center, Kuopio University Hospital , POB 100, 70029 Kuopio , Finland 4 Diagnostic Imaging Center, Division of Radiology, Kuopio University Hospital , POB 100, 70029 Kuopio , Finland 5 Harjula Hospital, the Municipal Hospital of Kuopio , Niuvantie 4, 70101 Kuopio , Finland Background: In rheumatoid arthritis-associated interstitial lung disease (RA-ILD), occurring in 10 % of patients with patients with RA, usual interstitial pattern (UIP) has shown to associate with poor prognosis but more detailed data about the course of the disease in different subtypes is limited. Our aim was to compare the disease course of patients with RA-ILD categorized into either UIP or other types of ILDs. Methods: Clinical and radiological information of 59 patients with RA-ILD were re-assessed and re-classified into UIP or non-UIP groups, followed by a between-group comparison of demographic data, lung function, survival, cause of death and comorbidities. Results: The majority of patients (n = 35/59.3 %) showed a radiological UIP-like pattern in high resolution computed tomography. The median survival was 92 months (95 % CI 62.8-121.2) in the UIP-group and 137 months (95 % CI 31.0-243.0) in the non-UIP-group (p = 0.417). Differences in course of disease were found in the number of hospitalizations for respiratory reasons (mean 1.9 ± 2.6 in UIP vs. 0.5 ± 0.9 in non-UIP group, p = 0.004), the use of oxygen therapy (8/22.9 % UIP patients vs. 0 non-UIP patients, p = 0.016), number of deaths (23/65.7 % vs. 10/41.7 %, p = 0.046) and decline in diffusion capacity (56 ± 20.6 vs. 69 ± 20.2, p = 0.021). Dyspnea and inspiratory crackles were detected more often in the UIP group. RA-ILD was the most common primary cause of death (39.4 % of cases). Hypertension, coronary artery disease, chronic obstructive pulmonary disease, heart insufficiency, diabetes and asthma were common comorbidities. ILD preceded RA diagnosis in 13.6 % of patients. Conclusions: The course of the disease in RA-UIP patients is different from the other RA-ILD subtypes. Several comorbidities associated commonly with RA-ILD, although ILD was the predominant primary cause of death. High-resolution computed tomography; Cause of death; Comorbidity Background Interstitial lung disease (ILD) is a rather common extraarticular manifestation of rheumatoid arthritis (RA) and a major cause of morbidity and mortality in RA patients [ 1, 2 ]. Approximately 10 % of patients with RA may develop clinically evident ILD with respiratory symptoms and/or a decline in pulmonary function tests [3]. In asymptomatic RA patients, high-resolution computed tomography (HRCT) scans commonly reveal evidence of interstitial lung involvement, and a large proportion of those with subclinical disease deteriorate with time [ 4, 5 ]. However, the clinical course of RA-ILD is highly heterogenic, as some patients remain stable for years, even decades, while others develop an insidious progressive disease [6]. While the overall mortality in RA has declined, the numbers of deaths due to RA-ILD have increased [ 7 ], although the results of studies investigating survival have been variable. Some studies have reported survival of 3 years, similar to that of idiopathic pulmonary fibrosis (IPF) [ 8, 9 ], whereas in others the prognosis of RA-ILD has been significantly better, with median survival of approximately 6–8 years [ 10, 11 ]. Since it lacks its own distinctive classification, the subtypes of RA-ILD have been categorized according to the subdivisions of the idiopathic interstitial pneumonias (IIP) [ 12 ]. Unlike the situation in other connective tissue diseases (CTD), the most common radiologic and histopathologic pattern of RA-ILD is usual interstitial pneumonia (UIP), whereas nonspecific interstitial pneumonia (NSIP) and other subtypes also exist to a lesser extent [ 13 ]. The clinical significance of these different histological and radiological patterns has become nowadays more important since the RA-ILD patient with the UIP pattern (RA-UIP) seems to have a significantly worse prognosis and reduced survival compared to other types such as NSIP and organizing pneumonia (OP) [ 11, 14–16 ]. Other diff (...truncated)


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Hanna Nurmi, Minna Purokivi, Miia Kärkkäinen, Hannu-Pekka Kettunen, Tuomas Selander, Riitta Kaarteenaho. Variable course of disease of rheumatoid arthritis-associated usual interstitial pneumonia compared to other subtypes, BMC Pulmonary Medicine, 2016, pp. 107, 16, DOI: 10.1186/s12890-016-0269-2