FEV1 over time in patients with connective tissue disease-related bronchiolitis

Abstract

Summary Background Fibrosis or inflammation of the bronchioles is a well-known manifestation of connective tissue disease (CTD). However, the natural history of CTD-related bronchiolitis is largely unknown. Methods We analyzed consecutive patients evaluated at National Jewish Health (Denver, CO) from 1998 to 2008 with CTD and surgical lung biopsy-confirmed bronchiolitis. Linear mixed effects models were used to estimate the longitudinal postbronchodilator FEV1 %predicted (%pred) course and differences between subjects with or without constrictive bronchiolitis (CB). Results Of 28 subjects with a mean age of 53 ± 9 years, fourteen (50%) had CB. The most common CTD diagnosis was rheumatoid arthritis (n = 14; 50%). There were no significant differences in demographics, smoking status, underlying CTD diagnoses, 6-min walk distance, dyspnea score or drug therapy between subjects with CB and those with cellular bronchiolitis. Three subjects with CB (11%) and four with cellular bronchiolitis (14%) died. Compared with subjects with CB, those with cellular bronchiolitis had higher mean FEV1 %pred at all times. There were no significant differences in FEV1 %pred slope within- or between-groups (CB vs. cellular bronchiolitis) preceding surgical lung biopsy or afterward. Conclusion Subjects with CTD-related CB had lower FEV1 %pred values than those with CTD-related cellular bronchiolitis at all time points, but FEV1 %pred remained stable over time in both groups regardless of therapy received.

Obliterative bronchiolitis associated with rheumatoid arthritis: analysis of a single-center case series

“…This has also been observed in other studies [ 16 , 23 ] and may reflect the female predominance associated with rheumatoid arthritis. Interestingly, Fernandez Perez et al noted that male gender was an independent predictor of mortality in connective tissue disease-associated obliterative bronchiolitis [ 24 ]. Our study did not show a similar relationship between gender and mortality.…”

“…While other studies have noted that FEV1 was significantly lower in patients previously on D-penicillamine [ 19 ], we did not find evidence of worsening pulmonary impairment in patients on this agent. Fernandez Perez et al investigated the longitudinal progression of pulmonary function testing in patients with connective tissue disease associated-obliterative bronchiolitis [ 24 ]. While their study did not distinguish between underlying rheumatoid arthritis versus other connective tissue diseases, they discovered that the rate of FEV1 change was minimal during their three-year follow-up, regardless of therapy [ 24 ].…”

“…Fernandez Perez et al investigated the longitudinal progression of pulmonary function testing in patients with connective tissue disease associated-obliterative bronchiolitis [ 24 ]. While their study did not distinguish between underlying rheumatoid arthritis versus other connective tissue diseases, they discovered that the rate of FEV1 change was minimal during their three-year follow-up, regardless of therapy [ 24 ]. Similarly, our study cohort had minimal change in FEV1, suggesting the clinical and functional course of RA-OB may stabilize for a period of time in some.…”

Lung Disease in Rheumatoid Arthritis

“…Prognosis is thought to be poor; however, in a recent prospective study in CTD-associated bronchiolitis (in which 50% of patients had RA), the forced expiratory volume in 1 second showed stability over time in both forms, suggesting that bronchiolitis associated with CTD may have a less aggressive course than idiopathic disease. 54,55 …”

Obliterative Bronchiolitis as a Systemic Manifestation of Cutaneous Lupus Erythematosus

“…2,3 Among these, it has most commonly been described in rheumatoid arthritis and occasionally with Sjögren syndrome and systemic lupus erythematosus (SLE). 2,5 Cutaneous lupus erythematosus (CLE) is a localized autoimmune skin disorder that does not fulfill the required diagnostic criteria for SLE. 6 Cutaneous lupus erythematosus is no longer believed to represent a clinically homogenous skin-limited entity, as studies have reported that approximately 15% of CLE patients will progress toward SLE.…”