Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Extrapulmonary Course of Connective Tissue Disease and Survival after Lung Transplant.

Takagishi,  Troy K., Ahmed,  Iffat, Tehrani,  Rodney


Interstitial lung disease (ILD) is a common cause of morbidity and mortality in patients with connective tissue diseases (CTD). Lung transplants (LT) are becoming an increasingly viable option for the management of these patients. A review of the literature revealed no studies evaluating for extrapulmonary flares of the primary CTD following LT. Very few studies have documented the survival outcomes of this group of patients. We hypothesize that extrapulmonary disease flares rarely occur following LT and also that survival (%) is similar in patients with LT for CTD as compared to LT for COPD.


We performed an observational and retrospective chart review of all patients in a large tertiary care medical center that had a LT for CTD (LT/CTD) between 1999 and 2010 and evaluated for any extrapulmonary flares of the primary CTD following LT. Also, we compared their survival outcomes to those who underwent LT for COPD (LT/COPD). COPD was chosen as the control group because it comprises the largest group of patients undergoing LT nationally. From 1995–2008 there had been 8,417 LT due to COPD documented in the UNOS national registry which is compared to 181 for CTD during the same time period.


From 1999–2010 at a large tertiary care medical center, 15 patients underwent LT for ILD secondary to their CTD: 5 with dermatomyositis or polymyositis (33%), 5 with rheumatoid arthritis (33%), 3 with lupus (20%), and 2 with mixed connective tissue disease (13%). 4/15 (27%) of the patients had an extrapulmonary flare of their primary CTD following LT. All episodes were arthritic flares (3 monoarticular and 1 polyarticular) and each occurred in a different patient (3 with RA and 1 with SLE). This data equates to 1 flare/15.71 patient years. The Kaplan-Meier survival curves for LT/CTD and LT/COPD respectively at 3 mo was 93% and 93%; at 1 yr was 92% and 93%; at 5 yrs was 75% and 77%.


There is no published literature on the extrapulmonary manifesations of CTD following LT. There is only scarce data on the survival outcome of this patient population. We found that extrapulmonary flares of the primary CTD are extremely rare following LT likely due to the use of immunosuppressants for LT. In addition, survival following LT for CTD was found to be equivalent to that for LT for COPD. LT appears to be a viable option in a select group of patients with end stage ILD secondary to CTD.

To cite this abstract, please use the following information:
Takagishi, Troy K., Ahmed, Iffat, Tehrani, Rodney; Extrapulmonary Course of Connective Tissue Disease and Survival after Lung Transplant. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :704
DOI: 10.1002/art.28472

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