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.

Shrinking Lung Syndrome Is Associated with Pleuritis.

Allen3,  David, Bshouty3,  Zoheir, Moyen3,  Mellissa, Robinson3,  David B., Peschken1,  Christine A., Hitchon3,  Carol A., El-Gabalawy2,  Hani S.

Univ of Manitoba, Winnipeg, MB, Canada
University of Manitoba, Winnipeg, MB, Canada
University of Manitoba, Winnipeg, MB, Canada


Shrinking lung syndrome (SLS) is a form of pulmonary damage in systemic lupus erythematosus (SLE) characterized by unexplained dyspnea, small lung volumes and restrictive lung physiology with or without diaphragmatic elevation. The pathophysiology of SLS is incompletely understood but is associated with significant morbidity and may require escalation of therapy to avoid fulminant respiratory failure. Pleuritis has been reported in 30–60% of patients with SLS. We set out to determine the frequency of SLS in patients with SLE and if pleuritis is associated with SLS.


Consecutive patients >= 18 years of age who met American College of Rheumatology (ACR) criteria for SLE were enrolled. Demographics, disease duration, smoking status, body-mass index (BMI), self-reported dyspnea, pleuritic chest pain (CP), autoantibodies and measures of disease activity; SLE Activity Measure (SLAM), cumulative organ damage (Systemic Lupus International Collaborating Clinics damage index (SDI), ACR criteria modified to exclude pulmonary variables (mACR) were recorded. All patients underwent pulmonary function testing (PFTs) and chest imaging (X-ray or CT scan).

Pleuritis was defined as serositis by ACR criteria, relying on the presence of CP, a rub or a history of pleural effusion on chest X-ray. At enrollment, SLS was defined as a forced vital capacity (FVC) or total lung capacity (TLC) <80% predicted without evidence of interstitial/pleural disease on imaging or myositis/myalgia causing impairment.

Univariate and multivariate logistic regression was used to determine factors associated with SLS and the relationship between pleuritis and SLS. Results are reported as mean ± SD unless stated otherwise.


One hundred patients (94% women, 81% Caucasian) with an age at diagnosis of 33.8 ± 13.8 years, disease duration of 13.2 ± 9.2 years, had a mACR of 5.5 ± 1.2, total SDI of 1.2 ± 1.2, and total SLAM of 7.1 ± 3.6. Fifty-four of 99 reported >= 1 respiratory symptom; 52/99 had dyspnea and 26/99 had CP. Thirty-four had SLS; 23/33 had dyspnea and 13/33 had CP. Dyspnea and CP was more common in those with SLS [n=25/34 (71%) vs n=27/65 (42%), p=0.006] and [n=15/34 (43%) vs n=11/64 (17%), p=0.007].

Thirty-five patients had pleuritis. SLS was more common among those with pleuritis compared to those without, [26/34 (57%) vs. 14/65 (22%), (p<0.0001)]. As well, disease duration (p=0.05), self-reported dyspnea (p=0.02), anti-RNP (p=0.001), anti-Sm (p=0.002), and mACR (p=0.02) were significantly associated with SLS. However, age at diagnosis, BMI, pCP, ethnicity, smoking status, SDI, SLAM and anti-dsDNA, anti-Ro, and anti-La, were not associated with SLS.

In multivariate analysis controlling for pleuritis, disease duration, self-reported dyspnea, mACR, presence of anti-RNP and Sm antibodies, only pleuritis remained associated with SLS [OR= 3.6, 95% CI of 1.3–10.1, p=0.02].


SLS was a frequent finding in this unselected cohort, occurring in 57% of patients and pleuritis was significantly associated with SLS. This suggests that pleural disease may be important in the etiology of SLS. Further, the presence of pleural disease in patients with SLE should result in an assessment for SLS.

To cite this abstract, please use the following information:
Allen, David, Bshouty, Zoheir, Moyen, Mellissa, Robinson, David B., Peschken, Christine A., Hitchon, Carol A., et al; Shrinking Lung Syndrome Is Associated with Pleuritis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1176
DOI: 10.1002/art.28942

Abstract Supplement

Meeting Menu