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.

Clinical Characteristics of Systemic Sclerosis Sine Scleroderma Associated Interstitial Lung Disease.

Aggarwal3,  Rohit, Lucas2,  Mary, Fertig2,  Noreen, Medsger1,  Thomas A.

Univ of Pittsburgh, Pittsburgh, PA
University of Pittsburgh
University of Pittsburgh Medical Center, Pittsburgh, PA


To describe the clinical characteristics features of systemic sclerosis sine scleroderma associated interstitial lung disease from a single tertiary care center over a 36- year period.


All 3322 patients from the tertiary care database with a clinical diagnosis of systemic sclerosis (SSc) with first visit between 1972–2008 were evaluated. Patients were classified as SSc-sine scleroderma (ssSS) if they had a clinical diagnosis of SSc with no skin thickening (Modified Rodnan skin score = 0) on physical examination and one or more of the following visceral involvement typical of SSc: distal esophageal hypomotility, pulmonary interstitial fibrosis, pulmonary arterial hypertension, SSc cardiac or renal disease. Pulmonary interstitial fibrosis was defined as the presence of pulmonary fibrosis seen on chest radiography (CXR) or high-resolution computed tomography (HRCT) scan, with or without pulmonary function criteria for restrictive lung disease: forced vital capacity in 1 second [FVC1] < 70% of predicted plus forced expiratory volume [FEV1] in 1 second/FVC > 80% of predicted). All SSss patients with pulmonary fibrosis at first visit formed our final cohort of SSss-ILD patients, who were further studied for clinical and serological features. Serological evaluation for all SSc specific/associated autoantibodies was done on all patients with available serum.


Our final cohort had 45 SSss patients with ILD at baseline. Demographics were: female (36/45, 80%), Caucasians (37/45, 82.2%), African American (7/45, 15.6 %). The mean (±SD) age (yrs) at first CTD symptom, first CTD diagnosis and first visit were 42.5 (17.2), 49.6 (16.9), and 53.5 (15.5), respectively. Mean diagnosis delay from first CTD symptom was 8.1 (9.6) yrs. Mean age at first pulmonary symptom was 51.5 (15.0) yrs. Mean duration from first non-pulmonary CTD symptom to pulmonary symptom was 8.6 (10.5) yrs, and most common first symptom was raynauds 29/45 (64.4%), followed by dyspnea (5/45, 11.1%), GI involvement (3/45, 6.7%). Most patients had multiple clinical features for SSc before the development of pulmonary disease: raynauds 36/41, 87.8 (median delay of 14.5 years from raynauds to pulmonary symptoms), arthritis 76.4%, esophageal symptoms 60.0%. 50% patients (18/36) had pulmonary symptoms before SSss diagnosis. Among various SSc-specific autoantibodies most common was ThTo 14/38 (36.8%), followed by U1 (or U2) 9/38 (23.7%), centromere 5/38. Positive ANA was seen in 93.3% (42/45) patients. Approximately 50% of patients (19/41, 46.3%) had nucleolar pattern, followed by speckled 10/41, centeromere 5/41. Most common clinical features at baseline evaluation were raynaud 39/45 (86.6%), telangiectasia 20/44, digital swelling 16/43, pyrositis 17/44, distal dysphagia 10/44. The Median, 5 and 10 year survival from the diagnosis was 16.9 years, 83.7% and 68.5% respectively.


Clinical features like Raynaud, arthritis and esophageal symptom generally precedes the development of pulmonary fibrosis and thus along with positive ANA can help diagnose SSss associated ILD. Nucleolar ANA pattern and anti-ThTo and anti-U1/U2 were the most common autoantibodies seen in SSss-ILD patients.

To cite this abstract, please use the following information:
Aggarwal, Rohit, Lucas, Mary, Fertig, Noreen, Medsger, Thomas A.; Clinical Characteristics of Systemic Sclerosis Sine Scleroderma Associated Interstitial Lung Disease. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :563
DOI: 10.1002/art.28332

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