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Original Research: INTERSTITIAL LUNG DISEASE |

Unique Characteristics of Systemic Sclerosis Sine Scleroderma-Associated Interstitial Lung Disease*

Aryeh Fischer, MD; Richard T. Meehan, MD; Carol A. Feghali-Bostwick, PhD; Sterling G. West, MD; Kevin K. Brown, MD
Author and Funding Information

*From the Division of Rheumatology (Drs. Fischer and Meehan) and Interstitial Lung Disease Program (Dr. Brown), Department of Medicine, National Jewish Medical and Research Center, Denver, CO; Division of Rheumatology (Dr. West), University of Colorado Health Sciences Center, Denver, CO; and Division of Pulmonary, Allergy, and Critical Care Medicine (Dr. Feghali-Bostwick), Department of Medicine, University of Pittsburgh, Pittsburgh, PA.

Correspondence to: Aryeh Fischer, MD, Assistant Professor of Medicine, Division of Rheumatology, National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206; e-mail: fischera@njc.org



Chest. 2006;130(4):976-981. doi:10.1378/chest.130.4.976
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Study objectives: To describe the characteristics of systemic sclerosis sine scleroderma (ssSSc)-associated interstitial lung disease (ILD) presenting as idiopathic interstitial pneumonia (IIP).

Design: Retrospective review of six patients with ssSSc-associated ILD diagnosed after referral for evaluation of IIP.

Measurement and results: All patients were white, their mean age was 56 years (range, 37 to 86), and gender was evenly divided. Sclerodactyly, skin thickening, and digital edema were absent in all patients. All patients had scattered telangiectasia, and four patients had Raynaud phenomenon with abnormal nailfold capillaroscopy findings. All described gastroesophageal reflux, and three patients had esophageal dysmotility by esophagography. All had restrictive pulmonary physiology and a reduced diffusion capacity. High-resolution CT revealed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) radiographic patterns. Of the three patients who underwent surgical lung biopsy, two patients had NSIP and one patient had UIP pathologic patterns. Five patients had asymptomatic pericardial effusions and elevated pulmonary artery pressures by echocardiography. All patients had nucleolar-staining anti-nuclear antibodies (ANAs), and one patient was anti-Scl-70 positive. All five anti-Scl-70–negative patients were anti-Th/To positive, and the anti-Scl-70–positive patient was anti-Th/To negative.

Conclusions: In the presentation of an IIP, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis. These findings should aid clinicians in the evaluation and treatment of patients with otherwise undefined ILD.

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