Chest
Original ResearchUnique Characteristics of Systemic Sclerosis Sine Scleroderma-Associated Interstitial Lung Disease
Section snippets
Materials and Methods
From August 2004 to February 2005, 235 patients were referred to the Interstitial Lung Disease Program at National Jewish Medical and Research Center in Denver, CO, for further evaluation of ILD. All of the patients underwent standardized evaluation including history and physical examination, thoracic high-resolution CT (HRCT) imaging, pulmonary physiology testing, and detailed rheumatologic serologic testing. After obtaining institutional review board approval, we retrospectively reviewed the
Results
All patients were white, mean age was 56 years (range, 37 to 86 years), and gender was evenly divided among the group (Table 1). The mean duration of dyspnea prior to referral was 11 months (range, 1 to 24 months), and mean duration of ILD diagnosis was 7 months (range, 1 to 16 months). All of the patients had restrictive pulmonary physiology and a reduced diffusion capacity. The diffusing capacity failed to normalize when corrected for alveolar volume. All patients had HRCT patterns suggestive
Discussion
In this study, we describe the clinical features of six patients with ssSSc-associated ILD diagnosed after presentation as an IIP. The diagnosis of ssSSc was based on finding the characteristic visceral and serologic manifestations of SSc in the absence of the cutaneous abnormalities characteristic of SSc (skin thickening).
We have identified several clinical features in patients with ssSSc-associated ILD that may help clinicians in the evaluation of patients with an idiopathic ILD. When a
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Chronic dyspnea with Raynaud's phenomenon and elevated ANA: A diagnosis of systemic sclerosis sine scleroderma
2023, American Journal of the Medical SciencesCitation Excerpt :Other specific antibody tests such as extractable nuclear antigen (ENA) antibodies panel for anti-Scl70, anti-SSA/Ro, anti-SSB/La, anti-Smith, anti-U1 RNP, and anti-Jo-1 are recommended if clinical suspicion for CTD is high based on clinical findings or supplant a positive ANA test.1,2,17 ANA antibodies (ab) are found in more than 94% of those with ssSSc, with the most frequent type being non-SSc-associated autoantibodies (44-50%) followed by anticentromere ab (29-50%), anti-Scl-70 ab (6-28%); anti-RNA polymerase III ab and anti-Th/To ab at 6%, respectively.1,5–7,10,20 Anti-Scl-70 ab has been associated with ssSSc-ILD and a more significant decline in PFT, whereas anticentromere ab may be considered protective against ILD but associated with an increased incidence of pulmonary arterial hypertension (PAH).2,3,11,20,21
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