Chest
Volume 130, Issue 4, October 2006, Pages 976-981
Journal home page for Chest

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

https://doi.org/10.1378/chest.130.4.976Get rights and content

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.

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

References (36)

  • JacobsenS et al.

    Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis

    J Rheumatol

    (2001)
  • SteenVD et al.

    Severe organ involvement in systemic sclerosis with diffuse scleroderma

    Arthritis Rheum

    (2000)
  • AltmanRD et al.

    Predictors of survival in systemic sclerosis (scleroderma)

    Arthritis Rheum

    (1991)
  • HubbardR et al.

    The impact of coexisting connective tissue disease on survival in patients with fibrosing alveolitis

    Rheumatology

    (2002)
  • KingTE

    Approach to the patient with interstitial lung disease

    (2004)
  • ReveilleJD et al.

    the ACR Ad HOC Committee on Immunologic Testing Guidelines. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies

    Arthritis Care Res

    (2003)
  • ReimerG et al.

    Correlates between autoantibodies to nuclear antigens and clinical features in patients with systemic sclerosis (scleroderma)

    Arthritis Rheum

    (1988)
  • ReichlinM et al.

    Antibodies to a nuclear/nucleolar antigen in patients with polymyositis overlap syndromes

    J Clin Immunol

    (1984)
  • Cited by (80)

    • Chronic dyspnea with Raynaud's phenomenon and elevated ANA: A diagnosis of systemic sclerosis sine scleroderma

      2023, American Journal of the Medical Sciences
      Citation 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

    • The lung in rheumatic disease

      2015, Rheumatology: Sixth Edition
    View all citing articles on Scopus

    The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

    View full text