Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 241-249, December 2010

Gastroesophageal Reflux Incites Interstitial Lung Disease in Systemic Sclerosis: Clinical, Radiologic, Histopathologic, and Treatment Evidence

  • Romy B. Christmann, MD, PhD

      Affiliations

    • Assistant Professor, Rheumatology Division, University of São Paulo, São Paulo, Brazil; Scholar Researcher, Arthritis Center, Boston University School of Medicine, Boston, MA
    • Corresponding Author InformationAddress reprint requests to Romy B. Christmann, MD, PhD, Boston University School of Medicine, Arthritis Center. 715 Albany Street, E5, Boston, Massachusetts 02118-2531
    • ,
  • Athol U. Wells, MD

      Affiliations

    • Professor of Respiratory Medicine, Royal Brompton Hospital and National Heart and Lung Institute, London, UK
    • ,
  • Vera L. Capelozzi, MD, PhD

      Affiliations

    • Pathology Division, University of São Paulo, São Paulo, Brazil
    • ,
  • Richard M. Silver, MD

      Affiliations

    • Distinguished University Professor, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC

published online 24 May 2010.

Objectives

Interstitial lung disease (ILD) is currently the main cause of death in systemic sclerosis (SSc) and has an unknown pathogenesis. Gastroesophageal reflux (GER) has been strongly implicated as a cause of ILD in several lung diseases, including SSc-ILD. This review summarizes clinical, radiologic, histopathologic, and treatment aspects of GER in SSc-ILD.

Methods

The PubMed database was searched using the following keywords: “systemic sclerosis, scleroderma, interstitial lung disease, and gastroesophageal reflux.” The research was limited to English-language studies that included SSc patients with ILD.

Results

Pulmonary function tests were related with the presence of GER in several esophageal functional tests (esophageal endoscopy, pH monitoring, and manometric analysis). Regarding the histopathologic data, a pattern called centrilobular fibrosis was described in 21% of 28 lung biopsies, with a bronchocentric distribution and with an intraluminal content resembling gastric fluid. Radiologic evidence of esophageal dilation is very frequent in SSc patients, and consolidation with a patchy distribution was almost exclusively found in SSc patients with centrilobular fibrosis lung pattern. Furthermore, high levels of serum KL-6, a marker of epithelial injury, are indicative of active ILD in SSc disease.

Conclusions

The association of GER with SSc-ILD is strongly supported by several studies. An aggressive treatment for reflux is recommended in all SSc patients with ILD; however, future studies need to be performed to prove a long-term benefit.

Keywords: scleroderma, systemic sclerosis, interstitial lung disease, centrilobular fibrosis, gastroesophageal reflux, treatment

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 Richard Silver receives grant support from Actelion, Bristol Myers Squibb, Boehringer Ingelheim, and United Therapeutics and acts as Speakers bureau for Actelion, Genentech, and Takeda.

 Romy Beatriz Christmann and Athol Wells have no conflicts of interest to disclose.

PII: S0049-0172(10)00044-2

doi:10.1016/j.semarthrit.2010.03.002

Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 241-249, December 2010

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