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Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis

Nature Reviews Rheumatology volume 5, pages 225–228 (2009)Cite this article

Abstract

Background. A 36-year-old woman developed new-onset Raynaud phenomenon and rapidly progressive dyspnea over a 2-week period. A lung biopsy demonstrated pauci-inflammatory nonspecific pneumonitis, which proved refractory to systemic corticosteroid and intravenous cyclophosphamide therapy. Her preterminal course in an intensive care unit was typified by sequential organ failure. Postmortem examination showed extensive organ fibrosis, including severe diffuse alveolar damage and parenchymal fibrosis, and a notable lack of potentially treatable tissue inflammation.

Investigations. Chest radiography, physical examination, screening for autoantibodies, measurement of serum creatinine, creatine phosphokinase, and brain natriuretic peptide levels, cardiac examination, pulmonary function tests, electrocardiography, transthoracic Doppler echocardiography, right heart catheterization, high-resolution thoracic CT, pulmonary ventilation/perfusion scan, lung biopsy.

Diagnosis. Interstitial lung disease associated with diffuse systemic sclerosis.

Management. Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide.

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Figure 1: Histological sample taken at autopsy from a patient with interstitial lung disease associated with systemic sclerosis, showing severe diffuse alveolar damage and interstitial fibrosis, with a notable lack of parenchymal inflammation.

References

  1. Steen, V. D. & Medsger, T. A. Changes in causes of death in systemic sclerosis, 1972–2002. Ann. Rheum. Dis. 66, 940–944 (2007).

    Article  Google Scholar 

  2. Ostojic, P. & Damjanov, N. Different clinical features in patients with limited and diffuse cutaneous systemic sclerosis. Clin. Rheumatol. 25, 453–457 (2006).

    Article  Google Scholar 

  3. Ostojic, P. et al. Interstitial lung disease in systemic sclerosis. Lung 185, 211–220 (2007).

    Article  Google Scholar 

  4. Hoyles, R. K. et al. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 54, 3962–3970 (2006).

    Article  CAS  Google Scholar 

  5. Highland, K. B. et al. The spectrum of scleroderma lung disease. Semin. Respir. Crit. Care Med. 28, 418–429 (2007).

    Article  Google Scholar 

  6. Diot, E. et al. Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest 114, 1623–1629 (1998).

    Article  CAS  Google Scholar 

  7. Goh, N. S. et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am. J. Respir. Crit. Care Med. 177, 1248–1259 (2008).

    Article  Google Scholar 

  8. Plastiras, S. C. et al. Scleroderma lung: initial forced vital capacity as predictor of pulmonary function decline. Arthritis Rheum. 55, 598–602 (2006).

    Article  Google Scholar 

  9. Morgan, C. et al. Predictors of end stage lung disease in a cohort of patients with scleroderma. Ann. Rheum. Dis. 62, 146–150 (2003).

    Article  CAS  Google Scholar 

  10. Baughman, R. P. & Raghu, G. Bronchoalveolar cellular analysis in scleroderma lung disease: does Sutton's law hold? Am. J. Respir. Crit. Care Med. 177, 2–3 (2008).

    Article  Google Scholar 

  11. Hsu, V. M. et al. Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: a comparison of Doppler echocardiography, cardiac MRI, and pulmonary function test to right heart catheterization. J. Rheumatol. 35, 458–465 (2008).

    PubMed  Google Scholar 

  12. Strange, C. & Seibold, J. R. Scleroderma lung disease: “if you don't know where you are going, any road will take you there”. Am. J. Respir. Crit. Care Med. 177, 1178–1179 (2008).

    Article  Google Scholar 

  13. Khanna, D. et al. Oral cyclophosphamide for active scleroderma lung disease: a decision analysis. Med. Decis. Making 28, 926–937 (2008).

    Article  Google Scholar 

  14. Tashkin, D. P. et al. Cyclophosphamide versus placebo in scleroderma lung disease. N. Engl. J. Med. 354, 2655–2666 (2006).

    Article  CAS  Google Scholar 

  15. Tashkin, D. P. et al. Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am. J. Respir. Crit. Care Med. 176, 1026–1034 (2007).

    Article  CAS  Google Scholar 

  16. Nannini, C. et al. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and a meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res. Ther. 10, R124 (2008).

    Article  Google Scholar 

  17. Wells, A. U. et al. Daily cyclophosphamide for scleroderma: are patients with the most to gain underrepresented in this trial? Am. J. Respir. Crit. Care Med. 176, 952–953 (2007).

    Article  Google Scholar 

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Authors and Affiliations

  1. University of Michigan, Ann Arbor, MI, USA

    Kristine Phillips, Cathryn Byrne-Dugan, Eric Batterson & James R. Seibold

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  1. Kristine Phillips
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  2. Cathryn Byrne-Dugan
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  3. Eric Batterson
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  4. James R. Seibold
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Correspondence to James R. Seibold.

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The authors declare no competing financial interests.

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Phillips, K., Byrne-Dugan, C., Batterson, E. et al. Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis. Nat Rev Rheumatol 5, 225–228 (2009). https://doi.org/10.1038/nrrheum.2009.30

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