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Idiopathic or connective tissue disease-associated interstitial lung disease: a case of HRCT mimicry
  1. Kevin R Flaherty1,
  2. Dinesh Khanna2
  1. 1Division of Pulmonary/Critical Care Medicine, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA
  2. 2Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA
  1. Correspondence to Dr Kevin R Flaherty, Division of Pulmonary/Critical Care Medicine, Department of Internal Medicine, University of Michigan, 1500 E. Medical Center Drive, 3916 Taubman Center, Ann Arbor, MI 48109, USA; flaherty{at}umich.edu

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Interstitial lung disease (ILD) induces overwhelming morbidity and kills more patients with connective tissue disease (CTD) than any other CTD-related manifestation.1–5 Because of this, there is a keen and growing interest in the rheumatology and pulmonary communities to identify prognostic variables for CTD-associated ILD (CTD-ILD). Treatment options for patients with ILD, idiopathic as well as those associated with CTD-ILD are limited. Determining prognosis is important, especially when considering therapeutic options such as lung transplantation as well as monitoring response to therapy.

High-resolution CT (HRCT) plays a central role in the diagnostic evaluation of patients with ILDs.6 Furthermore, features such as honeycombing and severity of fibrosis can both serve as diagnostic surrogates for surgical lung biopsy as well as predict the risk of subsequent mortality for patients with idiopathic interstitial pneumonias (IIP).7–9 Less is published regarding the prognostic characteristics of features seen on HRCT in patients with CTD-ILD; diseases that often have a better prognosis compared with patients with IIP10 ,11 and where surgical lung biopsy is less often performed.

Walsh et al12 evaluated the ability of radiographic and pulmonary function characteristics to predict subsequent prognosis in patients with CTD-ILD. They included a relatively large and diverse study population consisting of 168 patients with a variety of CTDs such as rheumatoid arthritis (n=39), systemic sclerosis (SSc) (n=32), mixed CTD (n=33), polymositis (n=33), systemic lupus erythematosus (n=17) and Sjogren's disease (n=14). Fifty-one patients had surgical lung biopsy available for evaluation.

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Footnotes

  • Competing interests DK was supported by NIH/NIAMS K24AR063120-02.

  • KRF was supported by NIH/NHLBI T32HL00749, K24PA10-061, R01HL091743, RC2 HL101740-01, P50 HL107177 and U01 HL105371.

  • Provenance and peer review Not commissioned; internally peer reviewed.

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