Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis

D. Tansey, A. U. Wells, T. V. Colby, S. Ip, A. Nikolakoupolou, R. M. Du Bois, D. M. Hansell, A. G. Nicholson

Research output: Contribution to journalArticle

216 Citations (Scopus)

Abstract

Aims and methods: Pulmonary parenchymal disease is common in patients with connective tissue disorders (CTDs). However, most reports precede recognition of non-specific interstitial pneumonia (NSIP). We have therefore reviewed 54 lung biopsies from 37 patients with polymyositis/dermatomyositis (PM/DM) (n = 13), Sjögren's syndrome (n = 5), rheumatoid arthritis (n = 17) and systemic lupus erythematosus (SLE) (n = 2) to assess the overall and relative frequencies of patterns of interstitial pneumonia and their impact on prognosis. Results and conclusions: NSIP was the most common pattern with an overall biopsy prevalence of 39% and patient prevalence of 41%. There was variation in prevalence between individual CTDs, with PM/DM commonly showing organizing pneumonia (n = 5), rheumatoid arthritis showing follicular bronchiolitis (n = 6) and Sjögren's syndrome showing chronic bronchiolitis (n = 4). These patterns presented either separately or in association with NSIP, occasionally with different patterns in biopsies from separate lobes. Only four patients showed a pattern of usual interstitial pneumonia (UIP): two with rheumatoid arthritis and one each with PM/DM and SLE. Overall mortality was 24%, the most frequently associated pattern being fibrotic NSIP (n = 5). In nine cases, pulmonary presentation preceded the systemic manifestation of the CTDs. When patients with CTDs present with chronic interstitial lung disease, the most common pattern is NSIP, although there is variation in pattern prevalence between individual disorders and patterns of interstitial pneumonia frequently overlap. These data suggest a different biology for intestitial pneumonias in CTDs when compared with the idiopathic interstitial pneumonias where UIP is the most common pattern. Mortality is similar to that seen in idiopathic NSIP and, coupled with pulmonary presentation occurring prior to the systemic manifestation of disease, this may have a bearing on the origin of some cases of putative idiopathic NSIP.

Original languageEnglish (US)
Pages (from-to)585-596
Number of pages12
JournalHistopathology
Volume44
Issue number6
DOIs
StatePublished - Jun 2004

Fingerprint

Interstitial Lung Diseases
Connective Tissue
Dermatomyositis
Rheumatoid Arthritis
Idiopathic Pulmonary Fibrosis
Bronchiolitis
Biopsy
Systemic Lupus Erythematosus
Lung
Pneumonia
Idiopathic Interstitial Pneumonias
Mortality
Lung Diseases

Keywords

  • Collagen vascular disease
  • Interstitial pneumonia
  • Lung
  • Polymyositis
  • Rheumatoid arthritis
  • Sjögren's syndrome
  • SLE

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Cell Biology

Cite this

Tansey, D., Wells, A. U., Colby, T. V., Ip, S., Nikolakoupolou, A., Du Bois, R. M., ... Nicholson, A. G. (2004). Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology, 44(6), 585-596. https://doi.org/10.1111/j.1365-2559.2004.01896.x

Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. / Tansey, D.; Wells, A. U.; Colby, T. V.; Ip, S.; Nikolakoupolou, A.; Du Bois, R. M.; Hansell, D. M.; Nicholson, A. G.

In: Histopathology, Vol. 44, No. 6, 06.2004, p. 585-596.

Research output: Contribution to journalArticle

Tansey, D, Wells, AU, Colby, TV, Ip, S, Nikolakoupolou, A, Du Bois, RM, Hansell, DM & Nicholson, AG 2004, 'Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis', Histopathology, vol. 44, no. 6, pp. 585-596. https://doi.org/10.1111/j.1365-2559.2004.01896.x
Tansey, D. ; Wells, A. U. ; Colby, T. V. ; Ip, S. ; Nikolakoupolou, A. ; Du Bois, R. M. ; Hansell, D. M. ; Nicholson, A. G. / Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. In: Histopathology. 2004 ; Vol. 44, No. 6. pp. 585-596.
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abstract = "Aims and methods: Pulmonary parenchymal disease is common in patients with connective tissue disorders (CTDs). However, most reports precede recognition of non-specific interstitial pneumonia (NSIP). We have therefore reviewed 54 lung biopsies from 37 patients with polymyositis/dermatomyositis (PM/DM) (n = 13), Sj{\"o}gren's syndrome (n = 5), rheumatoid arthritis (n = 17) and systemic lupus erythematosus (SLE) (n = 2) to assess the overall and relative frequencies of patterns of interstitial pneumonia and their impact on prognosis. Results and conclusions: NSIP was the most common pattern with an overall biopsy prevalence of 39{\%} and patient prevalence of 41{\%}. There was variation in prevalence between individual CTDs, with PM/DM commonly showing organizing pneumonia (n = 5), rheumatoid arthritis showing follicular bronchiolitis (n = 6) and Sj{\"o}gren's syndrome showing chronic bronchiolitis (n = 4). These patterns presented either separately or in association with NSIP, occasionally with different patterns in biopsies from separate lobes. Only four patients showed a pattern of usual interstitial pneumonia (UIP): two with rheumatoid arthritis and one each with PM/DM and SLE. Overall mortality was 24{\%}, the most frequently associated pattern being fibrotic NSIP (n = 5). In nine cases, pulmonary presentation preceded the systemic manifestation of the CTDs. When patients with CTDs present with chronic interstitial lung disease, the most common pattern is NSIP, although there is variation in pattern prevalence between individual disorders and patterns of interstitial pneumonia frequently overlap. These data suggest a different biology for intestitial pneumonias in CTDs when compared with the idiopathic interstitial pneumonias where UIP is the most common pattern. Mortality is similar to that seen in idiopathic NSIP and, coupled with pulmonary presentation occurring prior to the systemic manifestation of disease, this may have a bearing on the origin of some cases of putative idiopathic NSIP.",
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