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Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome.
Am J Respir Crit Care Med. 2002 Jun 15; 165(12):1581-6.AJ

Abstract

Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/ESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DL(CO)) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DL(CO) trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DL(CO) levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DL(CO) levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings.

Authors+Show Affiliations

Royal Brompton Hospital, London, United Kingdom.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Evaluation Study
Journal Article

Language

eng

PubMed ID

12070056

Citation

Bouros, Demosthenes, et al. "Histopathologic Subsets of Fibrosing Alveolitis in Patients With Systemic Sclerosis and Their Relationship to Outcome." American Journal of Respiratory and Critical Care Medicine, vol. 165, no. 12, 2002, pp. 1581-6.
Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165(12):1581-6.
Bouros, D., Wells, A. U., Nicholson, A. G., Colby, T. V., Polychronopoulos, V., Pantelidis, P., Haslam, P. L., Vassilakis, D. A., Black, C. M., & du Bois, R. M. (2002). Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. American Journal of Respiratory and Critical Care Medicine, 165(12), 1581-6.
Bouros D, et al. Histopathologic Subsets of Fibrosing Alveolitis in Patients With Systemic Sclerosis and Their Relationship to Outcome. Am J Respir Crit Care Med. 2002 Jun 15;165(12):1581-6. PubMed PMID: 12070056.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. AU - Bouros,Demosthenes, AU - Wells,Athol U, AU - Nicholson,Andrew G, AU - Colby,Thomas V, AU - Polychronopoulos,Vlasis, AU - Pantelidis,Panos, AU - Haslam,Patricia L, AU - Vassilakis,Dimitris A, AU - Black,Carol M, AU - du Bois,Roland M, PY - 2002/6/19/pubmed PY - 2002/7/27/medline PY - 2002/6/19/entrez SP - 1581 EP - 6 JF - American journal of respiratory and critical care medicine JO - Am J Respir Crit Care Med VL - 165 IS - 12 N2 - Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/ESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DL(CO)) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DL(CO) trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DL(CO) levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DL(CO) levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings. SN - 1073-449X UR - https://www.unboundmedicine.com/medline/citation/12070056/Histopathologic_subsets_of_fibrosing_alveolitis_in_patients_with_systemic_sclerosis_and_their_relationship_to_outcome_ L2 - https://www.atsjournals.org/doi/10.1164/rccm.2106012?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -