Skip to main content
Log in

Bronchoalveolar lavage in systemic sclerosis with lung involvement: role and correlations with functional, radiological and scintigraphic parameters

  • Original Article
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

To evaluate the role and the prognostic value of bronchoalveolar lavage (BAL) in scleroderma patients with interstitial lung disease. We reviewed the records of 79 patients with systemic sclerosis (SSc) who had dyspnea and pulmonary involvement and underwent BAL study. Sixty-two patients were prospectively followed up for 12–36 months and re-evaluated by pulmonary function tests (PFTs). Seventy-nine SSc patients were enrolled (71 F and 8 M), 55 with limited and 24 with a diffuse form; mean age 55 ± 13 years; mean disease duration 55.2 ± 59 months. All patients were ANA positive, of these 30 were anti-topoisomerase-1 positive (anti-Topo1) and 22 were anti-centromere positive (ACA). Thirty-one patients had alveolitis (39.2%) that was neutrophilic in 12 patients, eosinophilic in 3 and mixed (neutrophilic and eosinophilic) in 16 patients. Compared to patients without alveolitis, those with alveolitis had a significant reduction of carbon monoxide diffusing capacity (DLCO), forced vital capacity (FVC) and more elevated lung high-resolution computed tomography (HRCT) scores. Furthermore, alveolar clearance was significantly accelerated. No differences were found between patients with and without alveolitis regarding disease subsets (diffuse vs limited-SSc); a significant predominance of anti-Topo1 antibodies was found in the alveolitis group and of ACA antibodies in the non-alveolitis cohort. During the follow-up, (range: 12–36 months) 62 patients, 26 with and 36 without alveolitis were re-evaluated with PFTs. In the alveolitis group, 12 patients (46.1%) showed stable lung function parameters and 14 had worsened (53.8%). In this group, 20 patients (77%) received cyclophosphamide (CYC): 11 (55%) worsened (5 of them died of cardio-pulmonary complications) and 9 (45%) remained stable. Six patients could not be treated; of these 3 remained stable and 3 worsened. Among 36 patients with normal BAL, 11 (30.5%) showed stable lung function parameters, 13 improved (36.1%) and 12 worsened (33.3%); in this last group, 2 patients died of extra-pulmonary complications. Six patients, with progression of lung fibrosis, were treated with CYC: 3 of them improved and 3 remained stable. Our study revealed a trend toward a more severe course in the SSc patients with BAL alveolitis; probably the non-significant result is related to the low number of the examined subjects and to the selection criteria. However, BAL remains the only tool to exclude lung infections and, in our experience, a useful instrument to evaluate interstitial lung disease in SSc patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37:1283–1289. doi:10.1002/art.1780370903

    Article  PubMed  CAS  Google Scholar 

  2. White B, Moore WC, Wigley FM, Xiao HQ, Wise RA (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132:947–954

    PubMed  CAS  Google Scholar 

  3. Baker AM, Bowton DL, Haponik EF (1995) Decision making in nosocomial pneumonia. An analytic approach to the interpretation of quantitative bronchoscopic cultures. Chest 107:85–95. doi:10.1378/chest.107.1.85

    Article  PubMed  CAS  Google Scholar 

  4. Strange C, Bolster MB, Roth MD et al (2008) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 177:91–98. doi:10.1164/rccm.200705-655OC

    Article  PubMed  CAS  Google Scholar 

  5. Subcommittee for Progressive Systemic Sclerosis (1980) Criteria of the American rheumatism association diagnostic and therapeutic criteria committee: preliminary criteria for the classification of systemic sclerosis (Scleroderma). Arthritis Rheum 23:581–590. doi:10.1002/art.1780230510

    Article  Google Scholar 

  6. LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (Systemic sclerosis): classification, subset and pathogenesis. J Rheumatol 15:202–205

    PubMed  CAS  Google Scholar 

  7. Kahaleh MB, Sultan GL, Smith EA et al (1986) A modified scleroderma skin scoring method. Clin Exp Rheumatol 4:367–369

    PubMed  CAS  Google Scholar 

  8. Sato S, Hamaguchi Y, Hasegawa M, Takeara K (2001) Clinical significance of antitopoisomerase I antibody levels determined by ELISA in systemic sclerosis. Rheumatology 40:1135–1140. doi:10.1093/rheumatology/40.10.1135

    Article  PubMed  CAS  Google Scholar 

  9. Mahler DA, Weinberg DH, Wells CK, Feinstein AR (1984) The measurement of dyspnea: contents, interobserver agreement, and physiologic correlates of two new clinical indexes. Chest 85:751–758. doi:10.1378/chest.85.6.751

    Article  PubMed  CAS  Google Scholar 

  10. Klech H, Pohl W (1989) Technical recommendations and guidelines for bronchoalveolar lavage (BAL). Report of the European society of pneumology task group on BAL. Eur Respir J 2:561–585

    Google Scholar 

  11. Behr J, Vogelmeier C, Beinert T et al (1996) Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung. Am J Respir Crit Care Med 154:400–406

    PubMed  CAS  Google Scholar 

  12. The Joint statement of the ATS and ETS (2000) Idiopathic pulmonary fibrosis: diagnosis and treatment, International Consensus Statement. Am J Respir Crit Care Med 161:646–664

    Google Scholar 

  13. American Thoracic Society (1995) Standardization of spirometry-1994 update. Am J Respir Crit Care Med 152:1107–1136

    Google Scholar 

  14. American Thoracic Society (1995) Single breath carbon monoxide diffusing capacity (transfer factor). Recommendation for a standard technique. Am J Respir Crit Care Med 152:2185–2198 Update (1995)

    Google Scholar 

  15. Warrick JH, Balla M, Schabel SI, Silver RM (1991) High resolution computer tomography in early scleroderma lung disease. J Rheumatol 18:1520–1528

    PubMed  CAS  Google Scholar 

  16. Coates G, O’Brodovich H (1986) Measurement of pulmonary epithelial permeability with Tc-99 m-DTPA aerosol. Semin Nucl Med 16:275–284. doi:10.1016/S0001-2998(86)80014-9

    Article  PubMed  CAS  Google Scholar 

  17. Lo Monaco A, La Corte R, Prandini N et al (2005) Alveolar Clearance of 99 mTc-DTPA, Pulmonary high resolutioon CT and respiratory function tests correlations in the evaluation of lung involvement in 207 patients with systemic sclerosis. Arthritis Rheum 52(suppl):S163

    Google Scholar 

  18. Kon OM, Danil Z, Black CM, du Bois RM (1999) Clearance of inhaled Tc-99m-DTPA as a clinical index of pulmonary vascular disease in systemic sclerosis. Eur Respir J 13:133–136. doi:10.1183/09031936.99.13113399

    Article  PubMed  CAS  Google Scholar 

  19. Diot E, Giraudeau B, Maillot F, Diot P (1999) Decrease in DLCO in systemic sclerosis correlates with acceleration of DTPA clearance. Eur Respir J 14:728–729. doi:10.1034/j.1399-3003.1999.14c42.x

    Article  PubMed  CAS  Google Scholar 

  20. Wells AU, Hansell DM, Rubens MB et al (1994) Fibrosing alveolitis in systemic sclerosis: bronchoalveolar lavage findings in relation to computed tomographic appearances. Am J Respir Crit Care Med 150:462–468

    PubMed  CAS  Google Scholar 

  21. Wells AU, Hansell DM, Haslam PL et al (1998) Bronchoalveolar lavage cellularity: lone cryptogenic fibrosing alveolitis compared with the fibrosing alveolitis of systemic sclerosis. Am J Respir Crit Care Med 157:1474–1482

    PubMed  CAS  Google Scholar 

  22. Goh NSL, Veeraraghavan S, Desai SR et al (2007) Bronchoalveolar lavage and cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 56:2005–2012. doi:10.1002/art.22696

    Article  PubMed  Google Scholar 

  23. Clements PJ, Goldin JG, Kleerup EC et al (2004) Regional differences in bronchoalveolar lavage and thoracic high-resolution computed tomography results in dyspneic patients with Systemic Sclerosis. Arthritis Rheum 50:1909–1917. doi:10.1002/art.20265

    Article  PubMed  Google Scholar 

  24. Fathi N, Furst DE, Clements PJ (2007) Management of Interstitial lung disease in systemic sclerosis: lessons from SLS and FAST. Curr Rheumatol Rep 9:144–150. doi:10.1007/s11926-007-0009-y

    Article  PubMed  CAS  Google Scholar 

Download references

Disclosures

I would like to make the following statements: All authors have contributed significantly to the research described in the paper and have read and approved the final manuscript; the manuscript is the original work of the authors; neither this manuscript nor another manuscript with substantially similar content under our authorship has been published, has been accepted, or is being considered elsewhere for publication; there are no affiliations or financial involvement with any entity with a financial interest in the subject matter. No conflict of interest; all individuals who have contributed significantly to the work reported in this manuscript are acknowledged by name and have granted permission to be named.

Conflict of interest statement

All the authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Stefania Volpinari.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Volpinari, S., La Corte, R., Bighi, S. et al. Bronchoalveolar lavage in systemic sclerosis with lung involvement: role and correlations with functional, radiological and scintigraphic parameters. Rheumatol Int 31, 1183–1188 (2011). https://doi.org/10.1007/s00296-010-1390-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-010-1390-9

Keywords

Navigation