Skip to main content
PMC home page
Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 1999 Oct;58(10):635–640. doi: 10.1136/ard.58.10.635

Pulmonary involvement in diffuse cutaneous systemic sclerosis: broncheoalveolar fluid granulocytosis predicts progression of fibrosing alveolitis

C Witt 1, A Borges 1, M John 1, I Fietze 1, G Baumann 1, A Krause 1
PMCID: PMC1752778  PMID: 10491363

Abstract

OBJECTIVE—The clinical course of fibrosing alveolitis (FA) in patients with systemic sclerosis (SSc) may vary considerably from stable condition for years to continuous fatal progression. This prospective study aimed at identifying the prognostic value of bronchoalveolar lavage fluid (BALF) analysis in FASSc.
METHODS—Seventy three consecutive patients with SSc and clinical signs of pulmonary involvement were enrolled. Every patient underwent clinical examination, lung function tests, computed tomography (CT), gallium scan, echocardiography, and bronchoalveolar lavage (BAL). Forty nine patients, 26 with pathological and 23 with normal BALF findings were prospectively followed up for two years and re-evaluated annually.
RESULTS—At baseline, 51 subjects (70%) showed radiological signs of lung fibrosis and/or alveolitis by CT and diffusion capacity for carbon monoxide (DLco) was decreased in 47 patients (64%). Thirty five patients (48%) had pathological BALF findings. BALF differential counts included BALF granulocytosis in 18, BALF lymphocytosis in 12, and a mixed increase of both granulocytes and lymphocytes in five patients. On follow up, a progression of FA with a significant decrease of DLco was only observed in patients with BALF granulocytosis. In contrast, patients with BALF lymphocytosis or normal BALF cell count had stable lung funtion parameters during the study period. In none of our patients echocardiography showed evidence of pulmonary hypertension.
CONCLUSION—BALF granulocytosis predicts progression of FA with deterioration of lung function, which is most sensitively monitored by DLco. Immunosuppressive treatment is recommended in patients with granulocytic FASSc.



Full Text

The Full Text of this article is available as a PDF (79.1 KB).

Figure 1  .

Figure 1  

Open in a new tab

DLco of SSc patients with normal BALF analysis, BALF lymphocytosis, and BALF granulocytosis, respectively, at baseline and after two years of follow up.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Behr J., Vogelmeier C., Beinert T., Meurer M., Krombach F., König G., Fruhmann G. Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung. Am J Respir Crit Care Med. 1996 Aug;154(2 Pt 1):400–406. doi: 10.1164/ajrccm.154.2.8756813. [DOI] [PubMed] [Google Scholar]
  2. Bolster M. B., Ludwicka A., Sutherland S. E., Strange C., Silver R. M. Cytokine concentrations in bronchoalveolar lavage fluid of patients with systemic sclerosis. Arthritis Rheum. 1997 Apr;40(4):743–751. doi: 10.1002/art.1780400422. [DOI] [PubMed] [Google Scholar]
  3. Chan K. L., Currie P. J., Seward J. B., Hagler D. J., Mair D. D., Tajik A. J. Comparison of three Doppler ultrasound methods in the prediction of pulmonary artery pressure. J Am Coll Cardiol. 1987 Mar;9(3):549–554. doi: 10.1016/s0735-1097(87)80047-5. [DOI] [PubMed] [Google Scholar]
  4. Harrison N. K., Myers A. R., Corrin B., Soosay G., Dewar A., Black C. M., Du Bois R. M., Turner-Warwick M. Structural features of interstitial lung disease in systemic sclerosis. Am Rev Respir Dis. 1991 Sep;144(3 Pt 1):706–713. doi: 10.1164/ajrccm/144.3_Pt_1.706. [DOI] [PubMed] [Google Scholar]
  5. Jacobsen S., Halberg P., Ullman S., Høier-Madsen M., Petersen J., Mortensen J., Wiik A. A longitudinal study of pulmonary function in Danish patients with systemic sclerosis. Clin Rheumatol. 1997 Jun;16(4):384–390. doi: 10.1007/BF02242456. [DOI] [PubMed] [Google Scholar]
  6. Krause A., Hohberg B., Heine F., John M., Burmester G. R., Witt C. Cytokines derived from alveolar macrophages induce fever after bronchoscopy and bronchoalveolar lavage. Am J Respir Crit Care Med. 1997 May;155(5):1793–1797. doi: 10.1164/ajrccm.155.5.9154894. [DOI] [PubMed] [Google Scholar]
  7. Müller N. L., Miller R. R. Computed tomography of chronic diffuse infiltrative lung disease. Part 1. Am Rev Respir Dis. 1990 Nov;142(5):1206–1215. doi: 10.1164/ajrccm/142.5.1206. [DOI] [PubMed] [Google Scholar]
  8. Rossi G. A., Bitterman P. B., Rennard S. I., Ferrans V. J., Crystal R. G. Evidence for chronic inflammation as a component of the interstitial lung disease associated with progressive systemic sclerosis. Am Rev Respir Dis. 1985 Apr;131(4):612–617. doi: 10.1164/arrd.1985.131.4.612. [DOI] [PubMed] [Google Scholar]
  9. Rudd R. M., Haslam P. L., Turner-Warwick M. Cryptogenic fibrosing alveolitis. Relationships of pulmonary physiology and bronchoalveolar lavage to response to treatment and prognosis. Am Rev Respir Dis. 1981 Jul;124(1):1–8. doi: 10.1164/arrd.1981.124.1.1. [DOI] [PubMed] [Google Scholar]
  10. Salaffi F., Manganelli P., Carotti M., Baldelli S., Blasetti P., Subiaco S., Binci M. C., Bichi Secchi E., Amici F., Cervini C. A longitudinal study of pulmonary involvement in primary Sjögren's syndrome: relationship between alveolitis and subsequent lung changes on high-resolution computed tomography. Br J Rheumatol. 1998 Mar;37(3):263–269. doi: 10.1093/rheumatology/37.3.263. [DOI] [PubMed] [Google Scholar]
  11. Silver R. M., Miller K. S., Kinsella M. B., Smith E. A., Schabel S. I. Evaluation and management of scleroderma lung disease using bronchoalveolar lavage. Am J Med. 1990 May;88(5):470–476. doi: 10.1016/0002-9343(90)90425-d. [DOI] [PubMed] [Google Scholar]
  12. Ungerer R. G., Tashkin D. P., Furst D., Clements P. J., Gong H., Jr, Bein M., Smith J. W., Roberts N., Cabeen W. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med. 1983 Jul;75(1):65–74. doi: 10.1016/0002-9343(83)91169-5. [DOI] [PubMed] [Google Scholar]
  13. Wallaert B., Hatron P. Y., Grosbois J. M., Tonnel A. B., Devulder B., Voisin C. Subclinical pulmonary involvement in collagen-vascular diseases assessed by bronchoalveolar lavage. Relationship between alveolitis and subsequent changes in lung function. Am Rev Respir Dis. 1986 Apr;133(4):574–580. doi: 10.1164/arrd.1986.133.4.574. [DOI] [PubMed] [Google Scholar]
  14. Weaver A. L., Divertie M. B., Titus J. L. Pulmonary scleroderma. Dis Chest. 1968 Dec;54(6):490–498. doi: 10.1378/chest.54.6.490. [DOI] [PubMed] [Google Scholar]
  15. Wells A. U., Hansell D. M., Rubens M. B., Cullinan P., Haslam P. L., Black C. M., Du Bois R. M. Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings in relation to computed tomographic appearance. Am J Respir Crit Care Med. 1994 Aug;150(2):462–468. doi: 10.1164/ajrccm.150.2.8049830. [DOI] [PubMed] [Google Scholar]
  16. Wells A. U., Hansell D. M., Rubens M. B., King A. D., Cramer D., Black C. M., du Bois R. M. Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum. 1997 Jul;40(7):1229–1236. doi: 10.1002/1529-0131(199707)40:7<1229::AID-ART6>3.0.CO;2-W. [DOI] [PubMed] [Google Scholar]
  17. Witt C., Dörner T., Hiepe F., Borges A. C., Fietze I., Baumann G. Diagnosis of alveolitis in interstitial lung manifestation in connective tissue diseases: importance of late inspiratory crackles, 67 gallium scan and bronchoalveolar lavage. Lupus. 1996 Dec;5(6):606–612. doi: 10.1177/096120339600500609. [DOI] [PubMed] [Google Scholar]

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Publishing Group

RESOURCES