Extended report
Pulmonary involvement in diffuse cutaneous systemic sclerosis:
broncheoalveolar fluid granulocytosis predicts progression of fibrosing
alveolitis
Christian Witt, Adrian C Borges, Mathias John, Ingo Fietze, Gert Baumann, Andreas Krause
Department of
Medicine, Charité University Hospital, Berlin, Germany
Correspondence to: Dr C Witt, Department of Medicine, Pneumology and Cardiology, Charité
University Hospital, Schumannstra
e 20/21, D-10098 Berlin, Germany.
Accepted for publication 28 May 1999
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.
METHODSSeventy 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.
RESULTSAt 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.
CONCLUSIONBALF
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.
© 1999 by Annals of the Rheumatic Diseases
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