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Rheumatology Advance Access published online on February 16, 2005
Rheumatology, doi:10.1093/rheumatology/keh575
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© British Society for Rheumatology 2005; all rights reserved
Received May 18, 2003
Accepted January 14, 2005

Original Papers

Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD)

E. Bodolay 1*, Z. Szekanecz 2, K. Dévényi 3, L. Galuska 4, I. Csípo 1, J. Vègh 1, I. Garai 4, and G. Szegedi 1

1 Division of Clinical Immunology, University of Debrecen, Medical and Health Science Center, Debrecen, H-4004, Hungary
2 Division of Rheumatology, 3rd Department of Internal Medicine, University of Debrecen, Medical and Health Science Center, Debrecen, H-4004, Hungary
3 Department of Radiology, University of Debrecen, Medical and Health Science Center, Debrecen, H-4004, Hungary
4 Department of Nuclear Medicine, University of Debrecen, Medical and Health Science Center, Debrecen, H-4004, Hungary

* To whom correspondence should be addressed.
E. Bodolay, E-mail: bodolai{at}iiibel.dote.hu


  Abstract

Objective. Interstitial lung disease (ILD) may be a characteristic, often serious, manifestation of mixed connective tissue disease (MCTD). In this retrospective study, the frequency and clinical picture of ILD were determined in patients with MCTD using two diagnostic tests: high-resolution computed tomography (HRCT) and inhaled aerosol clearance times of 99mTc-labelled diethylene-triamine pentaacetate (99mTc-DTPA). In addition, pulmonary function, effects of therapy and a variety of immunoserological markers were also assessed.

Methods. One hundred and forty-four consecutive patients with MCTD were selected from the clinic, irrespective of the presence or absence of ILD. All patients underwent a detailed clinical assessment, chest HRCT scanning, chest radiography, inhaled aerosol of 99mTc-DTPA clearance times, and all pulmonary function tests. Patients who had active ILD received corticosteroid (CS) or CS in combination with cyclophosphamide (CPH). All investigations were repeated after 6 months of immunosuppressive therapy.

Results. Ninety-six out of 144 MCTD patients (66.6%) had active ILD, 75 of this group (78.1%) showed ground glass opacity, 21 patients (21.8%) ground glass opacity with mild fibrosis with HRCT. Forty-five patients with active ILD received 2 mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2 mg/kg/day). Six months later, after therapy, 67 out of 96 MCTD patients with ILD (69.8%) showed a negative HRCT pattern, ground glass opacity with mild fibrosis developed in 15 patients (15.6%), and fibrosis was detected in 13 patients (13.5%). Only one patient showed subpleural honeycombing. 99mTc-DTPA was rapid in all 96 MCTD patients with active ILD (28.7 ± 8.2 min, normal value >40 min). After therapy the 99mTc-DTPA was normalized, 79 out of 96 patients (82.3%). Carbon monoxide diffusion capacity (DLCO) was reduced in 33 out of 96 MCTD patients with active ILD (34.3%), while there were no significant differences in the pulmonary function tests between the active versus inactive stage of ILD or versus patients without ILD. The sera of 96 MCTD patients with active ILD contained a high level of immune complexes (ICs), and the total haemolytic complement levels (CH50/ml U) decreased. After 6 months of therapy, the IC levels decreased and CH50/ml levels normalized (MCTD patients before and after active ILD: IC optical density=355 ± 227 vs 206 ± 92, P<0.001; CH50/ml, 38.0 ± 12.6 U vs 64.3 ± 13.0 U, P<0.001).

Conclusions. HRCT is the gold standard for diagnosis of ILD. However, we used another method, 99mTc-DTPA, in order to compare this technique with HRCT. This latter technique has not been studied previously in MCTD. The elevated levels of IC and increased complement consumption indicated that IC-mediated alveolocapillary membrane damage and tissue injury might play a role in the pathogenesis of ILD in MCTD.

Keywords: Mixed connective tissue disease; Interstitial lung disease; High resolution computed tomography; Lung scintigraphy.
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