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

E. Bodolay, Z. Szekanecz, K. Dévényi, L. Galuska, I. Csípő, J. Vègh, I. Garai, G. Szegedi

Research output: Contribution to journalArticle

87 Citations (Scopus)

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 2mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2mg/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 ± 8.2min, normal value >40min). 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/mlU) 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, P99mTc-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.

Original languageEnglish
Pages (from-to)656-661
Number of pages6
JournalRheumatology
Volume44
Issue number5
DOIs
Publication statusPublished - May 2005

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Mixed Connective Tissue Disease
Interstitial Lung Diseases
Antigen-Antibody Complex
Tomography
Adrenal Cortex Hormones
Glass
Fibrosis
Respiratory Function Tests
Aerosols
Cyclophosphamide
Thorax
Blood-Air Barrier
Complement Hemolytic Activity Assay
Therapeutics

Keywords

  • High resolution computed tomography
  • Interstitial lung disease
  • Lung scintigraphy
  • Mixed connective tissue disease

ASJC Scopus subject areas

  • Neuroscience(all)
  • Rheumatology

Cite this

Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). / Bodolay, E.; Szekanecz, Z.; Dévényi, K.; Galuska, L.; Csípő, I.; Vègh, J.; Garai, I.; Szegedi, G.

In: Rheumatology, Vol. 44, No. 5, 05.2005, p. 656-661.

Research output: Contribution to journalArticle

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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 2mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2mg/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 ± 8.2min, normal value >40min). 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/mlU) 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, P99mTc-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.",
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T1 - Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD)

AU - Bodolay, E.

AU - Szekanecz, Z.

AU - Dévényi, K.

AU - Galuska, L.

AU - Csípő, I.

AU - Vègh, J.

AU - Garai, I.

AU - Szegedi, G.

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N2 - 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 2mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2mg/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 ± 8.2min, normal value >40min). 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/mlU) 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, P99mTc-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.

AB - 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 2mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2mg/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 ± 8.2min, normal value >40min). 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/mlU) 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, P99mTc-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.

KW - High resolution computed tomography

KW - Interstitial lung disease

KW - Lung scintigraphy

KW - Mixed connective tissue disease

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