Background/Purpose: Evaluate the progression of interstitial lung disease (ILD) in a 10-year period of a group of 53 MCTD patients previously evaluated 10 years ago.

Methods: Pulmonary function tests (PFTs) and chest HRCT images were performed: current results were compared with the patientxs own results obtained ten years ago (baseline value). HRCT images were analyzed qualitatively and quantitatively. Two thoracic radiologists scored images independently using a global quantitative score (ILD-HRCT), that gives an estimate of lung involvement (0% – 100%), as previously described. Decreases in forced vital capacity (FVC) of 10%/200ml and/or in carbon monoxide diffusing capacity (DLCO) of 15% were considered significant. Structured interview, and retrospective collection of clinical data was also performed. Ethics committee approval: 0099/11

Results: From the 53 patients, 7 patients lost follow-up, 3 died, and 4 changed diagnosis. Therefore 39 patients were re-evaluated (table 1): 92% underwent PFTs and 86% chest HRCT

Treatment was given according to the attending physician. All patients received omeprazol. 5% received only chloroquine, 18% received variable doses of corticosteroids and chloroquine, and 77% received corticosteroids, chloroquine and another medication: azathioprine, methotrexate, cyclophosphamide, or leflunomide.

22% of patients had normal HRCT (0% ILD-HRCT score) baseline and follow-up. In the remaining, ground glass opacities and reticulate were the most common image findings. Signs of fibrosis increased in the follow-up images (honeycomb and bronchiolectasis), nevertheless, overall lung involvement was mild and only slightly progressed (note baseline and follow-up ILD-HRCT score, table 2).

From the subgroup of patients who had normal baseline PFTs, half of them worsened FVC and/or DLCO in follow-up (mean decrease: FVC -10.4% and DLCO -27%). Despite functional loss, 33% remained with normal follow-up chest HRCT images (0% ILD-HRCT score) and 22% with stable ILD-HRCT scores.

From the subgroup of patients with a restrictive pattern in baseline (mild reduction in FVC and FEV₁ with normal DLCO): Half of them presented a reduction in FVC and/or DLCO (mean decrease FVC: -17.5% and DLCO: -33%).

Conclusion: In this cohort, ILD associated with MCTD was mild in extent and slowly progressed in 10 years in half of the patients. These results suggest that a systematic lung assessment should be performed in all subjects with MCTD in a regular basis.

Table 1. Actual characteristics of the MCTD patients

Table 2:  Evolution of Chest HRCT findings in MCTD patients in 10 years