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FRI0134 Shrinking lung syndrome as a clinical manifestation of patients with sle: 5 cases report
  1. J Gratacós,
  2. E Naval,
  3. M Larrosa,
  4. CH Domingo,
  5. M Bosque,
  6. C Tolosa,
  7. E Casado,
  8. A Marín
  1. Rheumatology Unit, University Hospital C.S.P.T., Sabadell, Spain

Abstract

Objectives To describe the clinical manifestations and pulmonary function tests (PFTs) of 5 patients with SLE and shrinking lung syndrome (SLS).

Methods 5 patients (3F/2M) with an age range between 11–76 years. Rewiew of clinical manifestations; laboratory testing (hemogram, antinuclear antibodies, rheumatoid factor-RF- and muscular enzimes); PFTs; X-rays; thoracic CT scan; treatment and follow-up.

Results SLR was detected 2 months to 20 years after the diagnosis of SLE. All cases were ANA and AntiDNA positives and quiescent disease. Only one patient had visceral manifestations (nephropathy). The SLEDAI score ranged between 6 and 13. Poliarthritis was present in two cases (erosive in one) with RF negative. Dispnea was the manifestation which led lo SLS diagnosis with no clinical or laboratory evidence of peripheral miopathy. PTFs showed: FVC 1954 ± 563 ml (65.4 ± 15.4%), FEV1 1562 ± 384 ml (69.8 ± 15%), FEV1/FVC 81.4 ± 7.2%, TLC 3400 ± 1032 ml (68 ± 10%), RV 1148 ± 626 ml (59 ± 17.6%), DLCO 13.2 ± 3.4 ml/min/mmHg (60.6 ± 17.9%), KCO 4.4 ± 2.2 (86.2+/_15.9%), MIP 59.5 ± 17.5 cmH2O (77 ± 21%), MEP 88.7 ± 25.4 cmH2O (97.7 ± 16%), PaO2 86.5 ± 4.3 mmHg, PaCO2 26.4 ± 14.7 mmHg, Aa gradient 17 ± 8. Radiological studies showed reduced lung size with no parenchimal disease. MIP was impaired in two patients at 3 and 24 months from SLS diagnosis. All patients were treated with corticosteroids (4–20 mg/day). Theophilin (10 mg/kg/day) was added to one patient. No effective responsiveness was detected on the follow-up (2 to 24 months).

Conclusion All patients with SLS met 4 or more revised ARA criteria of SLE. Dispnea was the main clinical manifestation at SLS onset. Characteristically, SLS presented with restrictive PFTs without parenchimatous disturbances. Treatment response was ineffective and functional and clinical stabilitation was the rule on follow-up.

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