Cardiovascular magnetic resonance detects silent heart disease missed by echocardiography in systemic lupus erythematosus.

Background Accurate diagnosis of cardiovascular involvement in systemic lupus erythematosus (SLE) remains challenging, due to limitations of echocardiography. We hypothesized that cardiovascular magnetic resonance can detect cardiac lesions missed by echocardiography in SLE patients with atypical symptoms. Aim To use cardiovascular magnetic resonance in SLE patients with atypical symptoms and investigate the possibility of silent heart disease, missed by echocardiography. Patients/methods From 2005 to 2015, 80 SLE patients with atypical cardiac symptoms/signs (fatigue, mild shortness of breath, early repolarization and sinus tachycardia) aged 37 ± 6 years (72 women/8 men), with normal echocardiography, were evaluated using a 1.5 T system. Left and right ventricular ejection fractions, T2 ratio (oedema imaging) and late gadolinium enhancement (fibrosis imaging) were assessed. Acute and chronic lesions were defined as late gadolinium enhancement-positive plus T2>2 and T2<2, respectively. Lesions were characterized according to late gadolinium enhancement patterns as: diffuse subendocardial, subepicardial and subendocardial/transmural, due to vasculitis, myocarditis and myocardial infarction, respectively. Results Abnormal cardiovascular magnetic resonance findings were identified in 22/80 (27.5%) of SLE patients with normal echocardiography, including 4/22 with recent silent myocarditis, 5/22 with past myocarditis (subepicardial scar in inferolateral wall), 9/22 with past myocardial infarction (six inferior and three anterior subendocardial infarction) and 4/22 with diffuse subendocardial fibrosis due to vasculitis. No correlation between cardiovascular magnetic resonance findings and inflammatory indices was identified. Conclusions Cardiovascular magnetic resonance in SLE patients with atypical cardiac symptoms/signs and normal echocardiography can assess occult cardiac lesions including myocarditis, myocardial infarction and vasculitis that may influence both rheumatic and cardiac treatment.

Determinants of pulmonary function improvement in patients with scleroderma and interstitial lung disease.

BACKGROUND: In patients with scleroderma-related interstitial lung disease (ILD), improvements of pulmonary function have been reported after treatment with cyclophosphamide (CYC) alone or CYC and high-dose steroids. The study objective was to identify therapeutic regimen that alone or in combination with laboratory or clinical characteristics were associated with pulmonary function improvement in these patients. METHODS: Scleroderma patients with ILD and serial pulmonary function measurements were retrospectively analyzed. We recorded forced vital capacity (FVC, % predicted), diffusion capacity (DLCO, % predicted), type of therapy, and various clinical and laboratory parameters. Treatment with IV CYC was recorded as cumulative dose (grams) and treatment with steroids as high or low dose; outcome was defined as a sustained increase in FVC (% predicted) >or= 10 points. RESULTS: Of the 59 patients who were included in the study, 29 (49 %) patients received IV CYC (cumulative dose 13.9 +/-6.2, range 5.2-26.2 gr) for 3.3 +/- 2.4 years (range 5-60 months). Eighteen out of 59 (30 %) patients received high-dose prednisolone and 41 (70 %) received low-dose prednisolone. In an ordinal logistic model, patients receiving > 12 gr of CYC were 6 times more likely to improve FVC than to decrease or maintain FVC, compared to those who did not receive CYC (p = 0.02). In multivariate analysis, the effect of high dosage CYC on FVC persisted (OR 10.82, p = 0.02). Steroid dosage (high or low) was not associated with FVC improvement (p < 0.05). CONCLUSION: In patients with scleroderma and ILD, treatment with CYC is the only variable that is independently associated with pulmonary function improvement and that prolonged (> 1 year) CYC therapy increases the probability of pulmonary function improvement more than shorter CYC courses.