Etiopathogenic role of surfactant protein d in the clinical and immunological expression of primary Sjögren syndrome.

OBJECTIVE: To analyze the etiopathogenic role of genetic polymorphisms and serum levels of surfactant protein-D (SP-D) in primary Sjögren syndrome (pSS). METHODS: We analyzed 210 consecutive patients with pSS. SFTPD genotyping (M11T polymorphism rs721917) was analyzed by sequence-based typing and serum SP-D by ELISA. RESULTS: Thirty-two patients (15%) had the Thr11/Thr11 genotype, 80 (38%) the Met11/Met11 genotype, and 96 (46%) the Met11/Thr11 genotype; 2 patients could not be genotyped. Patients carrying the Thr11/Thr11 genotype had a higher prevalence of renal involvement (13% vs 1% and 4% in comparison with patients carrying the other genotypes, p = 0.014). Serum SP-D levels were analyzed in 119 patients (mean 733.94 ± 49.88 ng/ml). No significant association was found between serum SP-D levels and the SP-D genotypes. Higher mean values of serum SP-D were observed in patients with severe scintigraphic involvement (851.10 ± 685.69 vs 636.07 ± 315.93 ng/ml, p = 0.038), interstitial pulmonary disease (1053.60 ± 852.03 vs 700.36 ± 479.33 ng/ml, p = 0.029), renal involvement (1880.64 ± 1842.79 vs 716.42 ± 488.01 ng/ml, p = 0.002), leukopenia (899.83 ± 661.71 vs 673.13 ± 465.88 ng/ml, p = 0.038), positive anti-Ro/SS-A (927.26 ± 731.29 vs 642.75 ± 377.23 ng/ml, p = 0.006), and positive anti-La/SS-B (933.28 ± 689.63 vs 650.41 ± 428.14 ng/ml, p = 0.007), while lower mean values of serum SP-D were observed in patients with bronchiectasis (489.49 vs 788.81 ng/ml, p = 0.019). CONCLUSION: In pSS, high SP-D levels were found in patients with severe glandular involvement, hypergammaglobulinemia, leukopenia, extraglandular manifestations, and positive anti-Ro/La antibodies. The specific association between SP-D levels and pulmonary and renal involvements may have pathophysiological implications.

Factors influencing dry mouth in patients with primary Sjögren syndrome: usefulness of the ESSPRI index.

OBJECTIVE: To evaluate health-related quality of life in a large series of primary SS patients using the recently-proposed ESSPRI questionnaire and to evaluate the relationship between the intensity of oral dryness and other signs and symptoms frequently found in these patients. METHODS: We evaluated 90 primary SS patients seen consecutively; all fulfilled the current classification criteria. All patients completed the ESSPRI questionnaire. We compared the mean values of the ESSPRI-dry mouth item with other ESSPRI items related to sicca features, general symptoms, quality of life, quality of sleep, psychological and psychiatric features, extraglandular involvement, laboratory features and immunological markers and cardiovascular risk factors. Multivariate regression analysis with a backwards stepwise selection method was performed to identify those variables that were independently associated with dry mouth. RESULTS: Mean intensity of oral dryness measured by the corresponding ESSPRI item was 7.17±0.23. Oral dryness correlated with age both at diagnosis and at study inclusion (p=0.013), but not with gender or with time of disease evolution. No significant correlation was found with the SF-36, HAQ and FIQ questionnaires. We found a significant correlation between the intensity of oral dryness and the quality of sleep (p=0.001), anxiety and depression measured by the GH28 (p=0.004 and 0.024, respectively), and a statistically-significant trend for anxiety and depression measured by the HADS (p=0.08 and 0.07, respectively). No significant correlation was found with the main extraglandular and immunological features; however, a significant correlation between oral dryness and hypertension (p=0.019), type II diabetes mellitus (p=0.005) and hypercholesterolemia (p=0.011) was found. Multivariate regression analysis shows that fatigue measured by ESSPRI (p=0.049), sleep quality (p=0.008) and hypercholesterolemia (p=0.008) were independently associated with dry mouth. CONCLUSION: We report on the usefulness of the ESSPRI index in evaluating HRQOL associated with oral dryness in primary SS patients. Oral dryness correlated with age and the other sicca symptoms measured by ESSPRI, but not with the main systemic and immunological SS features. In contrast, oral dryness was strongly correlated with fatigue, pain, psychological distress, poor sleep and vascular risk factors. A multidisciplinary therapeutic approach may be the best way of minimizing oral dryness and its consequences in primary SS patients.