RESUMO
To assess the presence of persistent serological activity and its association with clinical outcomes in primary Sjögren's syndrome. Clinical charts of 275 patients were reviewed retrospectively. Persistent serological activity was defined as an increase IgG ≥ 1.6 mg/dL or globulins > 3.7 g/dL or diminished C3 < 52 mg/dL or C4 < 12 mg/dL at least during two consecutive visits during a year (index period). The ClinESSDAI at the index period and the cumulative ClinESSDAI and the SSDDI at the last medical appointment were scored. A total of 159 patients with complete serological data were included mostly women and median disease duration of 10.2 years. Persistent serological activity was identified in 85 patients (53.1%). Only 13 patients changed their status to serological inactivity though the follow-up. Comparison of patients with (n = 85) versus without persistent serological activity (n = 74) showed that the first group had a higher frequency of impaired non-stimulated salivary flow, anti-La/SSB antibody, and RF, as well as higher ClinESSDAI scores. The most affected domains were the constitutional, glandular, cutaneous, renal, and hematological domains. On logistic regression analysis, the RF (OR 6.4, 95% CI 1.8-22, p = 0.003), the renal (OR 12.8, 95% CI 1.7-92, p = 0.01), and the hematological involvement (OR 4.7, 95% CI 1.6-13.4, p = 0.004) remained associated. Half of the patients studied had persistent serological activity, being this status constant through the follow-up. Persistent serological activity was associated with positive RF and higher ESSDAI scores due to hematological and renal activity. Scoring serological activity is an important issue in SS patients.
Assuntos
Anticorpos Antinucleares/sangue , Índice de Gravidade de Doença , Síndrome de Sjogren/diagnóstico , Adulto , Idoso , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Sjogren/sangueRESUMO
AIM: Increased serum viscosity is recognized in primary Sjögren's syndrome (pSS); however, a classic hyperviscosity syndrome (HVS) is rare. We compared the clinical and serological profile among three groups of pSS patients: (i) with HVS; (ii) with high serum viscosity (≥ 1.9 cP [centipoises]) but without HVS; and (iii) with normal viscosity (< 1.9 cP). METHODS: We identified four pSS patients with HVS and retrospectively assessed their clinical/serological features. We included as controls 62 pSS patients and registered their clinical features. We also measured the serum viscosity, C3, C4, immunoglobulins and evaluated the European League Against Rheumatism SS Disease Activity Index (ESSDAI) score at the last visit. We used χ2 , Mann-Whitney U-tests and logistic regression analysis. RESULTS: Patients were predominantly female (95%), mean age 54 ± 14.2 years, median disease duration 9 years. All the HVS cases were diagnosed concomitantly with the onset of SS and had higher titers of immunoglobulin G (IgG), IgM, IgA and a higher prevalence of vasculitis, neutropenia, lymphopenia and splenomegaly. At the multivariate analysis, the variables vasculitis odds ratio (OR) 14.8 (95% CI 1.3-99, P = 0.02) and splenomegaly OR 25.3 (95% CI 1.68-380, P = 0.01) remained associated with HSV. Viscosity levels correlated with rheumatoid factor titers. Thirty (48.3%) patients had high viscosity but without HSV; this group had higher median ESSDAI scores and more vasculitis than patients with normal viscosity. CONCLUSION: High viscosity was present in almost half of the patients and was associated with vasculitis and higher activity scores. Conversely, HVS was infrequent and was associated with vasculitis and splenomegaly. It seems that both conditions have different physiopathological, clinical and treatment implications.