RESUMEN

Objectives: This study aims to evaluate the prevalence of thrombocytopenia in a cohort of patients with primary and secondary antiphospholipid syndrome (APS) and to examine the relation of thrombocytopenia to the clinical, laboratory findings, and damage index for antiphospholipid syndrome (DIAPS). Patients and methods: Between August 2018 and February 2019, a total of 168 patients (16 males, 152 females; mean age: 32.5±8.4 years; range, 18 to 59 years) who were followed in our clinic for APS were retrospectively analyzed. Medical records of the patients were screened and clinical data, laboratory investigations, and treatments applied were recorded. The DIAPS was calculated for all patients. The patients were divided into two groups according to the presence or absence of thrombocytopenia and both groups were compared regarding clinical, laboratory findings and DIAPS. Further subgroup analysis was done for patients with primary APS. Results: The most common clinical manifestations in our patients were obstetric manifestations (77.4% in pregnant women), musculoskeletal manifestations (69%) and peripheral vascular thrombosis (54.8%). The prevalence of thrombocytopenia in our study was 42.3%, and it was significantly associated with musculoskeletal manifestations (p=0.043), vascular thrombosis (p=0.043), neurological manifestations (p=0.030), cutaneous manifestations (p=0.006), and use of immunosuppressives (p=0.047). The DIAPS was significantly higher in the thrombocytopenia group (p=0.034). Further subgroup analysis of patients with primary APS revealed that neurological manifestations (p=0.010) were significantly higher in the thrombocytopenia group, while the DIAPS was higher in the thrombocytopenia group, but it did not reach statistical significance (p=0.082). Conclusion: Thrombocytopenia may be associated with a higher incidence of vascular thrombosis, neurological manifestations, musculoskeletal manifestations, use of immunosuppressive treatment, and DIAPS. In primary APS patients, thrombocytopenia may be a risk for neurological manifestations.

RESUMEN

BACKGROUND: Avascular necrosis is a common complication in patients with SLE. OBJECTIVE: This study aimed to investigate the risk factors for the occurrence of avascular necrosis among SLE patients receiving steroid therapy at various doses in combination with immunosuppressants. METHODS: In this retrospective study, the medical records of all SLE patients under follow-up at the outpatient clinics of Cairo and Kafr Elsheikh University hospitals through the period from November 2014 to August 2019 were included. Avascular necrosis was diagnosed by the findings of different imaging modalities. RESULTS: We retrieved the medical records of 770 SLE patients during the study period; of them, 55 patients (7.1%) had avascular necrosis. There was significant higher usage of cyclophosphamide (p = 0.003), total cumulative dose of steroids 15-35g plus immunosuppressants (p < 0.001), and steroids >35g plus immunosuppressants (p = 0.016) in the avascular necrosis cohort. Based on the univariate analysis, disease duration of more than five years and cumulative use of steroids were statistically significant predictors for the evolvement of avascular necrosis. Multivariate logistic regression analysis revealed that a disease duration of more than five years was associated independently with avascular necrosis. CONCLUSION: Our data seem to show a role of the association of immunosuppressants plus steroids in the risk of developing avascular necrosis.

Asunto(s)

Lupus Eritematoso Sistémico , Osteonecrosis , Egipto/epidemiología , Humanos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Osteonecrosis/inducido químicamente , Osteonecrosis/epidemiología , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Esteroides/uso terapéutico

RESUMEN

Background: Systemic Juvenile Idiopathic Arthritis (sJIA) is a unique category of juvenile arthritis in which interleukin 6 plays a major pathogenic role. This study aimed to describe the therapeutic short-term outcomes among patients with sJIA starting tocilizumab (TCZ) therapy and to identify possible predictors of treatment response. Methods: We conducted a prospective observational study including 65 patients with sJIA meeting ILAR classification criteria with active disease despite conventional therapy that were treated by TCZ between August 2019 and October 2020 as the first-line biological therapy. Clinical and serological parameters were recorded at baseline and after 1 year of TCZ therapy. Results: After 1 year, 25% of the patients achieved minimal disease activity and 35% achieved clinically inactive disease. A significant reduction of the 10-joint juvenile arthritis disease activity score and acute phase reactants was also observed. Patients with younger age (≤7 years), shorter disease duration (≤3 years), lower disease activity, and higher serum ferritin and systemic manifestations showed more favorable results. Conclusion: Patients with sJIA showed favorable disease outcomes with TCZ treatment for 1 year, especially if the drugs were administered earlier in the disease course and in younger patients with a more pronounced inflammatory status. Our results may help to define the profile of patients with sJIA who are more likely to benefit from IL-6 blockade.