Central nervous system vasculitis (CNSV) is an uncommon and poorly understood form of vasculitis. Early recognition is important because medical treatment might improve the outcome. However, randomized clinical trials on CNSV treatment do not exist. Endovascular treatment has been reported in few cases, but no data exist for intracranial stenting. We report two cases of patients with suspected CNSV and recurrent clinical episodes, treated with intracranial stenting. A 48-year-old man had relapsing episodes of right hemiparesis. Neuroradiological exams showed severe left carotid terminus stenosis. Despite immunosuppressive therapy, neuroradiological follow-up exams showed a worsening of the aforementioned stenosis with many transient episodes of weakness in the right limbs and aphasia. A 64-year-old woman had a sudden onset of dysarthria and transient aphasia. Neuroradiological exams showed a severe arterial stenosis involving the origin of left ACA and MCA. Despite dual antiplatel therapy she presented an acute onset of severe aphasia, due to an occlusion of the left carotid terminus and proximal MCA. In both cases endovascular procedure and intracranial stenting was performed, with marked improvement of cerebral blood flow. No more clinical episodes were reported. Intracranial stenting may be a valid therapeutic option in selected patients with CNSV and involvement of medium or large size vessels with clinical worsening despite best medical treatment.
Leflunomide is an antirheumatic drug. One of its main features is its ability to inhibit de novo pyrimidine ribonucleotide biosynthesis. It has been reported as an effective drug in the treatment of patients with Rheumatoid Arthritis. Recently pilot studies have demonstrated the benefit of leflunomide in systemic lupus erythematosus patients. Herein we describe the successful treatment of two lupus patients with leflunomide and review the current literature.
Abstract Background: Immune semaphorins are important players in controlling both innate and adaptive immune responses. The regulatory role of semaphorin3A (sema3A) in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and other autoimmune diseases is widely reported. Decreased levels of serum sema3A were shown to be associated with SLE disease activity. Objectives: To assess urine concentrations of sema3A in SLE patients and its correlation with renal involvement and disease activity. Methods: Urine levels of sema3A were analyzed in 38 SLE patients of whom 13 had renal involvement and were compared to 10 healthy controls and 8 RA patients (disease control group). Results: The secretion of urine sema3A was found to be significantly lower in SLE patients compared to healthy controls and RA patients (4.9±3.9 ng/ml, 8.5±2.7 ng/ml, 9.85±1.7 ng/ml, respectively, p = 0.0006). Urine sema3A was significantly lower in SLE patients with lupus nephritis than in patients without nephritis (4.0±3.4 ng/ml vs 6.5±3.8 ng/ml, p=0.03). Urine sema3A was inversely correlated with proteinuria and SLE disease activity. Conclusion: Urine sema3A is decreased in lupus patients and should be further evaluated as a possible biomarker for disease activity and renal involvement.
Semaphorin 3A (sema3A) plays a regulatory role in immune responses, mainly affecting the activation of regulatory T cells. It has been found to correlate with disease activity in rheumatoid arthritis and systemic lupus erythematosus (SLE).
Aim
To investigate the expression of sema3A in patients with systemic sclerosis (SSc) compared to healthy controls and SLE disease controls and to correlate its expression with clinical characteristics.
Methods
27 SSc patients, 42 SLE patients and 18 healthy controls were enrolled. Serum level of sema3A was measured by ELISA and expression of sema3A on regulatory T cells was evaluated by FACS analysis. SSc patients were evaluated for demographics, clinical manifestations, routine laboratory results, nailfold videocapillaroscopy patterns, pulmonary function tests, echocardiograms, modified Rodnan skin score (mRSS) and disease activity and severity scores.
Results
Serum levels of semaphorin 3A were lower in SSc compared to healthy controls 14.38 ± 5.7 ng/ml vs. 27.14 ± 8.4 ng/ml, p < 0.0001 and similar to SLE 15.7 ± 4.3 ng/ml. The expression of semaphorin 3A on regulatory T cells was also lower in SSc compared to healthy controls 61.7 ± 15.7% vs. 88.7 ± 3. 7% (p < 0.0001). Semaphorin 3A serum level inversely correlated with the duration of disease; r = -0.4, p = 0.036 and with low C4 level r = 0.66 p = 0.026. SCL-70 antibody positivity was associated with a lower semaphorin 3A level in serum (difference in mean of 3.44 p = 0.06).
Conclusion
Sema3A expression is low in SSc serum and more specifically on regulatory T cells. This may help explain the reduced activation of regulatory T cells in SSc, contributes to our understanding of the pathogenesis of the disease and may serve as a future target for treatment.
