Maria Usman Khan

High Vitamin D Levels May Downregulate Inflammation in Patients with Behçet’s Disease

Vitamin D plays a significant role in the immune system modulation and may confer a protective role in autoimmune diseases. We conducted a case-control study to compare 25(OH)D levels in patients with BD who were managed at a regional rheumatology programme in the midwest region of Ireland compared to matched controls. Healthy controls were selected from the Irish health system and matched in 1 : 5 ratio for age, sex, and the month of the year. 25(OH)D levels <20 nmol/L were classified as deficient while levels between 20 and 40 nmol/L were classified as insufficient. Differences between groups were assessed using Mann–Whitney test and associations between cases and controls were expressed as odds ratios and 95% confidence intervals. Nineteen patients with BD were compared with 95 controls matched by age, sex, and month of blood draw. 25(OH)D levels were significantly higher in patients in BD than in matched controls (median values: 45 nmol/L versus 22 nmol/L, p < 0.005) and tended to be lower in patients with active disease than in those without (median values: 35 nmol/L (IQR: 22.75–47.25 nm/L) versus 50 nmol/L (IQR: 35–67 nmol/L), p = 0.11). Compared to controls, patients with BD were significantly less likely to have 25(OH)D deficiency or insufficiency (OR: 0.09, 95% CI: 0.03–0.28, p < 0.001). Our findings suggest a possible role for 25(OH)D in modifying the inflammatory response in BD and uncover a potential opportunity to assess whether correction of Vit D deficiency confers protective benefits.

The real-world use of different anti-tumor necrosis factor agents in a Northern European population of patients with Behçet's disease

Objective The aim of this study was to evaluate prescription practices, treatment responses, and serious adverse events of anti-tumor necrosis factor (anti-TNF) therapies in Behçets disease (BD). Material and Methods Patients with BD satisfying the International Study Group for Behçets Disease or the International Criteria for Behçets Disease criteria were recruited from a regional rheumatology program. The choice of anti-TNF, treatment response, and adverse events were specified. Response to treatment was evaluated by the detection of new, worsening, or improving clinical features, and management was benchmarked against current The European League against Rheumatism recommendations published in 2008. Results Out of the total of 22 patients, 18 (81.9%) received anti-TNF therapies, resulting in 14 (77.8%) complete and 4 (22.2%) partial remissions. Eleven (61.1%) patients switched to a second anti-TNF, seven patients (38.9%) required three different anti-TNFs, and one required a fourth anti-TNF to achieve remission. Two patients required retrials before their disease was controlled. Anti-TNF therapy included infliximab (IFX): n=15, 83.3%; adalimumab (ADA): n=9, 50%; golimumab: n=6, 33.3%; etanercept: n=5, 27.8%; and certolizumab pegol: n=2, 11.1%. Secondary failure was observed with IFX (4/15; 26.7%) and ADA (2/9; 22.2%), and these (100%) were manifested after at least 2 years of treatment. Five patients with potentially life-threatening laryngeal involvement received anti-TNFs successfully halting disease progression. Five allergic reactions were encountered, and five serious infections were documented involving three patients aged ≥ 50 years, all with the use of IFX. Conclusion Anti-TNF therapy induced a clinical response in 100% patients and achieved complete remission in 78% patients. It provides an effective alternative option for first-line therapy in severe BD where many conventional immunosuppressive therapies fail. Patients with BD who do not respond to one or more anti-TNFs because of intolerance, ineffectiveness, or secondary failure might benefit from switching to another drug from this group or even a retrial of a previously administered anti-TNF because unsatisfactory results with one biologic is not predictive of response to another anti-TNF. For those with potentially life-threatening destructive laryngeal manifestation, anti-TNF as a first choice may be considered.

Adhesive arachnoiditis in mixed connective tissue disease: a rare neurological manifestation

The overall incidence of neurological manifestations is relatively low among patients with mixed connective tissue disease (MCTD). We recently encountered a case of autoimmune adhesive arachnoiditis in a young woman with 7 years history of MCTD who presented with severe back pain and myeloradiculopathic symptoms of lower limbs. To the best of our knowledge, adhesive arachnoiditis in an MCTD patient has never been previously reported. We report here this rare case, with the clinical picture and supportive ancillary data, including serology, cerebral spinal fluid analysis, electrophysiological evaluation and spinal neuroimaging, that is, MRI and CT (CT scan) of thoracic and lumbar spine. Her neurological deficit improved after augmenting her immunosuppressant therapy. Our case suggests that adhesive arachnoiditis can contribute to significant neurological deficits in MCTD and therefore requires ongoing surveillance.

03.08 High vitamin d levels may downregulate inflammation in behçet’s disease patients

Background Vitamin D has been shown to be directly or indirectly involved in the regulation of proliferation, differentiation, and function of immune cells. Many studies have revealed higher levels of Vitamin D deficiency among patients with autoimmune diseases compared to controls. Aim Aim of the study was to evaluate the serum 25-hydroxyvitamin D (25(OH)D) levels in Behçet’s disease (BD) patients in the midwest region of Ireland, and to correlate with its disease activity. Methods All BD patients attending our rheumatology service and satisfying the ISGBD/ICBD criteria were included in the study and compared in a 1:5 ratio with samples taken from controls matched for age, gender- and the month of the year. Exclusion criteria included other rheumatological or bone/skeletal diseases, a history of chronic kidney disease or other chronic systemic diseases, malignancies, limited physical activity, and if on vitamin D supplementation or medications that could have affected vitamin D metabolism including calcium supplements, cytotoxic drugs, anticonvulsants, bisphosphonates and thyroxine but not glucocorticoids and disease-modifying anti-rheumatic drugs. The total serum 25(OH)D was measured by using competitive chemiluminescence immunoassays (DiaSorin, Dietzenbach, Germany). Levels<20 ng/ml were defined as deficient, between 20–40 ng/ml as insufficient. Results 19 Caucasian BD patients were included in the study (5 male, 14 female, median age of 37.5 years (interquartile range (IQR), 24.3–51.2 years). The median 25(OH)D of BD patients and controls were 45 ng/ml (IQR,33–65 ng/ml) and 22 ng/ml (IQR, 15–31 ng/ml) respectively. The median 25(OH)D was relatively lower in active BD patients in comparison to inactive patients: 35 ng/ml (IQR, 22.75–47.25 ng/ml) compared to 50 ng/ml (IQR, 35–67 ng/ml). Overall, none of the patients had Vit D deficiency, however 6 patients had Vit D insufficiency. Conclusion In contrast to many previous studies in other BD cohorts and other autoimmune diseases our study suggests the mean 25(OH)D levels was significantly higher in this BD group. In patients with active disease however serum levels were relatively low compared to the inactive group which is in concordance with the literature. Our findings suggest vitamin D may be a potential suppressor of inflammation in BD, however larger studies are needed to support this thesis and to conclusively understand its role in the inflammatory pathway.