Koray Tascilar

Increased enthesopathy among Behçet's syndrome patients with acne and arthritis: An ultrasonography study

OBJECTIVE Behçets syndrome is characterized by clusters of disease expression, one of which is the coexistence of acne and arthritis. The aim of this study was to test the hypothesis that enthesopathy is increased in this cluster, having features reminiscent of acne-associated arthritis. METHODS The study group comprised 35 patients with Behçets syndrome who had acne and arthritis (BS-AA), 38 patients with Behçets syndrome who did not have arthritis (BS-WA), 37 patients with ankylosing spondylitis (AS), 25 patients with rheumatoid arthritis (RA), and 25 healthy control subjects. Five entheseal sites (the superior and inferior poles of the patella, the tibial tuberosity, and the superior and inferior poles of the calcaneus) on both lower extremities were examined by 2 independent observers, using ultrasonography. A previously validated composite score was calculated for each patient. The numbers of entheseal sites giving a positive power Doppler signal in each group were also compared. RESULTS Patients with AS had the highest enthesopathy score (mean +/- SD 4.1 +/- 2.4; F [4df] = 8.43, P < 0.001) followed by BS-AA patients (3.0 +/- 1.9; F [3df] = 3.63, P = 0.015). The mean +/- SD enthesopathy scores among the remaining 3 groups were similar (for BS-WA, 1.8 +/- 1.4; for RA, 2.2 +/- 1.6; for healthy controls, 2.0 +/- 1.5 [F (2df) = 0.65, P = 0.53]). Power Doppler scores were highest for the BS-AA group (mean +/- SD 3.0 +/- 1.5; F [4df] = 15.54, P < 0.001) followed by the AS group (2.7 +/- 1.8; F [3df] = 14.38, P < 0.001), the RA group (2.6 +/- 1.8; F [2df] = 13.88, P < 0.001), the BS-WA group (1.2 +/- 1.3; F [1df] = 4.48, P = 0.038), and the control group (0.5 +/- 1.1). There was 87% agreement between the 2 observers (kappa = 0.55, intraclass correlation coefficient 0.71). CONCLUSION The increased presence of enthesopathy among BS-AA patients compared with that among BS-WA patients further supports the hypothesis that patients with Behçets syndrome who also have arthritis and acne form a distinct cluster.

Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course

OBJECTIVE Some features of Behçets syndrome (BS) tend to go together. We aimed to explore the association and timing of various vascular events in both the venous and the arterial vascular tree. METHODS We conducted a chart survey on the type and time of vascular involvement of BS. The cross-relationships of involvement were assessed by phi correlation coefficients. Multiple correspondence analysis was used to identify patterns of vascular involvement. The risk of vascular recurrence was also estimated. RESULTS We identified 882 patients with vascular involvement among 5970 BS patients (14.7%). Deep vein thrombosis (DVT), almost always in the legs, was the most frequent single vascular event (592/882; 67.1%). The cumulative risk of a recurrent vascular event was 38.4% at 5 years. Patients with extrapulmonary artery involvement (EPAI) were significantly older than those with venous and pulmonary artery involvement (PAI). There were significant correlations between dural sinus thrombosis (DST) and PAI, Budd-Chiari syndrome (BCS) and inferior vena cava syndrome (IVCS) and between IVCS and superior vena cava syndrome (SVCS). Multiple correspondence analysis further indicated clustering of PAI, DST, BCS, IVCS and SVCS. However, EPAI and DVT clustered separately from forms of vascular disease, the separate clustering of the DVT being attributed to its propensity to occur solo. CONCLUSION The most common type of vascular involvement in BS is solo DVT, almost always occurring in the legs. Various forms of venous disease in BS segregate together and PAI is included in this group. EPAI segregates separately.

The Unique Features of Vasculitis in Behçet’s Syndrome

The presence of a true vasculitis is difficult to discern in some of the more common manifestations of Behçet’s syndrome, like the papulopustular lesions of the skin. On the other hand, a true vasculitis is seen in all vessel sizes in the majority of the patients. The pathogenesis is not yet known. A Th1-type inflammatory reaction is seen like in some other primary vasculitides. However, in contrast to other vasculitides, granuloma formation is absent. Behçet’s syndrome is unique among the vasculitides with its differing geographic distribution in disease expression, the distinctly more severe disease among the male, the predominance of venous disease, and the generally abating disease course with lack of associated increased atherosclerosis.

Quantiferon-TB Gold in tube assay for the screening of tuberculosis before and during treatment with tumor necrosis factor alpha antagonists

IntroductionThe usefulness of interferon-gamma (IFN-γ) release assays for tuberculosis screening before tumor necrosis factor-alpha (TNF-α) antagonists and for monitoring during treatment is a contraversial issue. The aims of this study were to determine whether TNF-α antagonists affect the results of the Quantiferon-TB Gold in-tube assay (QTF); to assess how QTF performs in comparison with the tuberculin skin test (TST) in rheumatoid arthritis (RA) patients who are about to start treatment with TNF-α antagonists, RA patients who are not candidates for treatment with TNF-α antagonists, rheumatology patients with confirmed current or past tuberculosis infection, and healthy controls, and to determine the specificity of the QTF test to differentiate leprosy patients, another group of patients infected with mycobacteria.MethodsThe 38 RA patients who were prescribed TNF-α antagonists, 40 RA patients who were not considered for TNF-α antagonist use, 30 rheumatology patients with a history or new diagnosis of tuberculosis, 23 leprosy patients, and 41 healthy controls were studied. QTF and TST were done on the same day, and both were repeated after a mean of 3.6 ± 0.2 months in patients who used TNF-α antagonists.ResultsTreatment with TNF-α antagonists did not cause a significant change in the QTF or TST positivity rate (34% versus 42%; P = 0.64; and 24% versus 37%; P = 0.22). Patients with leprosy had a trend for a higher mean IFN-γ level (7.3 ± 8.0) and QTF positivity (61%) than did the other groups; however, the difference was not significant (P = 0.09 and P = 0.43).ConclusionsTreatment with TNF-α antagonists does not seem to affect the QTF test to an appreciable degree. The higher IFN-γ levels in leprosy patients deserves further attention.

Prognosis of Behçet's syndrome among men with mucocutaneous involvement at disease onset: long-term outcome of patients enrolled in a controlled trial

OBJECTIVE To assess the influence of being free of major organ involvement during the early years of the disease on the prognosis of men with Behçets syndrome (BS). METHODS Ninety-six men with BS, who had only active mucocutaneous manifestations when entering a controlled trial of thalidomide mean (s.d.) 11.7 (0.8) years ago, were re-evaluated for the use of immunosuppressives as an indication of major organ involvement during the post-trial period. RESULTS Outcome information was obtained in 91 (95%) patients. Thirty-nine (43%) patients had to use immunosuppressives during the post-trial period. Immunosuppressive use was significantly more frequent among patients developing BS at younger age (76%; <or=24 years) than older age (30%; >or=25 years). Developing BS at young age (OR = 6.3; 95% CI 2.09, 19.04) and not using colchicine during the post-trial period (OR = 3.860; 95% CI 1.484, 10.034) were risk factors for immunosuppressive use. However, 82% of the patients using colchicine had onset during old age. Colchicine showed a significant effect in decreasing the use of immunosuppressives only among patients of old age at onset (Fishers exact test = 5.026; P = 0.031) in the subgroup analysis. Eye disease (18 patients) and vascular involvement (14 patients) were the most frequent indications for immunosuppressive use. CONCLUSIONS Being free of major organ involvement during the early years of BS does not indicate a mild prognosis for men developing BS at young age. Whether colchicine will reduce the need for immunosuppressive use among men developing BS at old age awaits formal studies.

Statins and Risk of Rheumatoid Arthritis ‐ A Nested Case‐Control Study

Statins have antiinflammatory/immunomodulatory effects that may be useful in preventing rheumatoid arthritis (RA), but previous observational studies about the risk of RA with statin use yielded conflicting results. The aim of this study was to determine whether high‐intensity statin treatment is associated with reduced risk of RA.

Ultrasonographic Maturation of Native Arteriovenous Fistulae: A Follow-Up Study

Background/aims. Ideal time needed for arteriovenous fistula (AVF) maturation is still controversial. In this study, we aimed to investigate the natural course of AVF maturation and also investigated the factors affecting AVF maturation. Methods. We studied 31 (21M/10F, mean age 55.8 ± 16.2) chronic renal failure patients. We evaluated the patients with color Doppler ultrasound examination before the fistula operation, at the first day, and at the first, second, third, and sixth months. Radial artery (RA) diameter, flow velocity, flow, resistance index, fistula vein diameter, flow velocity, and flow were measured. Results. Patency rates at the first post-operative day and the sixth month were 87.1% and 67.1%, respectively. Cephalic vein flow was 451.2 ± 248.6 mL/min at the first month and 528.6 ± 316.5 mL/min at the sixth month. Baseline RA diameter was lower in failing fistulas than that of patent fistulas. Failing fistulas were more common in women. Conclusion. Blood flow was enough for hemodialysis at the end of the first month. However, fistula maturation had continued until the end of the study; women and patients with low RA diameter are particularly prone to fistula failure. Therefore, especially in these patients, AVF must be created at least three or four months before the predicted hemodialysis initiation time.

No appreciable decrease in fertility in Behçet’s syndrome

OBJECTIVES Behçets syndrome (BS) follows an active course during the childbearing years in both men and women. We formally surveyed the infertility rate and the effect of drugs and types of organ involvement on fertility in BS. METHODS We compared fertility among BS patients with and without major organ involvement with those with FMF, AS and healthy controls. A structured interview was performed and the medical records of the patients were reviewed to confirm the sites of involvement and drugs they used during their entire follow-up. RESULTS The number of female patients who were not able to ever conceive, who were not able to conceive before or after disease onset or who were able to conceive late or only with assisted reproductive technology was not increased among the BS group. The same was true for the male patients to successfully achieve a conception and/or father a child. The average number of children, miscarriages, terminations and ectopic pregnancies were similar among the groups. Infertility was more common in BS patients with major organ involvement who used cyclophosphamide (CYC) compared with those who did not (P = 0.009). CONCLUSION Infertility is not appreciably increased among BS patients attending a dedicated outpatient clinic. Major organ involvement does not increase the risk of infertility and CYC is the only drug that seems to compromise fertility in BS.

Colchicine in Behçet syndrome: a longterm survey of patients in a controlled trial.

Objective. To test the hypothesis that colchicine use during early disease decreases immunosuppressive use in Behçet syndrome (BS) in the long term. Methods. Patients with BS who participated in a double-blind, placebo-controlled trial of colchicine 16.6 ± 1.1 years ago were evaluated for immunosuppressive use during the posttrial period. Results. We could contact 90/116 patients; 28 (31%) received immunosuppressives during the posttrial period, 14 being from the colchicine arm. Posttrial colchicine use and cumulative duration were similar between patients who received immunosuppressives and those who did not. Conclusion. Continuous use of colchicine, even when initiated at an early disease stage, does not seem to decrease the use of immunosuppressives in the long term.

Long-term follow-up of secondary amyloidosis patients treated with tumor necrosis factor inhibitor therapy: A STROBE-compliant observational study

Abstract There are no treatment modalities, which were proven to prevent the deposition of amyloid, proteinuria, and loss of renal function due to amyloidosis. Anti-tumor necrosis factor agents (anti-TNFs) were shown to decrease the production of serum amyloid A protein. We aimed to evaluate the long-term efficacy and safety of anti-TNFs in secondary (AA) amyloidosis patients treated in a single center. Thirty-seven patients with AA amyloidosis were started an anti-TNF for AA amyloidosis between March 2001 and June 2008 and followed until May 2016 unless deceased. They were surveyed for the endpoints of death, development of end-stage renal disease (ESRD), switch to another agent due to worsening of amyloidosis and adverse events. Among the 37 patients, 12 (32%) had died, 9 (24%) had ESRD, and 8 (22%) had started another group of biologic due to worsening of amyloidosis indicated by an increase in proteinuria, 5 (14%) patients are still doing well with anti-TNFs, and 3 (8%) are off treatment at the end of a median follow-up of 10 (interquartile range [IQR]: 5.5–10.5) years since the start of anti-TNFs and 10 (IQR: 8–13) years since the diagnosis of AA amyloidosis. Most common serious adverse events were sepsis and thrombotic events observed in 8 and 4 patients, respectively. Treatment with anti-TNFs may be associated with a higher survival rate compared with historic cohorts of AA amyloidosis, especially when started early with a lower serum creatinine level at baseline. Caution is needed regarding serious adverse events, especially infections.