A patient-reported outcome measures-based composite index (RAPID3) for the assessment of disease activity in ankylosing spondylitis.

A single questionnaire regarding to disease activity for all rheumatic diseases may present advantages to introduce quantitative measurement into routine care. The aim of this study was to evaluate the correlation of routine assessment of patient index data 3 (RAPID3) with Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Ankylosing Spondylitis Disease Activity Score (ASDAS). A total of 341 consecutive AS patients who met the modified New York classification criteria were included. All patients completed BASDAI and RAPID3 at each visit, and their physicians completed physician global assessment. ASDASs were calculated using defined formulas. Proposed RAPID3 severity categories were compared to BASDAI and ASDAS categories. Spearman's rho correlation test and kappa statistics were used to analyze statistical significance. The median age of AS patients was 34.0 (21.0-69.0) years and the median disease duration 10.0 (2.0-35.0) years. Median scores for RAPID3, BASDAI, ASDAS-CRP, and ASDAS-ESR were 13.0 (0.0-27.3), 4.7 (0.0-9.7), 3.0 (0.4-5.8), and 2.5 (0.5-6.3), respectively. RAPID3 was strongly correlated with BASDAI and ASDAS-ESR (r = 0.842, r = 0.815; p < 0.001, respectively). Among the 209 patients with high disease activity according to BASDAI, 83.3 % had high or moderate severity according to RAPID3 (kappa 0.693; p < 0.001). Among the 133 patients with moderate, high, and very high disease activity on ASDAS-CRP, 91.7 % had high or moderate severity according to RAPID3 (kappa 0.548; p < 0.001). RAPID3 is as informative as BASDAI and ASDAS in our cohort of AS patients. We therefore suggest that RAPID3 may be used to assess the patient status quantitatively in AS patients, as part of routine care.

Impact of rheumatoid arthritis in Turkey: a questionnaire study.

OBJECTIVES: Unmet needs of rheumatoid arthritis (RA) patients regarding physician/patient communication, treatment preferences and quality of life issues were investigated in a Turkish survey study. METHODS: The study was conducted with the contribution of 33 rheumatologists, and included 519 RA patients. The study population included patients who had been on biologic therapy for >6 months and were still receiving biologic therapy (BT group), and those who were biologic naive, but found eligible for biologic treatment (NBT group). Of the RA patients, 35.5% initially had a visit to an internal disease specialist, 25.5% to a physical therapy and rehabilitation specialist, and 12.2% to a rheumatology specialist for their RA complaints. The diagnosis of RA was made by a rheumatologist in 48.2% of patients. RESULTS: The majority of RA patients (86.3%) visit their doctor within 15-week intervals. Most of the physician-patient communication focused on disease symptoms (99.0%) and impact of the disease on quality of life (61.8%). The proportion of RA patients who perceived their health status as good/very good/excellent was higher in the BT group than in the NBT group (74.3% vs. 51.5%, p<0.001). However, of those RA patients in the NBT group, only 24.8% have been recommended to start a biologic treatment by their doctors. With respect to dose frequency options, once-monthly injections were preferred (80%) to a bi-weekly injection schedule (8%). CONCLUSIONS: In conclusion, RA patients receiving biologic therapy reported higher rates of improved symptoms and better quality of life and seemed to be more satisfied with their treatment in our study.

Coping strategies for activities of daily living in women whose hands affected by systemic sclerosis.

AIMS AND OBJECTIVES: To determine the challenges experienced by women with systemic sclerosis, whose hands affected, while performing activities of daily living and their coping strategies. BACKGROUND: Many of the patients with systemic sclerosis experience difficulties in performing daily activities. One of the most important reasons for that is the impaired hand function due to their diseases. DESIGN: A descriptive cross-sectional design was conducted and questionnaire was used in this study. METHODS: The study was performed in a Rheumatology Department at a tertiary-care hospital in Turkey between April 2010-December 2011. Nineteen patients with systemic sclerosis with hand involvement were enrolled in this study. The data were collected by using both a demographic data form and an Evaluation of Daily Activity Questionnaire. RESULTS: According to Evaluation of Daily Activity Questionnaire, the most scored dimension that patients can do with much difficulty was 'eating' and the dimension that patients unable to do was 'washing/clothes care'. In 'eating' dimension, the most difficult activities were 'opening glass jar', 'opening juice bottle' and 'opening bottle' that requiring the movement of rotation. Their coping strategies for these activities were as follows: try to open with a towel, try to remove the edge of the palm with a knife, use the hand palm and help from someone else (spouse, neighbour, etc.). In 'washing/clothes care' dimension, the most difficult activities were 'turning up hem of a skirt', 'washing up in bowl' and 'cutting out material'. For these activities, they use some coping strategies such as getting help from tailor, washing in the machine instead of hand washing. CONCLUSION: This study demonstrates that impaired hand function affects the daily life activities of patients with systemic sclerosis, and patients have developed some coping strategies to overcome these difficulties. RELEVANCE TO CLINICAL PRACTICE: The coping strategies used by patients can be helpful for the other patients with systemic sclerosis.

Relationship of ultrasonographic findings with synovial angiogenesis modulators in different forms of knee arthritides.

Angiogenesis is controlled by a variety of angiogenesis stimulators and inhibitors. The increased power Doppler (PD) signals determined by ultrasonography is an indirect marker of synovial vascularity in arthritis. We aimed to investigate relationship between ultrasonographic findings and synovial angiogenesis modulators. Thirteen Behcet's disease (BD), 15 spondyloarthropathy, 21 rheumatoid arthritis (RA), and 15 osteoarthritis (OA) patients with knee arthritis were included. Cumulative effusion, synovial hypertrophy, and PD signal scores were calculated in arthritic joints. In synovial fluid samples, angiogenesis inhibitors (angiostatin, thrombospondin-1, and endostatin) and stimulators [bFGF (basic fibroblast growth factor), angiopoietin-1] were studied. The comparisons between groups were made by Kruskal-Wallis test, and correlation analysis was calculated with Pearson and Spearman tests. Effusion scores were significantly higher in inflammatory arthritis than in OA. Synovial hypertrophy scores were higher in RA and spondylarthritis than in OA and BD. PD scores were not different between the groups. Synovial angiostatin and bFGF levels were significantly higher in patients with inflammatory arthritis than in OA. Cumulative effusion scores were positively correlated with angiopoietin-1, angiostatin, and bFGF and negatively correlated with thrombospondin-1 levels. Synovial hypertrophy scores were positively correlated with angiostatin and bFGF levels and negatively correlated with thrombospondin-1. No correlation was found between PD scores and modulators of angiogenesis. In large joints like knee, detecting PD signals alone was not sufficient to assess the angiogenesis. However, cumulative activity scores were positively correlated with angiogenesis stimulators. Therefore, when investigating the angiogenesis, PD technique should be added to gray-scale examinations.

The factors considered as trigger for the attacks in patients with familial Mediterranean fever.

Although the inflammatory cascade of familial Mediterranean fever (FMF) is partially understood, triggering factors of those attacks has not been studied well. It is supposed that physical stresses such as cold exposure, tiredness and emotional stresses could provoke attacks. This study is aimed to survey the factors regarded as triggering the attacks in patients with FMF and their relationship with MEFV gene mutations. Clinical findings and genetic mutations (consist of M694V, M694I, M680I, V726A, E148Q) of patients were recorded. Patients were questioned about cold exposure, emotional stress, tiredness, long-lasting standing, long-duration travel, starvation, high intake of food, trauma, and infection as triggering factors for the attacks with both serositis and musculoskeletal pain. The study is comprised of 275 FMF patients (male/female: 177/98). The most common triggering factors for the attacks with serositis were cold exposure (59.3 %), emotional stress (49.8 %), tiredness (40.0 %) and menstruation (33.7 % in females). Long-lasting standing (78.8 %), long-duration travel (64.1 %) and tiredness (47.8 %) were the triggering factors for the attacks with musculoskeletal symptoms. The relationships between MEFV mutations and triggering factors were found as M694V allele with starvation, E148Q allele with high intake of food and V726A allele with long-duration travel. The attacks with serositis seem to be triggered by those factors to which whole body exposed, whereas the attacks with musculoskeletal complaints seem to be triggered by those factors to which regional or local part of body exposed. Since the number of alleles was small, a clear conclusion for a relationship between a particular gene variant and a specific trigger was not made.

Systemic involvements and preferred treatments in a large cohort of Behçet's disease.

The immunosuppressive drugs are widely used in systemic involvements of Behçet's disease. This study is aimed to investigate the extent of clinical involvement and preferred treatment approaches for type of involvements in Behçet's patients from the whole country. All patients with the diagnosis of Behçet's disease were enrolled to the study. These patients analyzed whether they fulfill the International Study Group Criteria, and only those were further evaluated. Demographic and clinical characteristics, laboratory results and treatments ever used were recorded. Further analysis is done regarding clinical manifestations and preferred therapeutic approaches. A total of 863 patients with the diagnosis of Behçet's disease were detected, but 682 of them (female/male: 113/569) found to be appropriate for analysis. The remaining patients were included to the analysis. The frequencies of articular, ophthalmic and vascular involvement were 49, 43 and 21 %, respectively. Colchicine and corticosteroids were the most preferred agents. The immunosuppressive agents frequently used for organ involvements were azathioprine, cyclosporine A, interferon-α, sulphasalazine and cyclophosphamide with decreasing order of frequency. In this relatively young population composed from all over the country, the frequency of ophthalmologic, venous and neurological involvement is less frequent than previous reported cohorts. Azathioprine and cyclosporine were the drugs of choice as a chronic immunosuppressive agent in patients with organ involvement. The previously reported increased frequencies in other cohorts could be a result of the reference of severe patients to dedicated centers.

Value of DWI in visual assessment of activity of sacroiliitis in longstanding ankylosing spondylitis patients.

OBJECTIVE: To test contrast to noise ratios (CNRs) of both diffusion-weighted (DW) images and contrast enhanced images in terms of the visual assessment of activity in sacroiliitis of ankylosing spondylitis (AS) patients. MATERIALS AND METHODS: The study included 21 patients with AS. All patients were examined with STIR, FST1/Gd and DWI (b = 0,600). A total of 54 hyperintense lesions on STIR were noted in their sacroiliac joints divided into four quadrants. CNRs were calculated for all of the sequences above. A second group of patients (n = 7) with normal sacroiliac joints (SIJs) served as controls. A total of 56 CNR measurements from apparently normal subchondral bone marrow in this control group were done as well. The differences between scores were tested for significance (SPSS version 17.0) using Wilcoxon's test in which p values lower than 0.01 were considered statistically significant. RESULTS: In the first group with sacroiliitis, mean CNRs for STIR, FST1/Gd, DWI were 32.97, 30.16 and 24.47, respectively. Mean CNRs in the second group with normal SIJs were calculated as 3.52 , 2.99 and 3.96, respectively . There was a statistically significant difference between the CNR measurements of the first and the second group (p = 0.000). Hyperintense lesions on STIR were depicted as "active" in the first group. Except for four lesions that were not included into the study, all of these hyperintense lesions were enhanced after contrast media administration. All of the "active" lesions were observed on DWI as well, at b = 600. No statistically significant difference between CNRs of contrast enhanced images and DWI and of contrast enhanced images and fluid sensitive sequences were found in the first group with sacroiliitis (p > 0.01). CONCLUSION: The CNRs are highest on STIR, followed by contrast enhanced images and DWIs. In terms of DWI and contrast enhanced images, there is no statistically significant difference between these two. Hence, contrast enhanced imaging can be replaced by DWI for visual analysis of active sacroiliitis, which is easy to apply without adverse affects of contrast media.

The cost of care of rheumatoid arthritis and ankylosing spondylitis patients in tertiary care rheumatology units in Turkey.

OBJECTIVES: To determine the direct and indirect costs due to rheumatoid arthritis (RA) and ankylosing spondylitis (AS) patients in Turkey. METHODS: An expert panel was convened to estimate the direct and indirect costs of care of patients with RA and AS in Turkey. The panel was composed of 22 experts chosen from all national tertiary care rheumatology units (n=53). To calculate direct costs, the medical management of RA and AS patients was estimated using 'cost-of-illness' methodology. To measure indirect costs, the number of days of sick leave, the extent of disability, and the levels of early retirement and early death were also evaluated. Lost productivity costs were calculated using the 'human capital approach', based on the minimum wage. RESULTS: The total annual direct costs were 2,917.03 Euros per RA patient and 3,565.9 Euros for each AS patient. The direct costs were thus substantial, but the indirect costs were much higher because of extensive morbidity and mortality rates. The total annual indirect costs were 7,058.99 Euros per RA patient and 6,989.81 for each AS patient. Thus, the total cost for each RA patient was 9,976.01 Euros and that for an AS patient 10,555.72 Euros, in Turkey. CONCLUSIONS: From the societal perspective, both RA and AS have become burden in Turkey. The cost of lost productivity is higher than the medical cost. Another important conclusion is that indirect costs constitute 70% and 66% of total costs in patients with RA and AS, respectively.

Onycho-pachydermatit with extensive bone marrow edema predominant in the metacarpals: a "forme fruste" of POPP?

Psoriatic onycho-pachydermo-osteo/periostitis (POPP) syndrome is a rare form of psoriatic arthritis with a combination of (i) psoriatic onychodystrophy, (ii) connective tissue thickening, and (iii) periostitis of the distal phalanges. The treatment of the condition has generally been reported to be unsatisfactory with the traditional regimes. Here, we describe a case whom we believe is one presentation of POPP with extensive bone marrow edema of metacarpal bones without distinctive periostitis.

Investigation of the veins in patients with Behçet's disease with no known vascular event by Doppler ultrasonography.

In Behçet's disease, deep venous thrombosis occurs primarily in the lower extremities. Total recanalization rate is low, so thrombotic segment could be detected by imaging afterward. For disclosing vein involvement, leg swelling is commonly queried in the history taking in those patients. However, there are no data about the presence of "silent" thrombosis in patients with BD. We aimed at investigating the integrity of venous vessels in BD, without any known vascular event by using Doppler ultrasonography (DU). Patients having past events revealed in the vascular questionnaire or physical findings attributable to vascular disease were excluded. Various degree of venous insufficiency was detected in 74 patients in BD (74%), 24 out of 33 patients (72%) in AS and in 8 out of 34 (25%) in HC group. All were at the lower extremities, and there is no difference in the frequency between BD and AS, while both were significantly higher than in HC (P = 0.001, and 0.004, respectively). Six patients with BD (6%) have chronic venous thrombi at the lower extremities and none in either AS and HC. As a non-invasive method, DU of lower extremities may disclose "silent" thrombosis. Venous insufficiency in those patients should be considered cautiously as an indicator of vein involvement.

Living with scleroderma: patients' perspectives, a phenomenological study.

In this study, it is aimed to determine the daily life experiences of patients on the basis their own way of statement. Sixteen patients with scleroderma were enrolled to this qualitative study. Data were collected using both a demographic data form and a semi-structured interview form. Study was made on individual patient interview by face-to-face manner. Data were evaluated using Colaizzi's phenomenological data analysis method. Data analysis revealed four categories and nine topics. These categories were (1) physical impact of disease, (2) emotional impact of disease, (3) social impact of disease and (4) patient behaviours for the cope with the disease. As emotional impact, patients imply that they have experienced disappointment, hope to get well and have fears about the future. In the physical impact category, tight skin, limitations of hand skills, swelling of the hands and feet, fatigue, swallowing difficulties and deformation of their bodies were the prominent features. In patients with recognizable disease, difficulty to join to social activities increases and eventually leads to isolation. There was also some evidence that patients who have been supported by their family and close relatives seem to be more optimistic about their disease. Most patients are not willing to communicate with other patients, particularly in an advanced stage. Scleroderma patients imply that they experience several difficulties regarding emotional, physical and social aspects. Individual abilities to cope with the disease were much more improved among patients who have a sustained social support. For advanced patients with apparent deformations, an effective social support system should be introduced.

Varicocele and epididymitis in Behcet disease.

OBJECTIVE: Behçet disease is a systemic disease with protean manifestations. Vasculitis is a hallmark of the disease and may involve arteries, veins, and capillaries. Varicocele is dilatation of the pampiniform plexus veins. We aimed to investigate the incidence of varicocele in patients with Behçet disease. METHODS: The study included 47 male patients with Behçet disease and 31 healthy control participants. All underwent a clinical evaluation including a medical history and systemic and scrotal examinations. Subsequently, 2 investigators blinded to the clinical data performed sonographic examinations and measured pampiniform plexus vein diameters. RESULTS: The mean age of the patients ± SD was 23.4 ± 3.2 years; disease durations ranged from 3 to 120 months (46 ± 31 months). Scrotal pain or a palpable mass was detected by clinical examination in 24 patients with Behçet disease (51.1%) and 5 healthy participants (16.1%; P = .002). By color Doppler examination, left varicocele was diagnosed in 26 patients with Behçet disease (55.3%) and 9 healthy participants (29%; P = .02). All patients with right varicocele also had left varicocele; that condition was detected in 10.6% (5 patients) of the Behçet disease group and 6.4% (2 patients) of the control group (P > .05). Eight patients with Behçet disease (17%) had epididymitis, whereas none of the healthy participants did (P = .019, Fisher exact test). Genital ulcers and erythema nodosum lesions were more common among patients with varicocele (P = .034 and .058, respectively). There were no differences in smoking, epididymitis, arthritis, uveitis, or other clinical parameters for distinguishing varicocele in patients with Behçet disease. CONCLUSIONS: The incidence of varicocele was increased in Behçet disease. Whether varicocele confers fertility problems in patients with Behçet disease and the underlying mechanism for a possible association are yet to be determined.

No regression of renal amyloid mass despite remission of nephrotic syndrome in a patient with TRAPS following etanercept therapy.

Reactive systemic (AA) amyloidosis leading to renal failure is the most severe complication of tumor necrosis factor receptor-associated periodic syndrome (TRAPS). There is now growing evidence to suggest that anti-tumor necrosis factor (anti-TNF) agents may be an attractive treatment option for amyloidosis not only in TRAPS but in several forms of secondary amyloidosis complicating inflammatory rheumatic diseases. In most of the reported cases, anti-TNF agents were deemed successful on the basis of regression of proteinuria and either improvement or stabilization of creatinine clearance, while objective proof of renal amyloid regression either by serum amyloid P scintigraphy or biopsy is limited. We herein report a case of TRAPS complicated with nephrotic syndrome due to AA amyloidosis in which treatment with etanercept was associated with remission of the nephrotic syndrome but no regression of amyloid mass on the follow-up renal biopsy. Indeed, amyloid deposition was noted to be more pronounced on the second renal biopsy, particularly at tubular basement membranes. Although the variable relation between reduction in amyloid load and changes in organ function is well-known, the basis for renal recovery in association with stable or even progressive amyloid deposition is challenging. We suggest that in patients with secondary AA amyloidosis, mechanisms other than the reduction of amyloid mass could have contributed to the observed improvement of renal function with anti-TNF agents.

Evaluation of the short-term efficacy of NSAIDs on patients with active ankylosing spondylitis in daily practice: a 3-month, longitudinal, observational study.

The objective of the study was to investigate the response rate to non-steroidal anti-inflammatory drugs (NSAIDs) and the clinical parameters that might predict this response in patients with active ankylosing spondylitis. This is a prospective, observational, 3-month study that was conducted in a single center. Ninety-five consecutive patients with active ankylosing spondylitis were included in the study. Full dose NSAIDs (indometacin 150 mg daily or acemetacin [corrected] 180 mg daily) were given to patients. Relevant clinical data of all patients' were recorded at the beginning and on three consecutive monthly visits. At the end of the study period, patients who respond to NSAIDs were determined. Demographic, clinical, and laboratory parameters that might influence the response to the NSAIDs were investigated. The response rate to the full-dose NSAIDs according to the ASAS20 in patients with active ankylosing spondylitis was found as 29.5%. Similarly, 20.0% of the patients were responders according to the ASAS40 criteria, whereas 5.6% of the patients responded according to the 5-out-of-6 criteria at week 12. Patients who responded to the treatment were found to be younger at the study entry (P = 0.001) and had shorter disease duration (P < 0.001). Due to the markedly lower rate of response to the NSAIDs in patients with active ankylosing spondylitis, early identification of those patients who does not respond to NSAIDs and subsequent decision regarding the institution of second-line treatments (anti-TNF) may be of great value in the prevention of irreversible changes that might develop in most of the patients.

Ulnar artery aneurysm in a patient with Behçet's disease.

Behçet's disease is a systemic disease characterized by oral aphthosis, genital ulcers, ocular lesions, gastrointestinal, musculoskeletal, neurological and major vessel involvement. Arterial involvement, aneurysms and arterial thrombosis have been reported in 1.5-3% of patients. In this case report, we present a patient with ulnar arterial aneurysm associated with Behçet's disease.

Low-dose natural human interferon-alpha lozenges in the treatment of Behçet's syndrome.

OBJECTIVES: There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-alpha administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study. METHODS: Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for > or = 1 year were studied. When they had at least two OUs with a total diameter of > or = 4 mm, they were randomly allocated to (i) 2000 IFN-alpha IU/day, (ii) 1000 IFN-alpha IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12. RESULTS: Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint. CONCLUSIONS: Low-dose natural human IFN-alpha did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies. TRIAL REGISTRATION: ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184.

The close association between dental and periodontal treatments and oral ulcer course in behcet's disease: a prospective clinical study.

OBJECTIVE: The aim of the study was to evaluate the influence of dental and periodontal treatments to the course of oral ulcers in patients with Behcet's disease (BD). METHODS: Fifty-eight consecutive BD patients with oral ulcers were studied. Twenty-nine patients were in the intervention group (F/M: 15/14, mean age: 39.6 +/- 6.9 years) and 29 (F/M: 15/14, 39.4 +/- 10.6 years) were followed with a conventional treatment approach. In addition to oral hygiene education, dental and periodontal treatments were carried out in the intervention group, whereas the control group was only given oral hygiene education. Patients were evaluated in the pre-treatment observation period (1 month), treatment period (1 month) and 6 months after treatment. RESULTS: An increase in the number of new oral ulcers (4.1 +/- 3.5) was observed within 2 days during the treatment compared with 3-30 days during treatment month (2.3 +/- 1.2) (P = 0.002). However, 6 months after the treatment, the number of oral ulcers (1.9 +/- 1.5) was significantly lower compared with the pre-treatment observation (4.8 +/- 3.2) (P = 0.000) and treatment periods (6.4 +/- 2.3) in the intervention group (P = 0.05), whereas a similar oral ulcer presence was observed in the control group (2.8 +/- 2.4, 3.7 +/- 2.3 and 4.8 +/- 4.3, respectively) (P > 0.05). Dental and periodontal indices were also better in the intervention group during the 6-month follow-up. CONCLUSION: Our results suggest that, in BD patients, dental and periodontal therapies could be associated with a flare-up of oral ulcers in the short term, but may decrease their number in longer follow-up. They also lead to a better oral health.

Randomized trial of pimecrolimus cream plus colchicine tablets versus colchicine tablets in the treatment of genital ulcers in Behçet's disease.

BACKGROUND: Recurrent and painful genital ulcers are the hallmark of Behçet's disease. OBJECTIVE: To determine the efficacy of pimecrolimus (PIM) cream on the pain and healing time of genital ulcers. METHODS: A total of 76 patients were randomized to either receive PIM cream plus colchicine (COL) tablets (1-2 g/day) or COL tablets (1-2 g/day) alone for 1 month. Clinical evaluations were performed in 68 patients at the baseline and on the 3rd, 7th, 10th, 14th and 28th days of treatment. Also, genital ulcer pain was evaluated using a verbal pain score at each visit. Safety was monitored through adverse event reporting and laboratory tests. RESULTS: The mean healing time of genital ulcers was shorter in the PIM + COL group (4.2 +/- 1.5 days) than the COL group (4.7 +/- 1.8 days), without statistical significance (p = 0.399). Visual analog scale scores decreased in both groups significantly. Neither of the treatment modalities was found to be superior to the other; however, pain was relieved in 4.2 +/- 0.5 days in PIM + COL group and in 5.5 +/- 1.2 days in COL group in the intention to treat population (p = 0.023). Observed adverse events were transient. CONCLUSION: Compared to COL alone, COL + PIM cream shortens the pain duration without any significant effect on healing time.

Afibrinogenemia resulting from homozygous nonsense mutation in A alpha chain gene associated with multiple thrombotic episodes.

Congenital afibrinogenemia is a rare disorder characterized by the absence in circulating fibrinogen, a hexamer composed of two sets of three polypeptides (Aalpha, Bbeta and gamma). Although predisposition to thrombosis is a well known feature of dysfibrinogenemia, the relatively frequent thrombotic manifestations seen in congenital afibrinogenemia are puzzling. We herein report a mutational analysis of a young afibrinogenemic man from Turkey with multiple thrombo-embolic events involving both arteries and veins. Purified DNAs of the propositus was used for amplification by polymerase chain reaction of all the exons of the A subunit gene with primers allowing the analysis of the intron-exon boundaries. Analysis of the genes coding for the three fibrinogen chains of the propositus found a homozygous G to A transition in the exon 5 of the A alpha chain gene (g.g4277a; access number gi458553). The TGG to TGA codon change predicts a homozygous W315X in the A alpha chain (p.W334X when referring to the translation initiation codon). Both parents and his brother were found to carry this heterozygous mutation. This is the first report of a patient homozygous for this rare mutation associated with afibrinogenemia. Our patient was free of known risk factors as well as diseases associated with thrombosis including atherosclerosis, vasculitis, Buerger's disease, and it seems therefore probable that afibrinogenemia itself might have contributed to both arterial and venous thrombosis.

Accuracy of recall of the items included in disease activity forms of Behçet's disease: comparison of retrospective questionnaires with a daily telephone interview.

The assessment of disease activity in Behçet's disease (BD), either by BD Current Activity Form (BDCAF) or Iranian BD Dynamic Measure (IBDDAM), depends largely on the history of the clinical features. Accuracy of recall of a retrospective questionnaire might influence its reliability to some extent. The aim of this study was to investigate whether patients with BD can recall the items included in both of the disease activity forms accurately. Twenty patients with BD completed a retrospective questionnaire that included BDCAF and IBDDAM, twice, 1 month apart. We made some modifications in the IBDDAM, while the BDCAF (revised 8.5.2002, GL) was applied in its original structure. In contrast to the original application of IBDDAM, in which the history obtained depends on the variable time period, all items of IBDDAM in the present study asked subjects to report their symptoms over the preceding 4 weeks. Furthermore, those IBDDAM items for which the operational definitions are not clearly provided by the developers, as well as the items that depend on the objective examination rather than the history, were not included. Concurrent daily data were collected for 2 months via telephone interview. The level of agreement between the retrospective responses and those extracted from daily recordings were quantified for each item using intraclass correlation coefficient (ICC). The agreement between the recalled and daily reports was good (ICCs>0.60) for the majority of items (oral and genital ulceration, skin lesions, arthralgia, arthritis, and eye involvement) included in BDCAF. Exceptions were gastrointestinal symptoms (ICCs<0.40) and headache (ICCs 0.40-0.50). With regard to the IBDDAM, all of the items examined had good agreement (ICCs>0.60), except for headache, for which the agreement was moderate (ICCs 0.40-0.50) for the first and poor (ICCs<0.40) for the second month. For most of the BD-related features questioned in the disease activity forms, patients provided reasonably accurate reports in comparison to information obtained from daily records. However, it should be kept in mind that the limitations of the study design need to be considered while interpreting the results of the present study. Notably, unlike its original application in which the assessment depends on the variable time period, IBDDAM used in the present study assessed the symptoms present during the month prior to the date of assessment. Therefore, our findings regarding IBDDAM cannot be applied to its original use. Furthermore, lack of patients in some of the symptom groups, as well as possible manipulation of recall with daily questioning, might have influenced our results to some extent.