Synovitis in rheumatoid arthritis detected by grey scale ultrasound predicts the development of erosions over the next three years.

OBJECTIVES: To evaluate grey scale US (GSUS) and power Doppler US synovitis (PDUS), separately or in combination (CombUS), to predict joint damage progression in RA. METHODS: In this cohort study nested in the Swiss RA register, all patients with sequential hand radiographs at their first US assessment were included. We analysed the summations of semi-quantitative GSUS, PDUS and CombUS assessments of both wrists and 16 finger joints (maximum 54 points) at their upper limit of normal, their 50th, 75th or 87.5th percentiles for the progression of joint damage (ΔXray). We adjusted for clinical disease activity measures at baseline, the use of biological DMARDs and other confounders. RESULTS: After a median of 35 months, 69 of 250 patients with CombUS (28%), 73 of 259 patients with PDUS (28%) and 75 of 287 patients with available GSUS data (26%) demonstrated joint damage progression. PDUS beyond upper limit of normal (1/54), GSUS and CombUS each at their 50th (9/54 and 10/54) and their 75th percentiles (14/54 and 15/54) were significantly associated with ΔXray in crude and adjusted models. In subgroup analyses, GSUS beyond 14/54 and CombUS higher than 15/54 remained significantly associated with ΔXray in patients on biological DMARDs, while clinical disease activity measures had no significant prognostic power in this subgroup. CONCLUSION: Higher levels of GSUS and CombUS are associated with the development of erosions. GSUS appears to be an essential component of synovitis assessment and an independent predictor of joint damage progression in patients on biological DMARDs.

Behçet's syndrome: clinical presentation and prevalence in Switzerland.

OBJECTIVE: Behçet's syndrome is a rare systemic autoimmune/autoinflammatory disease affecting mucocutaneous tissues, the skin and the eyes, as well as the joints, the central nervous system, the gastrointestinal tract and blood vessels. Because of the lack of clinical data in Switzerland, the aims of this cohort study were to calculate the disease prevalence and to analyse the disease manifestations and the immune-suppressive medication. METHODS: Data were extracted from 52 patient charts. Thereafter, all patients were interviewed with a questionnaire and 46 had an additional physical examination and laboratory analyses. For calculation of prevalence, data of the national statistical bureau were used. RESULTS: A disease prevalence of 4.03/100,000 inhabitants was calculated. The mean delay between first disease manifestation and diagnosis was 8 years. It was 2 years longer for Swiss than for non-Swiss individuals (p = 0.45). The time intervals between diagnosis and occurrence of different organ manifestations ranged from +8 to -11 years. There was no difference in organ involvement between different ethnicities. Colchicine was prescribed for 52% of patients only, whereas tumour necrosis factor (TNF) inhibitors and glucocorticoids were most frequently prescribed (80 and 64%, respectively). In almost half of the patients, TNF blockers could be stopped and replaced by conventional immunosuppressive drugs. CONCLUSION: The data from this cohort of Behçet's syndrome patients, the largest in Switzerland, documents a prevalence higher than anticipated. The diagnostic delay underlines an urgent need to improve awareness of the disease and allow timely treatment.

Hypothesis-free analyses from a large psoriatic arthritis cohort support merger to consolidated peripheral arthritis definition without subtyping.

Current ClASsification criteria for Psoriatic ARthritis classification criteria for psoriatic arthritis (PsA) provide a preliminary definition of inflammatory articular disease. This study aimed to further characterize PsA peripheral arthritis using purely data-driven approaches for the affected joint distribution pattern. PsA patients from the Swiss Clinical Quality Management in Rheumatic Diseases (SCQM) database were clustered according to similarities in 66 swollen and in 68 tender joints. Clusters were compared in terms of other disease characteristics and studied for coincidence with traditional PsA subtypes, stability over time and treatment response upon first tumour necrosis factor alpha (TNF-α) therapy. Clustering of 957 patients resulted in an oligoarticular, a polyarticular hand dominated, a polyarticular foot dominated and a fourth cluster which was characterized by polyarticular involvement of the hands and feet. Of the traditional PsA subtypes, only a non-PsA-specific oligoarticular joint involvement pattern was retrieved by clustering. When comparing clusters in other disease manifestations, only minor and clinically probably irrelevant differences occurred. Over time, clusters were more robust than traditional PsA subtypes. Patients in different joint clusters had similar response rates upon first anti-TNF-α therapy, and minimal disease activity was achieved in 56% of 285 patients, irrespective of cluster membership. Hypothesis-free approaches to group PsA patients yield clusters with improved consistency, but without clinically important differences. Taken together, the current peripheral arthritis definition by GRAPPA without further specification into subtypes is strongly supported by the data.

Outcome and quality of life in patients treated for abdominal aortic aneurysms: a single center experience.

BACKGROUND: Durability of protection and long-term quality of life (QoL) are critical outcome parameters of abdominal aortic aneurysm (AAA) repair. The aim of the present study was to compare results of endovascular and open aneurysm repair (EVAR and OR) with adjusted standard populations, including stratification for urgency of presentation. METHODS: Retrospective analysis of prospectively collected data of 401 consecutive patients presenting with AAA between January 1998 and December 2002. Cross-sectional follow up was 58 +/- 29 months. Patients were grouped into three cohorts: elective EVAR (n = 68), elective OR (n = 244), and emergency OR (including symptomatic and ruptured AAA, n = 89). Endpoints were perioperative (i.e., 30 days or in-hospital) and late mortality rates, as well as long-term QoL as assessed by the Short Form health survey questionnaire (SF-36). RESULTS: Mean age was lower in the elective OR cohort (66 +/- 10 years) than in the EVAR cohort (72 +/- 7 years; p < .05). Perioperative mortality rates were 4.4%, 0.4%, and 10.1%, for the EVAR, elective OR, and emergency OR cohorts, respectively (p < .05). Corresponding cumulative survival rates after 4 years were 67%, 89%, and 69%, respectively. Long-term QoL SF-36 scores were in all cohorts similar to age- and gender-adjusted standard populations, which score between 85 and 115: 99.6 +/- 35.8 (EVAR), 101.3 +/- 32.4 (elective OR), and 100.4 +/- 36.5 (emergency OR). CONCLUSIONS: Long-term QoL is not permanently impaired after AAA repair, but returns in long-term survivors to what would be expected in a standard population. In this respect, differences were found neither between EVAR and OR, nor between elective and emergency repair. Perioperative mortality rates were highest in patients undergoing emergency OR. The outlook for such patients after the perioperative period, however, was similar to that for patients undergoing elective repair.

Does malperfusion syndrome affect early and mid-term outcome in patients suffering from acute type A aortic dissection?

It is well known that malperfusion syndrome (MPS) increases early mortality of patients suffering from acute type A aortic dissection (AADA). The aim of the present study was to analyze the outcome of patients who survived after surgical treatment of AADA with or without MPS. Data of 227 consecutive patients, who underwent surgery for AADA, were analyzed. The impact of MPS on in-hospital data and outcome was analyzed. Quality of life (QoL), using the short form 36 health survey questionnaire (SF-36), and late mortality were analyzed. Seventy-five patients (33%) with AADA had preoperative MPS. In 31 patients (41.3%), central nervous system (CNS) was involved and in 33 patients (44%) MPS of the extremities was present. Coronary malperfusion was found in 9 patients, renal in 8 patients and visceral malperfusion in 5 patients. Mean age in the group with MPS was 61.9+/-9.1 compared to 61.6+/-12.7 years without MPS (P=ns). In-hospital mortality was 18.7% in patients with MPS, compared to 9.9% without MPS (P<0.05). Follow-up revealed a significant poorer outcome in patients with MPS, with a 3-year-survival of 73.3% in patients with MPS and 86.2% without MPS (P<0.05). Average SF-36 values were lower in patients with MPS (78.3+/-12.8 vs. 87.8+/-11.9; P=ns), which is mainly due to patients with CNS-MPS, who showed an average SF-36 of 65.8+/-17.9 (P<0.05). AADA associated to MPS carries a higher early- and mid-term mortality. Postoperative mid-term QoL, however, except in patients with CNS-MPS and persistent neurological deficits, is fairly good and similar to patients who underwent successful surgery for AADA without MPS.