Acta reumatologica portuguesa最新文献

Bronchocentric granulomatosis (BcG) is characterized by granulomatous destruction of bronchial or bronchiolar walls and adjacent parenchyma, with debris and exudates filling the airway lumen. Approximately 50% of total cases have been associated with asthma and allergic bronchopulmonary aspergillosis, while it has been rarely reported in the context of rheumatoid arthritis (RA). We describe the case of a 69-year-old female RA patient with BcG presenting as a solitary cavitary pulmonary mass. In addition, we conducted a literature review about the clinical and imaging features of BcG in RA patients. A chronically immunosuppressed 69-year-old female patient with a 16-year history of RA presented with constitutional symptoms (low-grade fever, excessive sweating and malaise) and a sizeable cavitary lung lesion. Open lung biopsy was performed and histopathological findings were consistent with the diagnosis of BcG. Other seven cases of BcG have been previously reported in the context of RA, with clinical and laboratory characteristics described in five of them. Overall, pulmonary nodules or masses were the most frequent imaging finding of BcG, while no clear relationship with disease activity or previous treatment modalities could be established. Surgical resection followed by administration of oral steroids was effective for achieving complete remission of symptoms and radiological stability in most cases.

Objective: To evaluate and describe the strategies of Portuguese rheumatologists and paediatricians, regarding either the maintenance or the withdrawal of classic and biologic disease-modifying anti-rheumatic drugs (cDMARDs and bDMARDs, respectively), when patients with Juvenile Idiopathic Arthritis (JIA) achieved clinical inactive disease (CID).

Methods: We performed a 30-question questionnaire, which was sent to all the 35 clinicians enrolled in the Portuguese group of paediatric rheumatology.

Results: Twenty-three complete responses were obtained. The factors with the greatest impact on the decision to withdraw cDMARDs were: the duration of the CID, the therapy-induced toxicity, the presence of erosive disease and joint damage, the subtype of JIA, the time to reach inactive disease and the low adherence to therapy. These factors were classified as "very important" in this decision by more than 50% of the clinicians. The same factors, except for low adherence, had the greatest impact, when considering the withdrawal of bDMARDs. Withdrawal was more likely in patients with persistent oligoarticular JIA and less likely in rheumatoid factor positive polyarticular JIA. Sulfasalazine was more susceptible to be discontinued than methotrexate. Contrariwise, there were no differences concerning bDMARDs. Most participants reported that they started the drug withdrawal only after 12 months of sustained remission, by progressively tapering the dose of the cDMARD and spacing the intake of the bDMARD. Also, they reported that the decision to suspend the DMARD was based on imaging methods, preferably ultrasound, and in patient-reported outcomes. For patients on combination therapy, bDMARDs are reported to be the first to be withdrawn.

Conclusions: Literature is scarce on this matter and there are no well-defined guidelines on how to withdrawal cDMARDs or bDMARDs on JIA. Notwithstanding, most Portuguese physicians were in agreement on the factors that needed to be taken into account with respect to the withdraw decision.

Objectives: To evaluate belimumab effectiveness and safety in real-life Portuguese patients with Systemic Lupus Erythematosus (SLE).

Materials and methods: Multicenter cohort study including all SLE patients treated with belimumab in seven Portuguese rheumatology centers. Demographic, clinical and serological data were collected at baseline, 6, 12 and 24 months of treatment with belimumab. To evaluate effectiveness we used SLE Responder Index (SRI) rates and changes in SELENA-SLEDAI. Safety was evaluated by the number of adverse events.

Results: Thirty-eight patients were included: 37 (97.4%) female, with a mean age of 46.2±13.9 years. Mean SELENA-SLEDAI was 8.2±3.9, 78.8% had elevated anti-double-stranded DNA (anti-dsDNA) antibodies and 72.7% had complement consumption at baseline. Multiorgan involvement was the leading cause for the use of belimumab. SRI response was achieved in 51.9%, 60% and 91.7% at 6, 12 and 24 months of belimumab treatment, respectively. LUNDEX adjusted SRI response rates were 45.4%, 45.0% and 45.8% at 6, 12 and 24 months of belimumab, respectively. Mean SELENA-SLEDAI, anti-dsDNA antibodies and daily prednisolone dosage decreased significantly from baseline to 6, 12 and 24 months and C3 levels increased significantly at 12 months of belimumab treatment. Five patients presented adverse events (infections in three cases) and eleven patients discontinued belimumab (four due to inefficacy, three due to adverse events and four were lost to follow-up).

Conclusions: Our study confirmed, in real-life Portuguese patients with active SLE, the effectiveness of belimumab in reduction of disease activity, immunological response and steroid-sparing, with a good safety profile.

Objective: To evaluate the rate of early retirement due to rheumatoid arthritis (RA) in Portugal.

Methods: Prospective cohort study involving 11 Portuguese centers, including patients with a clinical diagnosis of RA, based on Reuma.pt registry, enrolled between 2008 and 2019.

Results: 3231 patients were included (81.5% female, aged 60.8 ± 13.0 years, mean disease duration 18.0 ± 10.3 years). Until the present time, 37.6% of these patients retired, 59.6% due to RA. Early retirement due to RA translated into losing 7 years of active work when compared to patients retired to other causes. Compared to professionally active patients, retired patients due to RA were diagnosed later in the disease process (p=0.003), had longer disease duration (p < 0.001), were more frequently positive for rheumatoid factor (p=0.043), had more frequently erosive disease (p < 0.001), had a blue-collar occupation (p < 0.001) and had a lower educational level (p < 0.001). Independent predictors for early retirement due to RA were: delayed diagnosis (OR: 2.23; 95% CI 1.18-4.21/year, p=0.013), erosive disease (OR: 2.21 95% CI 1.54-3.16, p < 0.001), need for biologic therapy (OR: 1.32; 95%CI 1.01-1.73, p=0.045) and lower educational level (OR: 0.83; 95%CI 0.79-0.86/year, p < 0.001).

Conclusion: RA is, itself, the leading cause of early retirement in RA patients, accounting for the loss of an average of 7 years of active work. Delayed diagnosis, erosive disease and lower educational level are the main predictors of early retirement associated with RA in this population.

Osteoarthritis (OA) is frequently regarded by patients and health care providers as a normal consequence of ageing and a minor condition. In contrast, rheumatoid arthritis (RA) is a pathological condition that usually requires prolonged treatment and regular Rheumatology follow-up. Pain and physical limitations are hallmarks of both conditions and some previous studies suggest that OA and RA may have a similar burden for both groups of patients although those works usually do not take into account the inflammatory activity of RA. With this work, the authors compare levels of pain, physical disability and health-related quality of life in patients with primary hand osteoarthritis (hOA) and with RA - active disease (aRA) or in remission (rRA). The results show that hOA may have similar or even higher burden of pain than RA even with clinically relevant inflammatory activity in hand joints. Rather than suggesting that OA could be as severe as RA (or more or less severe), this brief study highlights OA as a cause of severe pain, which should lead us to try to achieve better symptom control for these patients and encourage rheumatologists to endeavor efforts to perform more studies in the field of OA.

Objective: To evaluate serum matrix metalloproteinase (MMP)-3 levels as a prognostic marker for the progression of cartilage damage in patients with knee osteoarthritis (KOA).

Methods: Fifty-six patients, aged 40 to 80 years (62.59 ± 10.11 years) who met the ACR criteria for KOA, were included in a one-year observational prospective clinical study. Complete baseline and follow-up data were collected from 50 out of 56 patients. X-ray and magnetic-resonance images were carried out at baseline and after 12 months. They were evaluated according to the Kellgren-Lawrence and Whole-Organ magnetic Resonance iMaging Score (WORMS) semi-quantitative scales, respectively. Progression of cartilage damage in the medial tibiofemoral compartment was registered at the end of the follow-up using the change in WORMS. Serum levels of MMP-3 were measured during the baseline visit, using enzyme-linked immunosorbent assay.

Results: Significantly higher values of baseline MMP-3 levels were observed in patients with a registered progression of cartilage injury in the medial tibiofemoral compartment of the knee compared with patients with no progression (p = 0.005). Binary logistic regression analysis showed that levels of serum MMP-3 (ng/ml) were an independent predictor of subsequent progression of cartilage injury in the medial tibiofemoral compartment of the index knee (assessed by MRI) (OR = 1.042, CI 95%: 1.002-1.084). Receiver operating characteristic analysis was performed to delineate progressors from non-progressors.

Conclusion: Serum MMP-3 levels may serve as a potential prognostic biomarker for cartilage injury in patients with KOA.