Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.

OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud's phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs). METHODS: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined. RESULTS: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use. CONCLUSIONS: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.

Predictive factors for dose reduction and discontinuation of nintedanib in fibrotic interstitial lung diseases: Real life data.

Nintedanib, an intracellular inhibitor targeting multiple tyrosine kinases, has emerged as a standard treatment for various fibrotic lung diseases. Despite its efficacy, side effects such as nausea, diarrhea, and hepatotoxicity often lead to dose reduction or discontinuation. In this retrospective analysis at an university hospital's interstitial lung disease clinic, we aimed to identify baseline characteristics associated with dose adjustment or treatment discontinuation. Of the 58 patients included, 41.4% maintained the full nintedanib dose, while 31.0% required dosage reduction, and 27.6% discontinued treatment due to adverse events, predominantly gastrointestinal and hepatotoxic effects. Multivariate analysis revealed body surface area (BSA) as an independent and significant baseline risk factor (adjusted Odds Ratio [aOR] 0.22), suggesting a 78% decreased chance of requiring dose modification for every decimal point increase in BSA. A BSA cutoff of ≤1.73 m [2] exhibited a sensitivity of 73% and specificity of 91.7%, with significant impact on one-year survival under full-dose treatment (p < 0.001). Lower BSA was associated with early onset adverse effects, particularly gastrointestinal, supporting the need for regular clinical monitoring. The study emphasizes the importance of recognizing baseline factors to ensure the safety and tolerability of nintedanib, thereby preventing the progression of pulmonary fibrosis. These findings contribute to the evolving understanding of nintedanib management in fibrotic interstitial lung diseases, guiding clinicians in personalized treatment approaches.

Early factors associated with the initiation of treatment with biologics in patients with Axial Spondyloarthritis - results from a single centre retrospective cohort study.

BACKGROUND: Axial Spondyloarthritis (axSpA) refers to a group of rheumatic diseases that mainly affect the axial skeleton. Treatment with Biological Disease Modifying Anti-Rheumatic Drug (bDMARDs) is indicated when low disease activity is not achieved with Non-Steroid Anti-inflammatory Drugs. Certain clinical and socio-demographic features may be predictive of future need for treatment with bDMARDs in a patient with axSpA. OBJECTIVES: To study a population of patients with axSpA and determine whether the presence of certain factors at diagnosis is associated with a later need for biological treatment. METHODS: A single centre retrospective cohort study was conducted comprising 150 patients with axSpA that attended the Rheumatology Outpatient Clinic from January to December 2019. Logistic Multivariate Regression was performed to understand which factors independently contributed to the use of bDMARDs. RESULTS: Fifty-two patients (34,7%) were under biological treatment. In comparison to the group that was not under treatment with bDMARDs, these were significantly more likely to be hard-workers (57,8% vs 29,7%; p = ,003), to have had elevated C-Reactive Protein at the time of diagnosis (81,6% vs 48,9%; p < ,001), to have had a grade of sacroiliitis at diagnosis greater than 2 (67,4% vs 29,5%; p < ,001) and to have history of enthesitis, (32,7% vs 13,3%; p = ,006). In multivariate regression analysis, only the hard-worker type (OR = 3.09, CI: 1.14 - 8.37; p = .027) and the highest grade of sacroiliitis (OR = 4.41, CI: 1.69 - 11.50; p = .002) were found to be independently associated with the use of bDMARDs. CONCLUSION: In this study, the performance of work associated with greater biomechanical stress and the presence of greater structural damage at diagnosis were shown to be associated with the use of bDMARDs. The authors highlight the importance of recognizing these factors that seem to relate to more aggressive disease, with higher use of bDMARDs, thus suggesting a need for a tighter control management strategy in these patients.

Septic Polyarthritis by Staphylococcus aureus of "Unknown Origin".

ABSTRACT: More than 90% of septic arthritis cases are monoarticular. Joint infection can occur through several mechanisms such as hematogenous dissemination, by contiguity from adjacent infected soft tissue, surgical contamination, direct inoculation, or joint trauma.We report the case of a 69-year-old man admitted to our hospital with septic polyarthritis. The presented case is remarkable given its atypical presentation. The patient had no known risk factors for septic arthritis, comorbidities, or history of recurrent infections that could suggest some degree of immunosuppression. The atypical polyarticular involvement at presentation, the absence of sustained fever, and the good general condition of the patient delayed the diagnosis and treatment.

Remission of Rheumatoid Arthritis and Primary Biliary Cholangitis After Treatment With Tocilizumab.

Rheumatoid arthritis (RA) is characterized by synovitis of multiple joints which if untreated progresses to joint destruction. Primary biliary cholangitis (PBC) is an autoimmune and progressive disease of the liver of unknown origin. About 1.8-5.6% of individuals with PBC have RA and patients with RA are at higher risk of developing PBC compared to the general population. We report a case of a 76-year-old man, with a history of PBC, and a recent RA diagnosis, in which tocilizumab therapy was effective in the control of RA and PBC, and a literature review was performed. This case, along with only one case published in literature in which tocilizumab was used in the treatment of RA and PBC, suggests that tocilizumab may be effective and safe in the treatment of RA in patients with PBC. Inhibition of IL-6 may also be effective in PBC treatment.

Prevalence and clinical manifestations of Erasmus syndrome in systemic sclerosis: a cross-sectional study.

INTRODUCTION: Erasmus syndrome (ErS) is a rare entity in which Systemic Sclerosis (SSc) develops following exposure to silica, with or without associated silicosis. The objectives of this study were: 1) to evaluate the prevalence of ErS in our SSc cohort; 2) to characterize the cases; 3) to evaluate the clinical and laboratory characteristics of SSc in patients with (Ers) or without silica exposure. METHODS: Cross-sectional and analytical study. Sociodemographic, clinical and laboratory data were collected from all patients with SSc diagnosed in our department according to ACR / EULAR criteria. Data on professional activity and possible exposure to silica were obtained by phone interview. RESULTS: Among 48 patients with SSc, the prevalence of ErS was 16.7% (8/48). All cases identified were male, corresponding to 72.7% of men with SSc followed at our department. There was a statistically significant association between ErS and male gender (p.

Shoulder pain: are there predictive factors of response to treatment and of ultrasound findings?

INTRODUCTION: Shoulder pain is a common cause of consultation in Primary Health Care, and may correspond to up to 30% of the reasons for consultation. Pathology of the rotator cuff is the most common cause of pain. Ultrasound is a valuable diagnostic tool in assessing shoulder disorders; it can be as effective as magnetic resonance imaging. OBJECTIVE: To determine the predictive factors of response to treatment and ultrasound findings in shoulder pain. METHODS: We performed an analysis of the patients' cases sent to the rheumatology consultation with shoulder pain, every patient had an echography shoulder evaluation, and the rheumatologist decided treatment based on the guidelines for the treatment of shoulder tendinopathies. The use of nonsteroidal anti-inflammatory drug (NSAIDs) and muscle relaxant medications as well as the following techniques: corticosteroids local injection, barbotage, capsular distension and physiotherapy programs were some of the variables assessed. Posteriorly, the patients were clinically assessed in a follow-up visit. RESULTS: A total of 119 patients were evaluated. There was a statistically significant relationship between the time from the beginning of the symptomatology and treatment response. Diabetes mellitus, arterial hypertension and dyslipidaemia were statistically significantly associated with some rotator cuff lesions and distention of the subscapular bursa. Age is the main predictor of rotator cuff ultrasound findings. CONCLUSION: In patients with shoulder pain, early intervention positively influences the response to treatment. Thus, it is important that these patients are evaluated more promptly. Some comorbidities seem to be associated with a higher risk of specific rotator cuff lesions. No relationship was found between response to treatment and age, sex, occupation, previous treatments or type of therapy selected. The associations found in this study seem to have clinical implications. Prevention of rotator cuff disease is a matter of major relevance as well as early institution of treatment.

Smoking as associated factor for spondyloarthritis related uveitis: results from a single centre cross-sectional study.

Ocular involvement in spondyloarthritis (SpA) is a frequent extra-articular manifestation and contributes to the burden of disease. Factors associated with spondyloarthritis-related uveitis (SpA-U) are poorly defined in literature. The influence of smoking status on the occurrence of uveitis in SpA is controversial. To clarify the factors associated with SpA-U, we performed an observational cross-sectional study in a Tertiary Rheumatology Centre. Factors independently associated with uveitis were determined by logistic regression models. The study included 164 patients fulfilling the ASAS criteria for axial SpA with follow-up visit between January and June 2019. Smoking was independently associated with uveitis (OR=2.54; 95%CI [1.01-6.42]; p=0.03). Our study emphasizes the importance of smoking cessation in SpA which may have a positive effect in different disease features like uveitis and overall prognosis.

Intravenous human immunoglobulin for the treatment of recurrent focal myositis.

We present a case of recurrent focal myositis treated with intravenous human immunoglobulin, after ineffectiveness of analgesics, non-steroidal anti-inflammatory drugs, corticosteroids and immunosuppressive therapies. There was a prompt clinical and laboratorial improvement. To the best of our knowledge, this is the first case reporting the administration of this treatment in recurrent focal myositis.

Tocilizumab and rituximab have similar effectiveness and are both superior to a second tumour necrosis factor inhibitor in rheumatoid arthritis patients who discontinued a first TNF inhibitor.

OBJECTIVES: To compare the effectiveness of a 2nd TNF inhibitor (TNFi), Tocilizumab (TCZ) and Rituximab (RTX), measured by drug retention and by response rates, in RA patients after discontinuing a first-line TNFi and to clarify the reasons and predictors for discontinuation of a second-line biologic. MATERIAL AND METHODS: Non-interventional prospective study of RA patients exposed to a 2nd TNFi, TCZ or RTX after previous TNFi discontinuation using real-world data from Reuma.pt database. Drug retention was estimated using Kaplan-Meier analysis and Cox models. Crude and LUNDEX adjusted response rates were evaluated at 6 months, 1 and 2 years and reasons for discontinuation were compared according to biologic class. RESULTS: In total, 643 patients were included, 88.8% females, with a mean age of 59.4±12.8 years. Of those, 390 (60.7%) initiated a 2nd TNFi, 147 (22.9%) TCZ and 106 (16.5%) RTX. Drug retention was significantly greater among patients who initiated TCZ (76.4±4.3 months) or RTX (80.8±4.8 months), compared with those who initiated a 2nd TNFi (52.7±2.6 months) (log rank test, p < 0.001). In the adjusted Cox model, hazards of discontinuation were significantly lower for TCZ (HR 0.39, 95% CI 0.23-0.64, p < 0.001) and RTX (HR 0.42, 95% CI 0.25-0.72, p=0.001). Smokers had a significantly higher risk for discontinuation (HR 2.43, 95%CI 1.50-3.95, p < 0.001) as well as patients with higher HAQ at baseline (HR 1.51, 95%CI 1.14-2.00, p=0.004). The proportion of patients in remission or low disease activity according to Clinical Disease Activity Index (CDAI) at 6 months, 1 and 2 years was, respectively, 46.5%/50.0%/61.2% for TNFi, 52.9%/53.6%/ 69.2% for TCZ and 37.7%/48.0%/50.0% for RTX. After LUNDEX adjustment, response rates were, respectively, 33.0%/31.0%/31.8% for 2nd TNFi, 42.8%/41.8%/53.3% for TCZ and 32.0%/39.4%/39.0% for RTX. The main reasons for discontinuation were inefficacy for 2nd TNFi and RTX and adverse events for TCZ (p < 0.001). CONCLUSIONS: Our findings showed a significantly higher drug retention for TCZ and RTX, compared with 2nd TNFi, and similar persistence among TCZ and RTX, in patients who discontinued a first-line TNFi. These data corroborate the notion that switching to a biologic with a different mode of action is more effective than to a second TNFi.

Neuropathic pain in Systemic Sclerosis patients: A cross-sectional study.

OBJECTIVES: To investigate if patients with Systemic Sclerosis (SSc) show a higher prevalence of neuropathic pain (NP) in comparison with controls. To study the relationship between clinical variables of the disease and NP among SSc patients. MATERIAL AND METHODS: 48 patients and 45 controls were included. Presence of NP was assessed applying the DN4 "Douleur Neuropathique en 4 Questions" questionnaire. Different clinical variables were also assessed in patients. Statistical analysis included parametric, nonparametric tests and multivariate logistic regression. RESULTS: NP was significantly higher in SSc patients (56.2% vs 13.3%, p<0.001). Mean Modified Rodnan Skin Score was independently associated with the presence of NP (p<0.05, OR 1.90). CONCLUSIONS: Peripheral nervous system involvement in SSc is not well studied and, as far as the authors are aware, this is the first study published evaluating NP in SSc patients and controls. These findings should raise the awareness of the clinician to recognize and address the presence of NP in these patients, especially in those with severe skin involvement.

Obesity, metabolic syndrome and other comorbidities in rheumatoid arthritis and psoriatic arthritis: influence on disease activity and quality of life.

In obesity, especially if visceral, and in rheumatic diseases, the production of pro-inflammatory cytokines contributes to an increased cardiovascular risk. Moreover, classic cardiovascular risk factors are more common in these patients. We intended to assess the influence of body mass index (BMI), abdominal circumference (AC) and metabolic syndrome (MS) on disease activity and quality of life in Rheumatoid Arthritis (RA) and Psoriatic Arthritis patients and to compare the results between the RA and PsA patients. We performed a cross-sectional study, including 150 patients with RA and 75 patients with PsA. PsA patients had significantly higher BMI, AC and total of comorbidities than RA patients . Independently the underlying pathology (RA or PsA), the number of comorbidities was correlated positively with DAS28, HAQ , CRP and ESR. In RA group, overweight/obesity (BMI≥25kg/m2) were associated with at least one painful joint and the risk of having at least one swollen joint was 3.4 times higher in patients with increased AC. There was an association between the BMI and AC and the CRP value. Patients with BMI≥25 kg/m2 and increased AC had significantly higher DAS28 scores. MS was associated with significantly higher ESR. There was a positive correlation of both BMI and AC with HAQ and also MS was associated with highest HAQ values. In PsA group, patients with BMI≥25kg/m2 had equally more painful joints and higher CRP values. Patients with MS had higher CRP values, more joint pain and higher disease activity according to DAS28. None of the patients with normal BMI had swollen joints, however 20.4% of overweight patients had at least one swollen joint. The number of comorbidities showed to influence inflammatory parameters, disease activity and quality of life. We found that BMI, AC and MS are associated with disease activity, which may be improved by weight reduction and comorbidities control.

Systemic sclerosis and myositis as a paraneoplastic syndrome secondary to multiple myeloma.

We present a case of paraneoplastic systemic sclerosis (SSc) and myositis associated to a multiple myeloma (MM) in a 52-year-old Caucasian man. After MM treatment, skin and muscle changes improved, with no further relapses. Although rare, "scleroderma-like" or myositis lesions may be associated with MM. However, to the best of our knowledge, this is the first case reporting these two clinical conditions simultaneously associated with MM.