Predictors of Interstitial Lung Disease in Mixed Connective Tissue Disease.

Interstitial lung disease (ILD) frequently complicates mixed connective tissue disease (MCTD) and contributes to increased mortality. We aimed to identify predictors of ILD in MCTD patients. This is a nationwide, multicentre, retrospective study including patients with an adult-onset MCTD clinical diagnosis who met Sharp's, Kasukawa, Alarcón-Segovia, or Kahn's diagnostic criteria and had available chest high-resolution computed tomography (HRCT) data. Univariate and multivariate analyses were conducted. We included 57 MCTD patients, with 27 (47.4%) having ILD. Among ILD patients, 48.1% were asymptomatic, 80.0% exhibited a restrictive pattern on pulmonary function tests, and 81.5% had nonspecific interstitial pneumonia on chest HRCT. Gastroesophageal involvement (40.7% vs. 16.7%, p = 0.043) and lymphadenopathy at disease onset (22.2% vs. 3.3%, p = 0.045) were associated with ILD. Binary logistic regression identified lymphadenopathy at disease onset (OR 19.65, 95% CI: 1.91-201.75, p = 0.012) and older age at diagnosis (OR 1.06/year, 95% CI: 1.00-1.12, p = 0.046) as independent ILD predictors, regardless of gender and gastroesophageal involvement. This study is the first to assess a Portuguese MCTD cohort. As previously reported, it confirmed the link between gastroesophageal involvement and ILD in MCTD patients. Additionally, it established that lymphadenopathy at disease onset and older age at diagnosis independently predict ILD in MCTD patients.

Barriers and alternatives to pediatric rheumatology referrals: a survey of family doctors and pediatricians in Portugal.

BACKGROUND: Access to pediatric rheumatology (PR) is not well described in Portugal. The main goal of this study was to ascertain barriers to PR referrals and subsequent alternative referral patterns among family doctors and pediatricians. METHODS: A web-based survey was e-mailed to family doctors and pediatricians practicing in Portugal, in order to investigate access to PR care issues. Descriptive and comparative analysis was performed. RESULTS: Two hundred and ninety-two responses were obtained, 24.7% from pediatricians and 75.3% from family doctors. Only 12% claimed to have had specific education on PR. Nearly 70% worked less than one hour away from a PR center. Twenty eight percent had referred a patient to PR at least once, and 9.3% experienced a situation in which they considered referring to PR but ultimately did not. Many referred to other specialties, primarily pediatrics, adult rheumatology, and pediatric orthopedics. Pediatricians encountered more diversified rheumatic diseases. Fifty five percent had no opinion on PR centers' support, while 24% found it sufficient. Having specific training on PR, being a pediatrician and a specialist were associated with greater referrals to PR. The most rated measure for PR referrals' improvement was promoting education. Regional access to PR's discrepancies were documented. CONCLUSION: Mainly lack of education on PR, but also uneven national coverage and greater distances to some PR centers were the main barriers to PR referrals, in Portugal. Pediatricians seem to have better education, greater experience and more referrals to PR. The current alternatives for referral are pediatrics, adult rheumatology and pediatric orthopedics. Educational consolidation was the biggest and most rewarding inconsistency to battle against.

Predictive factors of fragility fractures and associated mortality: assessment of patients observed at emergency department.

AIM: To assess the predictive factors for a subsequent fragility fracture (FF) and mortality. METHODS: Retrospective monocentric study including patients observed at the emergency department (ED) of a referral hospital with a FF, between 1st January 2017 and 31st December 2018. Fractures events were identified through discharge codes using the 9th International Classification of Diseases codes and FF were adjudicated after revision of the clinical files. We identified 1673 patients with FF. After calculating a representative sample (95% confidence interval), 172 hip, 173 wrist and 112 vertebral fractures were included in the analysis. Their clinical files were reviewed until 31st December 2020. A multivariate analysis was performed in order to identify predictive factors for FF. RESULTS: Overall, during the follow-up period 76 patients (16.6%) had a new FF and 120 patients (26.3%) died. Multivariate analysis showed that previous visits to the ED due to falls (p=0.002) and malignancy (p=0.026) were independent risk factors for a new FF. The main predictors of mortality were age, hip fracture, oral corticosteroid treatment, normal or low BMI and cardiac, neurologic or chronic kidney disease. CONCLUSIONS: FF are a very prevalent public health problem that can lead to significant morbidity and death. Certain comorbidities seem to be associated with new FF and increased mortality. There might be a substantial missed opportunity for intervention in these patients, namely in ED visits.

ANCA-associated vasculitis in a patient with enteropathic spondylarthritis: a case report and literature review.

Coexistence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and inflammatory bowel disease (IBD) is rare (Sy et al. in Semin Arthritis Rheum 45:475-482, 2016). Nevertheless, we present a case of an AAV in a 53-year-old female with enteropathic spondylarthritis previously treated with tumor necrosis factor α inhibitors (TNFi). Management of vasculitis in a patient with IBD may be problematic due to the difficulty in distinguishing if the vasculitis is an extraintestinal manifestation of the IBD or a new coexistent entity. Moreover, in our report, the previous treatment with TNFi is a possible confounding factor due to the paradoxical effects induced by TNFi, including vasculitis (Ramos-Casals et al. in Curr Rheumatol Rep 10:442-448, 2008). The reported case alerts to the complexity in the management of patients with enteropathic spondylarthritis and vasculitis, as well as discusses the diversity of differential diagnosis in this particular clinical scenario.

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.

Are we overlooking osteoarthritis? - A comparative study of pain, function and quality of life in patiens with hand osteoarthritis and rheumatoid arthritis.

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.

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.

Physician-patient agreement at a rheumatology consultation - construction and validation of a consultation assessment instrument.

OBJECTIVES: After consultations, the physician's perceptions differ from the patient's perceptions concerning illness level, cause, and nature of the problem and content of the consultation. Agreement on problems requiring follow-up was associated with a better outcome. The primary aim of this study was to build and validate an instrument that could assess physician-patient agreement in the rheumatology consultation. The secondary objective was to assess agreement association with patient's clinical and sociodemographic data. MATERIALS AND METHODS: A ten-item questionnaire - "Consultation Assessment Instrument" (CAI) - was developed for this study to assess the physician-patient agreement. Ten physicians and 102 patients diagnosed with an inflammatory joint disease under biological therapy were included. The items were evaluated and the index of proportional agreement for the dichotomized answers "agree" (Ppos) and "disagree" (Pneg) was calculated. RESULTS: Consultation satisfaction was the item with the highest agreement. On the opposite end, the item about the explanation of treatment importance was the item with the lowest agreement between patient and physician. Except for one item, the high level of agreement between patient and physician was due to a higher Ppos. Index of proportional agreement was high for 9 of the 10 items (0.816≤ Iv ≤0.990). Patients with lower disease activity scores had a more positive experience. A good internal consistency was obtained for both patient's and physician's questionnaire (α = 0.88 and α = 0.80, respectively). CONCLUSIONS: Both patient and physician showed a positive experience towards Rheumatology consultation. Physician-patient agreement was high in the majority of the consultation aspects (mean Iv = 0,93). A good internal consistency was obtained for both patient's and physician's questionnaire. CAI could be useful as a mental checklist in daily practice or as an educational tool for training consultation skills.

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.

Reuma.pt/vasculitis - the Portuguese vasculitis registry.

BACKGROUND: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. RESULTS: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small- and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported. CONCLUSIONS: Reuma.pt/vasculitis is a bespoke web-based registry adapted for routine care of patients with this form of rare and complex diseases, allowing an efficient data-repository at a national level with the potential to link with other international databases. It facilitates research, trials recruitment, service planning and benchmarking.

Neuropathic pain in systemic sclerosis patients: a cross-sectional study / Dolor neuropático en pacientes con esclerosis sistémica: un estudio transversal

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

OBJETIVOS: Valorar si los pacientes con esclerosis sistémica (ES) presentan una mayor prevalencia de dolor neuropático (DN) respecto a un grupo control. Estudiar la asociación entre variables clínicas y DN entre los pacientes. MATERIAL Y MÉTODOS: Fueron evaluados 48 pacientes con ES y un grupo control de 45 individuos. La presencia de DN fue determinada utilizando el cuestionario DN4 «Douleur Neuropathique en 4 Questions». Diferentes variables clínicas se evaluaron entre los pacientes. El análisis estadístico incluyó test paramétricos, no paramétricos y regresión logística multivariable. RESULTADOS: La presencia de DN fue significativamente mayor en el grupo de pacientes (56,2 vs.13,3%; p < 0,001). La media de Modified Rodnan Skin Score se asoció de manera independiente a la presencia de DN (p < 0,05; OR: 1,90). CONCLUSIONES: La afectación del sistema nervioso periférico en la ES no está bien estudiada. Según el conocimiento de los autores, este es el primer estudio publicado en evaluar el DN en estos pacientes respecto a un grupo control. Estos hallazgos deberían llamar la atención para reconocer la presencia de DN en estos pacientes, especialmente si existe afectación cutánea grave

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.

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.