Uncommon causes of nerve compression in the distal forearm / Causas poco frecuentes de compresión nerviosa en el antebrazo distal

Introduction: Nerve compression by anomalous masses located at the wrist and distal forearm is an infrequent condition. They may compress underlying structures in the carpal tunnel region, causing pain and paresthesias, which leads to the wrong diagnosis of carpal tunnel syndrome. Clinical cases: We present three cases of patients with symptomatology and clinical tests compatible with compression of the median nerve in the carpal tunnel but whose physical examination showed a soft mass in the distal region of the forearm which was compressing the median nerve, as demonstrated by ultrasound evaluation. Discussion: The reported cases of accessory muscles or lipomas described in the literature as causes of median nerve compression clinic are mainly described only after the surgical decompression of the carpal tunnel, due to the maintenance of residual symptoms. Conclusion: Careful examination with an ultrasound evaluation prior to surgery may help to identify these cases and help planning surgical treatment.(AU)

Introducción: La compresión del nervio mediano por masas anómalas localizadas en la muñeca y en el antebrazo distal es una condición infrecuente. Las estructuras subyacentes en la región del túnel carpiano pueden ser comprimidas, causando dolor y parestesias, pudiendo llevar al diagnóstico erróneo de síndrome del túnel carpiano. Casos clínicos: Se presentan 3 casos de pacientes con sintomatología y pruebas clínicas compatibles con compresión del nervio mediano en el túnel carpiano. Sin embargo, a la inspección presentaban leve edema en la región proximal del canal, y en la evaluación por ultrasonido se detectó sendas tumoraciones que comprimían el nervio mediano previo a su ingreso en el canal carpiano. Discusión: En la literatura, los casos de músculos accesorios o lipomas como causas de compresión del nervio mediano son las causas más probables de persistencia de los síntomas tras la descompresión quirúrgica del canal carpiano. Conclusión: La exploración clínica detallada junto con la evaluación ultrasonográfica antes de la cirugía pueden ayudar a identificar estos casos y planificar un mejor abordaje quirúrgico.(AU)

Uncommon causes of nerve compression in the distal forearm.

INTRODUCTION: Nerve compression by anomalous masses located at the wrist and distal forearm is an infrequent condition. They may compress underlying structures in the carpal tunnel region, causing pain and paresthesias, which leads to the wrong diagnosis of carpal tunnel syndrome. CLINICAL CASES: We present three cases of patients with symptomatology and clinical tests compatible with compression of the median nerve in the carpal tunnel but whose physical examination showed a soft mass in the distal region of the forearm which was compressing the median nerve, as demonstrated by ultrasound evaluation. DISCUSSION: The reported cases of accessory muscles or lipomas described in the literature as causes of median nerve compression clinic are mainly described only after the surgical decompression of the carpal tunnel, due to the maintenance of residual symptoms. CONCLUSION: Careful examination with an ultrasound evaluation prior to surgery may help to identify these cases and help planning surgical treatment.

Remission of Rheumatoid Arthritis and Primary Biliary Cholangitis After Treatment With Tocilizumab / Remisión de artritis reumatoide y colangitis biliar primaria tras tratamiento con 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.(AU)

La artritis reumatoide (AR) se caracteriza por sinovitis de múltiples articulaciones que, de no tratarse, deriva en destrucción articular. La colangitis biliar primaria (CBP) es una enfermedad hepática autoinmune y progresiva de origen desconocido. Cerca del 1,8-5,6% de los individuos con CBP padecen AR, y los pacientes con AR tienen mayor riesgo de desarrollar CBP, en comparación con la población general. Reportamos el caso de un varón de 76 años con historia de CBP y diagnóstico reciente de AR, en el que la terapia con tocilizumab fue efectiva para el control de ambas situaciones y realizamos una revisión de la literatura sobre el caso. Este caso junto con otro publicado en la literatura, en el que se utilizó tocilizumab para tratar CBP y AR, sugieren que este fármaco puede ser efectivo y seguro para el tratamiento de AR en pacientes con CBP. La inhibición de IL-6 puede resultar también eficaz en el tratamiento de la CBP.(AU)

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.

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.

Strategies for the withdrawal of classic and biological DMARD in clinically inactive patients with juvenile idiopathic arthritis.

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.

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.

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.

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.