Hypokalemic paralysis due to renal tubular acidosis: uncommon initial manifestation of primary Sjögren´s syndrome.

Primary Sjögren´s Syndrome is an immune-mediated disease characterized by exocrine glands dysfunction due to lymphoplasmacytic infiltration with sicca symptoms being one of its main features. The disease may, however, present as distal renal tubular acidosis due to renal involvement, which can range from asymptomatic to life-threatening. We describe the case of a 33-year-old woman with hypokalemic paralysis and metabolic acidosis secondary to distal renal tubular acidosis, leading to the diagnosis of primary Sjögren´s Syndrome. Although rare, recognizing primary Sjögren´s Syndrome as a possible cause of distal renal tubular acidosis may elicit an earlier diagnosis and treatment, improving the patient´s prognosis.

Perioperative management of disease-modifying antirheumatic drugs and other immunomodulators.

OBJECTIVES: We aim to summarize the relevant evidence and provide guidance for perioperative management of disease-modifying antirheumatic drugs (DMARDs) and other immunomodulators used in the treatment of the various inflammatory rheumatic diseases in patients submitted to elective surgery. METHODS: This is a review article directed towards clinical practice, based on recent literature available in PubMed database, as well as guidelines published by Rheumatology Societies. RESULTS: Treatment with conventional DMARDs (methotrexate, hydroxychloroquine, sulfasalazine and leflunomide) can be continued perioperatively; targeted synthetic DMARDs should be suspended at least 3 to 7 days before surgery, depending on the drug, and restarted 3-5 days after the procedure, while biologic DMARDs should be withheld a dosing cycle prior to surgery and resumed at least 14 days after the procedure, with evidence of complete wound healing. In the case of Systemic Lupus Erythematosus (SLE), one should consider the severity of the condition to make the decision about discontinuing immunomodulators (mycophenolate mofetil, azathioprine, cyclosporine, or tacrolimus) as these should be continued in severe SLE because of the increased risk of life-threatening flares. The usual dose of glucocorticoids should be continued perioperatively; however, elective procedures with high-risk surgical site infection should be postponed in patients under ≥20 mg/day prednisone or equivalent until the inflammatory process is controlled with the minimum effective dose. CONCLUSIONS: The perioperative management of patients with rheumatic disease under DMARDs or other immunomodulators is challenging but vital for achieving the best outcome possible. A multidisciplinary approach agreed upon by the anesthesiologist, surgeon and rheumatologist is the best strategy for success.

Advances in Ultrasound Imaging in Rheumatology-What Do They Mean and What Challenges Do They Pose? The Example of Microvascular Imaging.

Musculoskeletal ultrasound is an important tool for the monitorization of inflammatory rheumatic diseases, with subclinical synovitis being frequently detected in ultrasound scans of patients in clinical remission. It has been shown, for example, that the presence of Power Doppler signal synovial membrane has prognostic value for patients with rheumatoid arthritis. Microvascular imaging technologies significantly improve the sensitivity for slow-flow vessels and may potentially detect subclinical inflammation when Power Doppler fails to do so. The authors briefly discuss the implications of the use of such techniques in rheumatology setting and review available evidence.

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.

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.

Cutaneous vasculitis and granulomatous hepatitis as paradoxical adverse events of Infliximab.

Biological agents revolutionised the treatment of inflammatory arthropathies. Paradoxical adverse events (PAEs) are rare immunological side effects caused by such agents. The authors report concurrent presentation of two rare PAEs of tumor-necrosis-factor alpha inhibitors (iTNFa) - cutaneous vasculitis and granulomatous hepatitis - in a patient with psoriatic arthritis treated with infliximab and briefly discuss aspects of diagnosis, pathophysiology and management of such events in light of the available evidence.

Salivary gland ultrasound findings are associated with clinical and serologic features in primary Sjögren's syndrome patients.

OBJECTIVE: Salivary gland ultrasound (SGUS) has been applied in the diagnosis of primary Sjögren's syndrome (pSS). To investigate the association of SGUS findings with clinical and analytical features of pSS patients. MATERIAL AND METHODS: 54 pSS patients underwent SGUS evaluation. The parenchymal homogeneity of bilateral parotid and submandibular glands was graded using a score of 0 (normal) to 4 (gross inhomogeneity). Patients were classified into two groups according to the highest SGUS score obtained. The grades 1 and 2 were considered to be normal and grades 3 and 4 to represent pathological SGUS findings. Demographic, clinical and laboratorial data were collected and compared between the groups. RESULTS: Nineteen of 54 patients had pathological SGUS findings. These were associated with higher ESSDAI and positivity for rheumatoid factor and anti-SSB antibody. Anti-SSB antibody was strongly and independently associated with pathological US findings in the salivary gland of pSS patients. CONCLUSION: This study provides further evidence that salivary gland ultrasound is linked to clinical and autoimmunity profile in pSS and confirm what has been reported in the literature. SGUS represents a useful imaging tool for diagnostic and prognostic of pSS.

Chikungunya arthritis - should we expect it to become more common in Portuguese rheumatology?

Chikungunya virus is known to cause acute disease characterized by fever, rash, myalgias, conjunctivitis and arthritis, having potential to cause chronic musculoskeletal disease, namely persistent arthritis. The area of spread of the virus in the world has been increasing and the migratory flows make the occurrence of Chikungunya induced chronic arthritis more and more scattered. Data regarding the experience of Portuguese rheumatology centres in identifying and treating chronic ChikV induced arthritis are not available. The authors describe the diagnosis and treatment aspects of three cases of "imported" Chikungunya induced chronic arthritis, briefly discuss its approach in the light of current knowledge and alert to the possibility this situation may become more prevalent in the Portuguese rheumatology setting.

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.

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

Portuguese recommendations for the use of ultrasound in rheumatology.

INTRODUCTION: Ultrasound (US) is a relatively cheap, easily available and reliable method to improve the care of rheumatic patients. However, its use in rheumatology practice is very heterogeneous and needs to be standardized. OBJECTIVES: To develop recommendations for the use of US in rheumatic diseases endorsed by the Portuguese Society of Rheumatology. METHODS: A systematic literature review of the available recommendations on the use of ultrasound in rheumatic diseases was performed and presented in a Portuguese Society of Rheumatology meeting to a subgroup of rheumatologists and rheumatology trainees with special interest in the subject. The most important topics to be addressed were selected and assigned to subgroups for literature review and draft recommendations. Following an iterative process of consensus, the final recommendations were developed, and their level of agreement voted anonymously online. A recommendation was approved when the average level of agreement was ≥ 7.5 in a 10-point Likert scale. RESULTS: Fourteen recommendations were produced regarding nine rheumatology topics: rheumatoid arthritis, spondyloarthritis, connective tissue diseases, polymyalgia rheumatica, vasculitis, crystal-deposition diseases, soft tissue rheumatism, osteoarthritis and ultrasound-guided procedures. CONCLUSION: We developed an up-to-date guidance in the form of recommendations for the use of US in nine different areas of rheumatology. As ultrasound is an important imaging modality with increasing use in the rheumatology setting, and there are frequent technological advances in the ultrasound machines and probes, in parallel with continuous associated research, these recommendations should be regularly updated.

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.

Median nerve ultrasound findings in systemic sclerosis patients: How do they relate to clinical features of the disease? / Hallazgos ecográficos del nervio mediano en pacientes con esclerosis sistémica: ¿cómo se comparan con aspectos clínicos de la enfermedad?

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