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.

Portuguese recommendations for the use of biological and targeted synthetic disease-modifying antirheumatic drugs in patients with rheumatoid arthritis - 2020 update.

OBJECTIVE: To update the recommendations for the treatment of rheumatoid arthritis (RA) with biological and targeted synthetic disease-modifying antirheumatic drugs (bDMARDs and tsDMARDs), endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists taking into account previous recommendations, new literature evidence and consensus opinion. At a national meeting, in a virtual format, three of the ten previous recommendations were re-addressed and discussed after a more focused literature review. A first draft of the updated recommendations was elaborated by a team of SPR rheumatologists from the SPR rheumatoid arthritis study group, GEAR. The resulting document circulated among all SPR rheumatologists for discussion and input. The level of agreement with each of all the recommendations was anonymously voted online by all SPR rheumatologists. RESULTS: These recommendations cover general aspects such as shared decision, treatment objectives, systematic assessment of disease activity and burden and its registry in Reuma.pt. Consensus was also achieved regarding specific aspects such as initiation of bDMARDs and tsDMARDs, assessment of treatment response, switching and definition of persistent remission. CONCLUSION: These recommendations may be used for guidance of treatment with bDMARDs and tsDMARDs in patients with RA. As more evidence becomes available and more therapies are licensed, these recommendations will be updated.

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.

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.

Effect of biologic disease-modifying anti-rheumatic drugs targeting remission in axial spondyloarthritis: systematic review and meta-analysis.

OBJECTIVES: To assess the efficacy of biologic DMARDs (bDMARDs) in achieving Assessment of Spondyloarthritis International Society partial remission (ASAS-PR) and/or Ankylosing Spondylitis Disease Activity Score inactive disease (ASDAS-ID), as remission-like surrogates, in axial SpA (axSpA). METHODS: Data from randomized controlled trials (RCTs), including long-term extensions, were included. A systematic literature review was performed using the MEDLINE database (first search May 2018, updated February 2020) and PICO criteria according to Patients-adults with radiographic or non-radiographic axSpA; Intervention-any bDMARD; Comparator-placebo and/or any different drug; Outcomes-ASAS-PR and/or ASDAS-ID as primary or secondary endpoints. Meta-analysis was performed after assessment of the homogeneity of study designs, populations and outcomes. RESULTS: After screening 155 references, a total of 22 RCTs and 28 long-term extensions were retrieved. ASAS-PR was the dominant remission-like definition used. Concerning TNF inhibitors, 14/17 RCTs provided evidence of efficacy in reaching remission at different time points: 12, 16, 24 and 28 weeks (ASAS-PR in 16-62% of patients and ASDAS-ID in 24-40% of patients). With a limited number of studies available, IL-17A inhibitors exhibited remission rates of 15-21% for ASAS-PR and 11-16% for ASDAS-ID at week 16. A meta-analysis regarding ASAS-PR was performed considering RCTs with a similar duration (12, 16 or 24 weeks). The relative risk for achieving remission was 3.864 (95% CI 2.937, 5.085). CONCLUSION: bDMARDs have a clear impact in axSpA remission evaluated by ASAS-PR. Nevertheless, these data show an unmet need for improved reporting of remission-like outcomes.

Ultrasound-guided core biopsy of the parotid gland: the procedure from the Rheumatology point of view.

Ultrasound-guided core biopsy is a minimally invasive technique able to identify lymphoma accompanying Sjögren syndrome, neoplasms or infiltrative diseases, with less complications compared to open biopsy. With these images, we aim to describe the ultrasound-guided core biopsy procedure, in a female patient with rheumatoid arthritis who presented evident inhomogeneity of the parotid gland. The procedure was performed by rheumatologists, trained in ultrasonography of the salivary glands, demonstrating that ultrasound-guided core biopsy is an easy and safe method to obtain salivary gland tissue.

Eligibility criteria for biologic disease-modifying antirheumatic drugs in axial spondyloarthritis: going beyond BASDAI.

OBJECTIVES: To compare definitions of high disease activity of the Ankylosing Spondylitis Disease Activity Score (ASDAS) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in selecting patients for treatment with biologic disease-modifying antirheumatic drugs (bDMARDs). METHODS: Patients from Rheumatic Diseases Portuguese Register (Reuma.pt) with a clinical diagnosis of axial spondyloarthritis (axSpA) were included. Four subgroups (cross-tabulation between ASDAS (≥2.1) and BASDAI (≥4) definitions of high disease activity) were compared regarding baseline characteristics and response to bDMARDs at 3 and 6 months estimated in multivariable regression models. RESULTS: Of the 594 patients included, the majority (82%) had both BASDAI≥4 and ASDAS ≥2.1. The frequency of ASDAS ≥2.1, if BASDAI<4 was much larger than the opposite (ie, ASDAS <2.1, if BASDAI≥4): 62% vs 0.8%. Compared to patients fulfilling both definitions, those with ASDAS ≥2.1 only were more likely to be male (77% vs 51%), human leucocyte antigen B27 positive (79% vs 65%) and have a higher C reactive protein (2.9 (SD 3.5) vs 2.1 (2.9)). Among bDMARD-treated patients (n=359), responses across subgroups were globally overlapping, except for the most 'stringent' outcomes. Patients captured only by ASDAS responded better compared to patients fulfilling both definitions (eg, ASDAS inactive disease at 3 months: 61% vs 25% and at 6 months: 42% vs 25%). CONCLUSION: The ASDAS definition of high disease activity is more inclusive than the BASDAI definition in selecting patients with axSpA for bDMARD treatment. The additionally 'captured' patients respond better and have higher likelihood of predictors thereof. These results support using ASDAS≥2.1 as a criterion for treatment decisions.

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.

Real-world Longterm Effectiveness of Tumor Necrosis Factor Inhibitors in Psoriatic Arthritis Patients from the Rheumatic Diseases Portuguese Register.

OBJECTIVE: To assess longterm effectiveness of tumor necrosis factor inhibitors (TNFi) in patients with psoriatic arthritis (PsA) registered in the Rheumatic Diseases Portuguese Register, exposed to at least 1 TNFi, prospectively followed between 2001 and 2017. METHODS: Kaplan-Meier analysis was performed for first-, second-, and third-line TNFi. Responses included European League Against Rheumatism (EULAR) criteria, Disease Activity Index for Psoriatic Arthritis (DAPSA), minimal disease activity (MDA), and Ankylosing Spondylitis Disease Activity Score (ASDAS) at 3 and 6 months. Baseline predictors of discontinuation and response were studied using Cox and multivariable multinomial/logistic regression models. RESULTS: The 750 patients with PsA showed drug retention of 4.1 ± 3.4 years (followup 5.8 ± 3.8 yrs) for first TNFi. Switching to a second (189 patients) or third (50 patients) TNFi further decreased survival by 1.1 years. Female sex, higher baseline 28-joint count Disease Activity Score, and infliximab were predictors of first TNFi discontinuation. After 6 months of the first TNFi, 48.7% of patients achieved a good EULAR criteria response and 20.9% were in DAPSA remission. There were 11.4% in MDA, and 56.4% had a good ASDAS. Responses to the second TNFi were significantly inferior compared to responses to the first TNFi. Female sex and higher baseline Health Assessment Questionnaire-Disability Index were negatively associated with good EULAR response at 3 months, and obesity decreased the chance of response at 6 months. CONCLUSION: In this study, switching to a second or third TNFi was associated with significantly lower drug survival and response rates for patients with axial and peripheral PsA subtypes. More successful therapeutic approaches will require considering the effect of sex and obesity on TNFi effectiveness.

Systemic sclerosis induced by interferon-alfa treatment of melanoma.

Systemic sclerosis is a connective tissue disease characterized by a complex pathogenesis and a multi organ involvement of unknown etiology. Genetic features and environmental factors, as the use of some drugs, influence the onset of the clinical picture. The authors describe a case of a patient who developed systemic sclerosis after treatment of melanoma with interferon alfa-2b, drug rarely implicated in the induction of this disease.