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.

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.

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.

Rituximab in connective tissue disease-associated interstitial lung disease.

INTRODUCTION/OBJECTIVES: To evaluate rituximab (RTX) effectiveness and safety in patients with interstitial lung disease (ILD) related to connective tissue diseases (CTD). METHODS: Retrospective multicenter cohort study, including patients with CTD-ILD, followed in six Portuguese rheumatology departments until November 2018. ILD diagnosis was based on high-resolution CT (HRCT) and/or lung biopsy. Results of HRCT, pulmonary function tests, and 6-min walking test before and after RTX were compared using the Wilcoxon matched pair test. Safety, including adverse events during treatment and reasons for RTX discontinuation, was also analyzed. RESULTS: A total of 49 patients were included, with rheumatoid arthritis being the commonest CTD (61.2%). The median interval between CTD onset and ILD diagnosis was 4 years (IQR 1-9.5) and median ILD duration at first RTX administration was 1 year (IQR 0-4). The median RTX treatment duration until the last follow-up was 3 years (IQR 1-6). Usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) were the commonest patterns, occurring in 20 and 18 patients, respectively. One year after RTX first administration, there was a stabilization in carbon monoxide diffusing capacity (DLCO; mean + 5.4%, p = 0.12) and improvement in forced vital capacity (FVC; mean + 4.3%, p = 0.03), particularly in patients with NSIP. Patients with UIP had less promising results, but at 1 year, pulmonary function tests remained stable (DLCO + 2.5%, p = 0.77; FVC + 4.2%, p = 0.16). Infection was the main reason for RTX discontinuation and led to two deaths. CONCLUSIONS: RTX seems to be a promising treatment for CTD-ILD patients, particularly when NSIP pattern is present. Key points • The use of rituximab in patients with interstitial lung disease related to connective tissue disease is associated with long-standing disease stability in a wide range of systemic rheumatic diseases. • Efficacy results were particularly impressive in patients with non-specific interstitial pneumonia pattern, although in a subgroup of patients with usual interstitial pneumonia pattern, disease progression was also hold with this treatment. • In a large number of patients, rituximab was used in monotherapy and as first-line treatment.