Adherence to hydroxychloroquine in patients with systemic lupus: Contrasting results and weak correlation between assessment tools.

OBJECTIVES: Hydroxychloroquine (HCQ) is an anchor drug in the treatment of systemic lupus erythematosus (SLE). Adherence to HCQ is key for efficacy. Inaccurate evaluation of adherence could lead to non-justified switch to more expensive or less tolerated drugs. METHODS: Severe non-adherence rate to HCQ was estimated in a sample of SLE patients during a routine visit using blood HCQ concentration<200µg/L. Adherence was assessesd by the Medication Adherence Self-Report Inventory (MASRI)<80/100, 8-item Morisky Medication Adherence Scale (MMAS-8) ≤6/8, Health Care Provider (HCP) visual analog scale (VAS)<80/100. Same procedures were to be repeated during a further routine visit 6 to 12 months later. We described agreement and correlations between tools and compared severely non-adherent patients and others on their characteristics. RESULTS: The study involved 158 patients (86.1% females) aged 42.2±12.6 years treated with HCQ for 9.6±6.9 years. Blood HCQ concentration (mean±standard deviation) was 1046±662µg/L at visit 1 and 855±577µg/L at visit 2. At visit 1, the non-adherence rate varied from 3.2% (blood HCQ level<200µg/L) to 7.7% (MASRI), 12.4% (HCP-VAS) or 32.5% (MMAS-8). 37.8% of patients met at least one of the definitions of non-adherence. Patients' characteristics including SLE activity, damage and quality of life were similar between severely non-adherent patients and others. Correlations between blood HCQ-concentration and self-questionnaires were weak (r<0.25) and agreement between methods was poor. CONCLUSION: Blood HCQ concentration<200µg/L reveals severe non-adherence. Combining blood HCQ concentration with MASRI and MMAS-8 may help to better identify non-adherence in SLE. Agreement between methods was poor and correlations with HCQ level and SLE activity were weak.

Consideration of coping strategies for patients suffering from systemic lupus erythematosus: reflection for a personalised practice of patient education.

OBJECTIVES: Systemic lupus erythematosus (SLE) is a chronic autoimmune multi-organ disease with an unpredictable course. SLE causes functional disability, changes in body appearance, and psychological distress. When faced with SLE, patients have to implement coping strategies. Therefore, the aim of this study was to describe patients' coping strategies, consider the implications for a personalised practice of patient education and evaluate patients' adherence to HCQ treatment. METHODS: One hundred and fifty-eight SLE patients receiving hydroxychloroquine (HCQ) treatment entered a prospective, non-comparative, longitudinal study aimed at describing patients' coping strategies and evaluating their adherence to the HCQ regimen. Coping strategies were evaluated using an abbreviated French version of the WCC-27 exploring 3 dimensions of coping: problem-centered coping, emotion-centered coping and search for social support. Adherence was assessed by the MASRI, the MMAS-8 and also objectively assessed by the patient's serum level of HCQ. Data collected at study entry also included disease activity: SLEDAI, and disease extent: SLICC damage index. The prevalence of anxious and depressive symptoms was evaluated with the HADS. Quality of life was evaluated using the LupusQoL questionnaire. RESULTS: Patients were clustered using an unsupervised hierarchical classification based on coping strategies. Four clusters of patients were individualised. The cluster of patients with low problem-centered coping, high emotion-centered coping and the lowest search for social support had worse quality of life and more psychological distress. We did not find any inter-cluster differences in terms of compliance to HCQ. CONCLUSIONS: Patients' knowledge is not the only parameter to consider for a personalised educational therapy: psychological parameters such as coping must also be considered to ensure the best possible quality of life. For educational therapy purposes, it is important not to group patients with the same coping style; heterogenous groups will enable patients to share their experiences and learn from the coping strategies of others.

Efficacy of Azacitidine in autoimmune and inflammatory disorders associated with myelodysplastic syndromes and chronic myelomonocytic leukemia.

This retrospective study describes efficacy of Azacitidine on autoimmune disorders (AID) associated with MDS/CMML in 22 patients. Response of AID to Azacitidine was observed in 19 patients (86%). Reduction or discontinuation of steroids and/or immunosuppressive therapy (IST) was possible in 16 cases (73%). Hematologic response was seen in 55% of the patients. MDS/CMML and AID evolution was concordant in 13 cases (59%): both favorable (n=11), both unfavorable (n=2), but AID improved while MDS/CMML worsened (n=8) and vice versa (n=1). Azacitidine frequently seems effective in controlling steroid-dependent AID associated with MDS/CMML, but prospective studies are necessary to confirm those findings.

In antisynthetase syndrome, ACPA are associated with severe and erosive arthritis: an overlapping rheumatoid arthritis and antisynthetase syndrome.

Anticitrullinated peptide/protein antibodies (ACPA), which are highly specific for rheumatoid arthritis (RA), may be found in some patients with other systemic autoimmune diseases. The clinical significance of ACPA in patients with antisynthetase syndrome (ASS), a systemic disease characterized by the association of myositis, interstitial lung disease, polyarthralgia, and/or polyarthritis, has not yet been evaluated with regard to phenotype, prognosis, and response to treatment. ACPA-positive ASS patients were first identified among a French multicenter registry of patients with ASS. Additionally, all French rheumatology and internal medicine practitioners registered on the Club Rhumatismes et Inflammation web site were asked to report their observations of ASS patients with ACPA. The 17 collected patients were retrospectively studied using a standardized questionnaire and compared with 34 unselected ACPA-negative ASS patients in a case-control study. All ACPA-positive ASS patients suffered from arthritis versus 41% in the control group (P < 0.0001). The number of swollen joints was significantly higher (7.0 ±â€Š5.0 vs 2.9 ±â€Š3.9, P < 0.005), with a distribution resembling that of RA. Radiographic damages were also more frequent in ACPA-positive ASS patients (87% vs 11%, P < 0.0001). Aside from a significantly higher transfer factor for carbon monoxide in ACPA-ASS patients, lung, muscle, and skin involvements had similar incidences, patterns, and severity in both groups. Although Nonbiologic treatments were similarly used in both groups, ACPA-positive patients received biologics more frequently (59% vs 12%, P < 0.0008), mostly due to refractory arthritis (n = 9). Eight patients received anti-Cluster of differentiation 20 (CD20) monoclonal antibodies (mAbs) with good efficacy and tolerance, whereas 2 of the 5 patients treated with antitumor necrosis factor drugs had worsened myositis and/or interstitial lung disease. After a >7-year mean follow-up, extra-articular outcomes and survival were not different. ACPA-positive ASS patients showed an overlapping RA-ASS syndrome, were at high risk of refractory erosive arthritis, and might experience ASS flare when treated with antitumor necrosis factor drugs. In contrast, other biologics such as anti-CD20 mAb were effective in this context, without worsening systemic involvements.