Herpes zoster in SLE: prevalence, incidence and risk factors.

OBJECTIVES: This study aimed to evaluate the prevalence and incidence of herpes zoster (HZ) events and describe its associated factors in a study of patients with SLE. METHODS: 491 consecutive SLE participants were screened for HZ events using a patient-reported questionnaire to capture outcomes on pain and other characteristics associated with HZ events. Sociodemographic, clinical and laboratory measures were also analysed, and time-dependent Cox regression survival analyses were performed to investigate factors associated with HZ events. RESULTS: Prevalence of HZ was 30.5%, incidence was 14.3 cases per 1000 person-years. Lymphopenia and glucocorticoid dosing were significantly associated with HZ events. CONCLUSIONS: HZ is highly prevalent in SLE, which may be linked to disease-related and treatment-related effects on cellular immunity. Our results suggest that the presence of certain risk factors may be useful to allow identification of patients at risk of HZ and improve its management in patients with SLE.

What is the prevalence of cognitive impairment in lupus and which instruments are used to measure it? A systematic review and meta-analysis.

OBJECTIVES: To systematically review the literature on the prevalence of Cognitive Dysfunction (CD) in SLE patients in studies that used a specified neuropsychological instrument. METHODS: This review was prepared with a protocol following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis-Protocols statement. Literature search in Ovid Medline, Embase, and Psyc INFO for articles on CD in adult SLE patients was conducted. Included studies were critically appraised (Newcastle-Ottawa Evaluation Scale) and the Pooled Prevalence (PP) of CD was studied for all instruments. The association between demographics and CD, the risk of CD in SLE compared to healthy subjects and patients with RA, and the course of CD over time were studied narratively whenever sufficient information was available. RESULT: Of 8054 references, 670 were selected for detailed review and 78 were included in the final analysis. Comprehensive Battery (CB) was utilized in 35 studies in 2463 SLE patients and PP was 38% (95%; CI: 33-43%). The CD prevalence was higher in NPSLE [PP 39% (95% CI: 24-55%]. Automated Neuropsychological Assessment Metric (ANAM) was utilized in 7 studies in 438 patients (PP of CD 26% (95% CI: 12-42%). Other less frequently utilized tools were the Modified Mini-Mental State Exam (MMSE), Montreal Cognitive Assessment (MoCA), Controlled Oral Word Association Test (COWAT) and The Hopkins Verbal Learning Test-Revised (HVLT-R) and subjective tools and others. The relative risk for CD in SLE was greater when compared to RA and to healthy individuals; RR being 1.80 and 2.80, respectively. Information on demographics and its association with CD was very heterogeneous among studies. CONCLUSION: Patients with lupus have a high prevalence of CD. The delay in diagnosis of CD is complex; although caregivers and patients express concerns about cognitive function, testing for CD often imposes administrative and cost burdens. There is an unmet need to identify the best screening, diagnostic metrics of CD. The assessment of cognitive function over time, and the association of demographics with CD, will require further research.

Mixed connective tissue disease: the King Faisal Specialist Hospital experience.

BACKGROUND: The aim of this study was to assess the clinical presentation, complications and serological analysis of mixed connective tissue disease (MCTD) at King Faisal Specialist Hospital & Research Centre (KFSH&RC), and to determine the long-term clinical and immunologic outcomes. PATIENTS AND METHODS: This was a retrospective study with prospective follow-up of 18 patients with MCTD who were followed at KFSH&RC between 1982 and 1999. RESULTS: The age at onset of the disease ranged from 6 to 44 years, with a mean age of 17.9 years. The female to male ratio was 2.5:1, and the mean follow-up time was 5 years. The most frequent presenting symptoms were arthralgia in all patients, Raynaud's phenomenon in 16 patients (88%) and swollen hands in 11 patients (61%). Arthritis was seen in 12 patients (67%) and definite myositis in 10 patients (58%). The most common skin rashes encountered included lupus-like rash in 8 patients (44%), scleroderma-like rash in 8 patients (44%), and cutaneous vasculitis in 5 patients (28%). Pulmonary hypertension occurred in 4 patients (22%). Other clinical manifestations encountered were esophageal hypomotility in 10 patients (56%), myocarditis in 2 patients (11%) and proteinuria in 2 patients (11%), while various neurological manifestations were present in 7 patients (39%). All patients exhibited high titer of ANA and anti-nRNP antibodies. Five of the 18 patients (28%) had marked reduction in the anti-nRNP during remission. Following treatment, features of inflammation as well as Raynaud's phenomenon and esophageal hypomotility diminished, while pulmonary hypertension persisted. A favorable outcome was observed in 12 patients (67%); 3 patients (17%) had continued active disease, while 3 patients (17%) died, with death related to pulmonary hypertension occurring in two patients (11%). CONCLUSION: The studied patients demonstrated the typical clinical and serological findings of MCTD, which support the correlation between anti-nRNP antibody specificities and MCTD. Autoantibody reactivity against nRNP polypeptides tends to regress during prolonged disease remission. The majority of our patients had favorable outcomes, with pulmonary hypertension being the most frequent disease-associated cause of morbidity and mortality.