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Objectives: The aim of this study was to add to the descriptive data pertaining to the epidemiology, presentation, and clinical course of multisystem inflammatory syndrome (MIS) temporally associated with coronavirus disease 2019 in adults and adolescents from low- and middle-income countries. Methods: Patients presenting to the adult wards (14 years and older) of three academic hospitals in South Africa, who were diagnosed with MIS between August 1, 2020 and May 31, 2021, were reviewed retrospectively. The presentation, laboratory and radiographic findings, and clinical course are described. Results: Eleven cases of MIS were reported, four in adolescents (14–19 years) and seven in adults (≥19 years). Fever was universal. Gastrointestinal symptoms (90.9%), cardiorespiratory abnormalities (90.9%), and mucocutaneous findings (72.7%) were prominent. Echocardiography in 10/11 patients (90.9%) showed a median left ventricular ejection fraction of 26.3% (interquartile range 21.9–33.6%). All patients required high care admission and 72.7% required inotropic support. Glucocorticoids were initiated in all cases and 72.7% received intravenous immunoglobulin. Conclusions: This constitutes the largest multicentre review of adults and adolescents with MIS in Africa. MIS may be overlooked in resource-limited settings, and heightened suspicion is needed in patients with multi-organ dysfunction, especially where repeated investigations for other aetiologies are negative.

Since late April 2020, a syndrome now termed Multisystem Inflammatory Syndrome in Children (MIS-C) has been seen in children and adolescents in association with COVID-19 infection. The definition of MIS-C involves fever, organ dysfunction and laboratory confirmation of inflammation in the context of laboratory or epidemiological evidence of SARS-CoV-2 infection in a patient under 21 years of age. Notably, cases are now being identified in adults termed Multisystem Inflammatory syndrome in Adults (MIS-A). Few cases have been reported in sub-Saharan Africa. We report a case of a young African male presenting with a maculopapular rash, persistent fever, elevation in inflammatory markers and a sudden, significant deterioration in cardiac function resulting in cardiogenic shock. The patient responded to intravenous steroids, intravenous immunoglobulin and background inotropic support. The recognition of this disease entity proves even more crucial now amidst the ongoing global wave of COVID-19 infection. It is paramount to identify these patients early, leading to prompt treatment avoiding further morbidity and mortality.

Objective.To investigate the association of specific amino acid positions, residues, and haplotypes of HLA-DRB1 in black South Africans with autoantibody-positive rheumatoid arthritis (RA).Methods.High-resolutionHLA-DRB1genotyping was performed in 266 black South Africans with autoantibody-positive RA and 362 ethnically and geographically matched controls. The alleles were converted to specific amino acid residues at polymorphic sites for downstream analyses. Logistic regression models were used to test whether variability at site, specific amino acid residues, and haplotypes (constructed from positions 11, 71, and 74) were associated with RA.Results.Of the 29 amino acid positions examined, positions 11, 13, and 33 (permutation p = 3.4e-26, 1.2e-27, and 2.1e-28, respectively) showed the strongest association with RA. Univariate analyses of individual amino acid residues showed valine at position 11 (OR 5.1, 95% CI 3.7–7.0) and histidine at position 13 (OR 6.1, 95% CI 4.2–8.6) conferred the highest risk. The valine containing haplotypes of position 11, 71, 74, V_K_A conferred the most risk (OR 4.52, 95% CI 2.68–7.61) and conversely the haplotype with serine at this position, S_K_R, conferred the most protection (OR 0.83, 95% CI 0.61–1.15).Conclusion.Autoantibody-positive RA in black South Africans is associated with histidine at position 13 and valine at position 11 of HLA-DRB1, and haplotypes with valine at position 11 conferred the highest risk; conversely, serine at position 11 conveyed protection.

Background Microvasculopathy is an important feature of systemic sclerosis (SSc), making its assessment a key issue in SSc clinical research. Nailfold videocapillaroscopy (NVC) is a valuable tool to detect and classify microvascular structural alterations. In contrast, laser speckle contrast analysis (LASCA), a non-invasive microvascular imaging tool, has been proposed as an objective technique to dynamically evaluate the peripheral blood perfusion (PBP). Objectives The specific objectives were 1) to perform a pilot study to investigate as first both the intra- and inter-rater reliability of LASCA in an unselected SSc cohort and descriptively in healthy subjects (HS) and 2) to identify the available literature on the reliability of LASCA in SSc by a systematic literature review. Methods First, a pilot study was performed to assess the reliability of LASCA to measure the PBP at the level of the fingers in an unselected cohort SSc patients and descriptively in HS. Intra-rater reliability was assessed by having a first anchor rater performing the measurements at 2 time-points (within 15 min) and inter-rater reliability by subsequently having the first anchor rater and a team of 3 s raters performing the measurements in 15 SSc and 30 HS (see figure 1). As external validation, the measurements were repeated with a second anchor rater in a distinct cohort of 15 SSc patients. Reliability was described by calculating the intraclass correlation coefficient (ICC). The systematic search was performed to identify relevant full-text articles in PubMed, EMBASE and Web of Science. All retrieved articles were screened on title, abstract and full-text level, reference lists were additionally searched. Results Thirty SSc patients (5 men, 25 women; mean age 52±17 y; 1LSSc, 25LcSSc, 4DcSSc; 14 vasodilatory therapy) and 30 HS (8 men, 22 women; mean age 33±11 y) underwent LASCA measurements. ICC for intra-rater reliability of the first anchor was 0.95 (95% CI 0.86–0.98) in SSc and 0.93 (95%CI 0.83–0.97) in HS, the ICC for inter-rater reliability was 0.97 (95%CI 0.90–0.99) in SSc and 0.93 in HS. Intra- and inter-rater reliability of anchor 2 was 0.78 (95%CI 0.46–0.92) and 0.87 (95%CI 0.67–0.96) respectively. The systematic search identified 64 unique articles, of which 12 were eligible for full-text review. Two additional references were identified through a reference search of retrieved articles. Only 1 of the 14 selected references that met the inclusion criteria documented reliability as outcome and was included in the final analysis. This pilot study by Lambrecht et al measured the PBP at the level of the fingertips and reported ICC values varying from 0.82–0.91 for the dorsal and 0.74–0.86 for the volar fingertips. Conclusions The body of knowledge regarding intra- and inter-rater reliability of LASCA in SSc is very limited. Only one manuscript reporting very good inter-rater reliability of PBP measurements of the distal fingertips by LASCA could be withheld. These results could be confirmed by our pilot study. In addition, we demonstrated excellent intra-rater reliability of LASCA measurements for the evaluation of the PBP of the hands in SSc patients and HS. Disclosure of Interest None declared

Objectives A reliable tool to evaluate flow is paramount in systemic sclerosis (SSc). We describe herein on the one hand a systematic literature review on the reliability of laser speckle contrast analysis (LASCA) to measure the peripheral blood perfusion (PBP) in SSc and perform an additional pilot study, investigating the intra- and inter-rater reliability of LASCA. Methods A systematic search was performed in 3 electronic databases, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In the pilot study, 30 SSc patients and 30 healthy subjects (HS) underwent LASCA assessment. Intra-rater reliability was assessed by having a first anchor rater performing the measurements at 2 time-points and inter-rater reliability by having the anchor rater and a team of second raters performing the measurements in 15 SSc and 30 HS. The measurements were repeated with a second anchor rater in the other 15 SSc patients, as external validation. Results Only 1 of the 14 records of interest identified through the systematic search was included in the final analysis. In the additional pilot study: intra-class correlation coefficient (ICC) for intra-rater reliability of the first anchor rater was 0.95 in SSc and 0.93 in HS, the ICC for inter-rater reliability was 0.97 in SSc and 0.93 in HS. Intra- and inter-rater reliability of the second anchor rater was 0.78 and 0.87. Conclusions The identified literature regarding the reliability of LASCA measurements reports good to excellent inter-rater agreement. This very pilot study could confirm the reliability of LASCA measurements with good to excellent inter-rater agreement and found additionally good to excellent intra-rater reliability. Furthermore, similar results were found in the external validation.

To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate-severe lung disease. Univariate predictors of ILD included: disease duration (OR 1.08, 95% CI 1.01-1.15); cough (OR 2.93, 95% CI 1.37-6.29); dyspnoea (OR 2.44, 95% CI 1.23-4.87); bibasal crackles (OR 7.58, 95% CI 3.31-17.37); diffuse cutaneous SSc (dcSSc) (OR 4.55, 95% CI 2.10-9.86) and a speckled anti-nuclear antibody (ANA) pattern (OR 2.47, 95% CI 1.25-4.90). Conversely, limited cutaneous disease (OR 0.22, 95% CI 0.09-0.50) and anti-centromere antibody (ACA) (OR 0.12, 95% CI 0.02-0.97) were protective. Independent predictors of ILD on multivariate analysis were bibasal crackles (OR 9.43, 95% CI 3.25-27.39), disease duration (OR 1.19, 95% CI 1.09-1.30) and speckled ANA (OR 2.95, 95% CI 1.22-7.15). Almost all (86.4%) patients received immunosuppressive treatment and the leading cause of death was related to ILD itself (44.4%). In this cohort of predominantly Black South Africans, SSc ILD was common and carried a poor prognosis. ILD occurred mainly, but not exclusively, in patients with dcSSc, especially those with a speckled ANA pattern. Conversely, the presence of ACA was protective against ILD.