Multi-centre national audit of juvenile localised scleroderma:Describing current UK practice in disease assessment and management

OBJECTIVE: To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations. METHODS: Patients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015-04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally. RESULTS: 149 patients were included with a median age of 12.5 years. The outcome measures recommended by the PRES scleroderma working party were not utilised widely. The localised scleroderma cutaneous assessment tool was only used in 37.6% of patients. Screening for extracutaneous manifestations did not meet recommendations that patients with head involvement have regular screening for uveitis and baseline magnetic resonance imaging (MRI) brain: only 38.5% of these patients were ever screened for uveitis; 71.2% had a MRI brain. Systemic treatment with disease-modifying anti-rheumatic drugs (DMARDs) or biologics was widely used (96.0%). In keeping with the recommendations, 95.5% of patients were treated with methotrexate as first-line therapy. 82.6% received systemic corticosteroids and 34.2% of patients required two or more DMARDs/biologics, highlighting the significant treatment burden. Second-line treatment was mycophenolate mofetil in 89.5%. CONCLUSION: There is wide variation in assessment and screening of patients with JLS but a consistent approach to systemic treatment within UK paediatric rheumatology. Improved awareness of PRES recommendations is required to ensure standardised care. As recommendations are based on low level evidence and consensus opinion, further studies are needed to better define outcome measures and treatment regimens for JLS

Chronic non bacterial osteitis- a multicentre study

Abstract Objective To understand the demographics, clinical features and treatment outcomes of Chronic Non-bacterial Osteitis (CNO) from three tertiary paediatric rheumatology services in the United Kingdom. Methods Children less than 18 years of age diagnosed with CNO between 2001 to 2016 from one tertiary service and between 2001 to 2017 from two tertiary services were included. Clinical notes were reviewed and all pertinent data were collected on a pre-defined proforma. One hundred and thirty one patients were included in the study. The Bristol diagnostic criteria were applied retrospectively. Results Retrospective analysis of the data showed that the disease was more common in girls than boys (2.5:1), median age at onset of symptoms was 9.5 years (IQR 8 to 11 years). Bone pain was the predominant symptom in 118/129 (91.4%) followed by swelling in 50/102 (49.01%). Raised inflammatory markers were present in 39.68% of the patients. Whole body Magnetic Resonance Imaging (MRI) was a useful diagnostic tool. Metaphyses of long bones were most often involved and the distal tibial metaphyses 65/131 (49.6%) was the most common site. Non-steroidal anti-inflammatory drugs were used as first line (81.67%) followed by bisphosphonates (61.79%). Treatment was escalated to a TNF blocker when response to bisphosphonates was suboptimal. The disease was in remission in 82.4% of the patients during the last follow up. Conclusion Our multicentre study describes features and outcomes of CNO in a large number of patients in the United Kingdom. Significance and innovation Raised inflammatory markers were present in 39.68% of our patients.Whole body MRI is useful for diagnosis and also determining response to treatment.A greater number of lesions were detected on radiological imaging compared to clinical assessment.Metaphyses of long bones were most often involved and the distal tibial metaphyses (49.6%) were the most common site.Non-steroidal anti-inflammatory drugs were used as first line (81.67%) followed by bisphosphonates (61.79%).There was no difference in number of medications used for management in unifocal versus multifocal disease.TNF blockers were used with good effect in our cohort

Checklist, typification details, and nomenclature status of ascomycetous fungi originally described in Sri Lanka.

Despite being a biodiversity-rich country, Sri Lanka’s fungal diversity remains largely unexplored. In the 19th century, British mycologists conducted extensive research, leading to the identification of more than 1,800 fungal taxa, including ascomycetous and basidiomycetous species. However, the majority of these taxa have not been revisited since their initial description, and molecular evidence is lacking. Moreover, a significant number of fungal taxa have been deemed invalid or illegitimate, indicating the need for a nomenclatural revision. To address this knowledge gap, this review provides a comprehensive list of ascomycetous taxa (including both lichen-forming and non-lichenized) that were originally described in Sri Lanka. Names are listed according to the original names which were included in the protologue. In the cases where species have been transferred to other genera, the current names and new classifications are provided. The typification details, nomenclature status, and classification are also presented. This checklist will serve as a valuable resource for the future epitypification of old taxa, as much of the existing information is poorly documented and scattered

Hymenochaete subpurpurascens Berk. & Broome, J. Linn. Soc., Bot.

<i> <i>S</i>.</i> <i>subpurpurascens</i> Berk. & Broome, J. Linn. Soc., Bot. 14 (no. 74): 66 (1873) [1875]/IF: 203170 <p> Current name: <i>Hymenochaete subpurpurascens</i> (Berk. & Broome) Massee, J. Linn. Soc., Bot. 27: 103 (1890) (New classification: <i>Hymenochaetaceae, Hymenochaetales, Agaricomycetes</i>)</p> <p>Typification Details: not indicated, On dead wood, Sri Lanka</p> <p>Reference: Berkeley & Broome (1875)</p>Published as part of <i>Karunarathna, Samantha C., Priyashantha, K Hasith, Tibpromma, Saowaluck, Galappaththi, Mahesh C. A., Premarathne, Bhagya M., Wijayawardene, Nalin N., Wimalasena, Madhara K., Jayalal, Udeni, Wickramanayake, Kawmini D., Dangalla, Hasanka, Jayathunga, Hashini, Brahmanage, Rashika S., Weerakoon, Gothamie, Ariyawansa, Kahandawa G. S. U., Yapa, Neelamanie, Nanayakkara, Chandrika M., Ediriweera, Surani, Fan, Xin-Lei, Kirk, Paul M., Zhang, Gui-Qing, Ediriweera, Aseni, Bhat, Jayarama, Dawoud, Turki M., Kumara, Wasantha & Deng, Chun Ying, 2023, Checklist, typification details, and nomenclature status of Basidiomycota, originally described from Sri Lanka, pp. 1-86 in Phytotaxa 621 (1)</i> on page 66, DOI: 10.11646/phytotaxa.621.1.1, <a href="http://zenodo.org/record/10065102">http://zenodo.org/record/10065102</a&gt

Favolus scaber Berk. & Broome, J. Linn. Soc., Bot.

<i> <i>F</i>.</i> <i>scaber</i> Berk. & Broome, J. Linn. Soc., Bot. 14 (no. 73): 57 (1873) [1875]/IF: 241279 <p> Current name: <i>Favolus tenuiculus</i> P. Beauv., Fl.Oware 1(8): 74[tab. 43,fig.2] (1806) (New classification: <i>Polyporaceae, Polyporales, Agaricomycetes</i>)</p> <p>Typification Details: K (M), Gardner 618; K (M), Gardner 46, On dead wood, Sri Lanka</p> <p>Reference: Berkeley & Broome (1873)</p>Published as part of <i>Karunarathna, Samantha C., Priyashantha, K Hasith, Tibpromma, Saowaluck, Galappaththi, Mahesh C. A., Premarathne, Bhagya M., Wijayawardene, Nalin N., Wimalasena, Madhara K., Jayalal, Udeni, Wickramanayake, Kawmini D., Dangalla, Hasanka, Jayathunga, Hashini, Brahmanage, Rashika S., Weerakoon, Gothamie, Ariyawansa, Kahandawa G. S. U., Yapa, Neelamanie, Nanayakkara, Chandrika M., Ediriweera, Surani, Fan, Xin-Lei, Kirk, Paul M., Zhang, Gui-Qing, Ediriweera, Aseni, Bhat, Jayarama, Dawoud, Turki M., Kumara, Wasantha & Deng, Chun Ying, 2023, Checklist, typification details, and nomenclature status of Basidiomycota, originally described from Sri Lanka, pp. 1-86 in Phytotaxa 621 (1)</i> on page 53, DOI: 10.11646/phytotaxa.621.1.1, <a href="http://zenodo.org/record/10065102">http://zenodo.org/record/10065102</a&gt