Diffuse cutaneous systemic sclerosis following SARS-Co V-2 vaccination.

The largest world-wide vaccination rollout ever is currently underway to tackle the covid-19 pandemic. We report a case of diffuse cutaneous systemic sclerosis (SSc) in a 70-year-old male with rapidly progressive skin thickening which developed two weeks after receiving the first dose of the ChAdOx1 nCOV-19 vaccine. As the onset of SSc skin was in close temporal proximity to the administration of the first dose vaccine with no other triggers, we suspected a possible adverse reaction to the ChAdOx1 nCOV-19 vaccine.

Reliability of digital ulcer definitions as proposed by the UK Scleroderma Study Group: A challenge for clinical trial design.

Introduction: The reliability of clinician grading of systemic sclerosis-related digital ulcers has been reported to be poor to moderate at best, which has important implications for clinical trial design. The aim of this study was to examine the reliability of new proposed UK Scleroderma Study Group digital ulcer definitions among UK clinicians with an interest in systemic sclerosis.

Methods: Raters graded (through a custom-built interface) 90 images (80 unique and 10 repeat) of a range of digital lesions collected from patients with systemic sclerosis.

Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study.

Objectives: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs).

Methods: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months).

Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study.

Objectives: Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features.

Methods: Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement.

Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS).

Objectives: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

Methods: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening).

Consensus best practice pathway of the UK Systemic Sclerosis Study group: management of cardiac disease in systemic sclerosis.

Objective: Cardiac disease in SSc can manifest in various ways and is associated with a poor prognosis. There is little evidence on how best to detect and manage cardiac disease in SSc. Our objective was to produce an expert consensus best practice pathway for the management of cardiac disease in SSc.

Clinical efficacy of oral alendronate in ankylosing spondylitis: a randomised placebo-controlled trial.

Objectives: A prospective, double blind, randomised, placebo controlled trial over 2 years was performed to test the efficacy of alendronate, an oral aminobisphosphonate, in improving symptoms and arrest disease progression in patients with mild to severe ankylosing spondylitis (AS).

Methods: 180 patients with AS were randomised to receive weekly alendronate 70 mg or placebo (1:1 randomisation). BAS-G was the primary outcome measure with Bath indices as secondary outcomes.

Secondary vasculitis in autoimmune connective tissue diseases.

Purpose Of Review: The current review of the vasculitis associated with autoimmune connective tissue diseases is very appropriate and timely, in view of the recently acquired expanding knowledge, over the last decade, on the clinical epidemiology, pathophysiology, and the innovative therapeutic strategies. This review will specifically focus on the clinical presentations of secondary vasculitides, pathophysiology, and their management.

Recent Findings: New knowledge on the immunological triggers and pathophysiology of these conditions, in relation to various infections and airborne substances are discussed apart from the novel targeted immunotherapy, which has a direct effect on the outcome and reduction of iatrogenic complications.

A case of multicentric carcinoid in a patient with psoriatic spondyloarthropathy.

We describe the first case of a patient presenting with multicentric carcinoid occurring in the lung and subsequently in the rectum, with chronic psoriatic arthritis. Although reports have been published regarding carcinoid syndrome occurring alongside rheumatoid arthritis, no reports have been made on such a case. Initial presentation of carcinoid syndrome in this patient was insidious and atypical with few symptoms, including shortness of breath and long standing abdominal bloating.

Tumour necrosis factor inhibitors versus combination intensive therapy with conventional disease modifying anti-rheumatic drugs in established rheumatoid arthritis: TACIT non-inferiority randomised controlled trial.

Objective: To determine whether intensive combinations of synthetic disease modifying drugs can achieve similar clinical benefits at lower costs to high cost biologics such as tumour necrosis factor inhibitors in patients with active rheumatoid arthritis resistant to initial methotrexate and other synthetic disease modifying drugs.

Design: Open label pragmatic randomised multicentre two arm non-inferiority trial over 12 months.

Setting: 24 rheumatology clinics in England.

Paget's disease: a clinical review.

Paget's disease was first described more than 150 years ago, but the exact cause is still unknown--genes and the environment are both important. This article explores the basic science and clinical aspects of this intriguing condition.

Second-line agents in myositis: 1-year factorial trial of additional immunosuppression in patients who have partially responded to steroids.

Objective: Ciclosporin and MTX are used in idiopathic inflammatory myopathies (DM and PM) when patients incompletely respond to glucocorticoids. Their effectiveness is unproved in randomized controlled trials (RCTs). We evaluated their benefits in a placebo-controlled factorial RCT.

Consensus best practice pathway of the UK scleroderma study group: gastrointestinal manifestations of systemic sclerosis.

Systemic sclerosis is an autoimmune connective tissue disorder, which can be progressive with multisystem involvement. Guidance on the management of complications is based on a limited data set and practice amongst clinicians can vary. The UK Scleroderma study group set up several working groups to agree some consensus pathways for the management of specific complications.

A randomized placebo-controlled trial of methotrexate in psoriatic arthritis.

Objective: MTX is widely used to treat synovitis in PsA without supporting trial evidence. The aim of our study was to test the value of MTX in the first large randomized placebo-controlled trial (RCT) in PsA.

Methods: A 6-month double-blind RCT compared MTX (15 mg/week) with placebo in active PsA.

Musculoskeletal examination--an ignored aspect. Why are we still failing the patients?

Over the past two decades, rheumatologists from around the world have not only championed the musculoskeletal system examination but also modified the undergraduate teaching curriculum. This has led to the development and adoption of the gait, arms, legs and spine (GALS) screening along with regional examination techniques. The purpose of this study is to review current practice, determining the frequency of patient exposure to appropriate examination and confidence of junior doctors when dealing with MSK conditions.

The BILAG multi-centre open randomized controlled trial comparing ciclosporin vs azathioprine in patients with severe SLE.

Objective: To determine whether low-dose ciclosporin was a more effective corticosteroid-sparing agent than AZA in patients with SLE.

Methods: Patients with SLE requiring a change or initiation of a corticosteroid-sparing agent and who were taking > or =15 mg of prednisolone/day were randomized to receive either ciclosporin or AZA during this 12-month open-label multi-centre trial. There were strict guidelines for the reduction of prednisolone.

Clinical outcomes, quality of life, and diagnostic uncertainty in the first year of polymyalgia rheumatica.

Objective: To evaluate the impact of polymyalgia rheumatica (PMR) on clinical outcomes and quality of life (QOL); the relationship between laboratory measures and clinical outcomes, and changes in QOL; and agreement between rheumatologists in confirming the initial diagnosis.

Methods: We conducted a prospective study of 129 participants in 8 hospitals in England who met a modified version of the Jones and Hazleman criteria and had not started steroid therapy. The main outcome measures were response to steroids after 3 weeks (minimum 50% improvement in proximal pain, morning stiffness <30 minutes, acute-phase response not elevated), relapses, QOL as measured by the Short Form 36 and Health Assessment Questionnaire, and diagnosis reassessment at 1 year.

A case of polymyalgia rheumatica, microscopic polyangiitis, and B-cell lymphoma.

Background: A 73-year-old, previously well woman was admitted to an emergency department because of a 3-month history of severe proximal girdle pain and stiffness with loss of appetite and weight. She was referred to a rheumatologist 10 days after her initial presentation. Within 4 weeks she presented to an outpatient clinic with nausea, vomiting, shortness of breath, painful mouth ulcers, rash on her legs and a further decline in appetite; she was readmitted to hospital.

Kaposi sarcoma in a patient with giant cell arteritis.

Kaposi sarcoma usually occurs in immunosuppressed patients. A classic type has been reported in elderly men of Jewish and Mediterranean origin. We report a case of an elderly woman with giant cell arteritis (GCA) who developed Kaposi sarcoma while on a double blind trial for GCA with an anti-tumor-necrosis-factor medication.

Evidence-based clinical guidelines: a new system to better determine true strength of recommendation.

Rationale, Aims And Objectives: Clinical practice guidelines often grade the 'strength' of their recommendations according to the robustness of the supporting research evidence. The existing methodology does not allow the strength of recommendation (SOR) to be upgraded for recommendations for which randomized controlled trials are impractical or unethical. The purpose of this study was to develop a new method of determining SOR, incorporating both research evidence and expert opinion.