Added value of Joint ENT-Rheumatology clinic in the management of ANCA-associated vasculitis: One year's experience.

PURPOSE: ANCA-associated vasculitides (AAV) represent a group of diagnoses, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA). Most commonly, they present initially with ENT-associated symptomatology, and therefore they often pose a diagnostic challenge. We aim to present our one-year experience in the joint management of AAV in a multi-disciplinary setting. METHODS: We performed a retrospective analysis based on the records of 39 patients who were seen in the joint clinic, during a period of one year. RESULTS: After clinical assessment, 13 patients had changes made to their ENT treatment, 2 had some changes in their immunosuppression, while 11 had changes in both ENT and Rheumatology treatment. Six patients did not require any alterations to their therapeutic scheme. On average three separate appointments were reduced to a single appointment in the joint clinic where definitive treatment decisions were made. This led to significant cost reductions. CONCLUSIONS: Cost-effectiveness, patient satisfaction, rapid multi-disciplinary evaluation, avoidance of unnecessary immunosuppression, patient education and medical training are only a few of the many advantages of this proposed joint service.

Identifying the best predictive diagnostic criteria for psoriasis in children (< 18 years): a UK multicentre case-control diagnostic accuracy study (DIPSOC study).

BACKGROUND: In children, psoriasis can be challenging to diagnose. Difficulties arise from differences in the clinical presentation compared with adults. OBJECTIVES: To test the diagnostic accuracy of previously agreed consensus criteria and to develop a shortlist of the best predictive diagnostic criteria for childhood psoriasis. METHODS: A case-control diagnostic accuracy study in 12 UK dermatology departments (2017-2019) assessed 18 clinical criteria using blinded trained investigators. Children (< 18 years) with dermatologist-diagnosed psoriasis (cases, N = 170) or a different scaly inflammatory rash (controls, N = 160) were recruited. The best predictive criteria were identified using backward logistic regression, and internal validation was conducted using bootstrapping. RESULTS: The sensitivity of the consensus-agreed criteria and consensus scoring algorithm was 84·6%, the specificity was 65·1% and the area under the curve (AUC) was 0·75. The seven diagnostic criteria that performed best were: (i) scale and erythema in the scalp involving the hairline, (ii) scaly erythema inside the external auditory meatus, (iii) persistent well-demarcated erythematous rash anywhere on the body, (iv) persistent erythema in the umbilicus, (v) scaly erythematous plaques on the extensor surfaces of the elbows and/or knees, (vi) well-demarcated erythematous rash in the napkin area involving the crural fold and (vii) family history of psoriasis. The sensitivity of the best predictive model was 76·8%, with specificity 72·7% and AUC 0·84. The c-statistic optimism-adjusted shrinkage factor was 0·012. CONCLUSIONS: This study provides examination- and history-based data on the clinical features of psoriasis in children and proposes seven diagnostic criteria with good discriminatory ability in secondary-care patients. External validation is now needed.

An unusual case of inflammatory meningitis in a young man with systemic lupus erythematosus.

We describe a man presenting with unusual neurological manifestations of systemic lupus erythematosus (SLE) including pachymeningitis, aseptic meningitis and encephalitis with grossly elevated cerebrospinal fluid protein, responding to immunosuppression. Initially he had intermittent dysarthria, dysphasia and unilateral upper limb weakness. One month later he experienced dysphasia, right-sided hemiparesis and confusion. Cerebrospinal fluid (CSF) analysis showed a white cell count of 70 x 106/litre and an unusually elevated protein level of 5.39 g/litre. An MRI brain showed dural and leptomeningeal enhancement compatible with a meningitic process. He improved with cefotaxime and aciclovir. On day seven of antimicrobials he developed left-sided weakness, sensory inattention and a left homonymous hemianopia. He responded well to intravenous methylprednisolone. On switching to oral prednisolone he developed expressive dysphasia, a right inferior quadrantanopia and seizures. His bloods were suggestive of macrophage activation syndrome. The patient improved with methylprednisolone and intravenous immunoglobulins, and the improvement was sustained on switching back to oral prednisolone. The prevalence of neuropsychiatric manifestations of SLE varies between 14 and 80% and according to the American College of Rheumatology includes 19 conditions. This case is unique because although some features were in keeping with aseptic meningitis the MRI appearances were also suggestive of pachymeningitis.

Multiple halo naevi associated with tocilizumab.

Tocilizumab, a humanized monoclonal antibody directed against the interleukin (IL)-6 receptor, is approved for the treatment of rheumatoid arthritis and juvenile idiopathic arthritis (JIA). We describe a case of multiple halo naevi occurring in a patient with a history of JIA treated with tocilizumab. IL-6 is a key cytokine in the setting of cancer through its effects on angiogenesis and inhibition of adaptive anti-tumour immunity. IL-6 also plays a role in melanocyte function, and increased levels have been noted in vitiligo skin, where it is a paracrine inhibitor of melanocytes. Tocilizumab may therefore lead to the development of halo naevi secondary to subsequent activation of adaptive immunity. Alternatively, as tocilizumab results in increased serum IL-6 levels, the epidermal cytokine profile is altered. Increased levels of IL-6 may therefore have a direct inhibitory effect on melanocytes, where access by tocilizumab may be limited due to differential size difference.

Vertebral fracture assessment scans enhance targeting of investigations and treatment within a fracture risk assessment pathway.

UNLABELLED: Vertebral fracture assessment (VFA) scanning is a useful tool to aid vertebral fracture identification. In this evaluation, we show that introduction of a comprehensive fracture risk assessment pathway incorporating VFA has enhanced diagnosis of vertebral fractures and improved targeting of investigations and treatment. INTRODUCTION: Vertebral fractures are a common manifestation of osteoporosis and are associated with an increased risk of future vertebral and non-vertebral fractures. VFA is a method of imaging the thoraco-lumbar spine and a useful tool to aid vertebral fracture identification. In August 2008, a new one-stop pathway was introduced incorporating VFA and laboratory investigations at the time of bone mineral density assessment. The aims of this evaluation were to evaluate the clinical utility of VFA in identifying vertebral fractures which had not presented clinically and to evaluate the impact of this on management. METHODS: We performed a retrospective 6-month review of the new pathway focussing on those patients undergoing VFA who were suspected to have a vertebral fracture. The outcomes of VFA, spinal X-rays and investigations were evaluated. RESULTS: Three thousand five hundred twenty-six individuals underwent fracture risk assessment over a 6-month period, of which 1,833 underwent VFA. Previously undiagnosed vertebral fractures were found in 202 individuals (36 were in retrospect apparent on prior imaging, and 29 were new vertebral fractures in patients with pre-existing vertebral fractures). Diagnosis of a vertebral fracture led to further investigation in all individuals and altered management in 59 (29 %) individuals. A potentially modifiable underlying cause was found in 42 (21 %). CONCLUSIONS: Introduction of a fracture risk assessment service incorporating VFA and a one-stop pathway has enhanced vertebral fracture identification and targeting of treatment and management.