Elderly versus younger patients with microscopic polyangiitis vasculitis (MPA): a single-center retrospective study.

Microscopic polyangiitis (MPA) is a form of necrotizing vasculitis affecting the small vessels. Our study is the first study with the objective of describing the difference in clinical presentation of MPA and response to treatment at 6 months based on the age of disease onset. All patients seen at a tertiary vasculitis clinic between 2015 and 2023 with a diagnosis of MPA were included. Patients were divided into an elderly group (age > = 65 years) and a younger group (age < 65). Comparative analysis was conducted to characterize differences amongst the elderly and younger patients, including differences in organ involvement and clinical presentation, Birmingham Vasculitis Activity Score (BVAS) scores, Vasculitis Damage Index( VDI) scores, and response to treatment at 6 months. Thirty-one patients were included in our study. Younger MPA patients (n = 18) with mean age at diagnosis of 53.17 years were compared with older MPA patients(n = 13) with mean age at diagnosis of 76.08 years. The younger patients had statistically significant higher BVAS scores (p = 0.009), along with higher incidence of renal (p = 0.028), pulmonary (p = 0.0069), and cutaneous (p = 0.026) manifestations at disease onset. Furthermore, amongst the younger population, there was noted statistically significant clinical improvement at 6 months following treatment induction, particularly in the domains of general symptoms (p = 0.011), MSK (p = 0.019), cutaneous (p = 0.011), and pulmonary symptoms (p = 0.04). In contrast, the elderly population presented with a predominant of non-specific constitutional symptoms, with statistically significant improvement in the domain of non-specific general symptoms at 6 months (p = 0.00008). All MPA patients responded well to treatment, with statistically significant improvement amongst younger patients (p = 0.0032), but no statistically significant improvement amongst elderly patients (p = 0.67). Our study findings concluded that MPA's clinical presentation differed by age group. Younger patients had more aggressive vasculitis disease presentation with better response to treatments, whereas, elderly patients had less severe disease presentation with predominant of general symptoms at disease onset. Key Points • MPA clinical presentation differed by age at disease onset. Younger patients presented with more aggressive and classic vasculitis-like presentations, with multi-system organ involvement and statistically significant higher incidence of renal, pulmonary, and cutaneous manifestations. In contrast, elderly patients had a predominant of constitutional and non-specific symptoms with often delayed diagnosis. • All MPA patients responded well to treatment. Amongst the younger population, there was statistically significant improvement in BVAS scores after treatment induction at 6 months; however, there was no statistically significant improvement of BVAS scores in the elderly population. • Future studies are required to further understand the difference in the clinical presentation of MPA based on the age at disease onset, and the implication on disease diagnosis and management.

Painless ulceration due to digital hypoperfusion ischaemic syndrome: case report and literature review.

Digital hypoperfusion ischaemic syndrome (DHIS), also known as steal syndrome, is a well recognised serious complication of haemodialysis (HD) access creation. The clinical presentation varies from cyanosis to tissue loss due to necrosis or gangrene. In this article, we present a case of painless digital ulceration due to DHIS and provide a review of the literature. A 40-year-old-female presented with multiple painless digital ulcerations of the left hand. Her medical profile included atherosclerotic disease, hypertension, hyperparathyroidism and type I diabetes causing retinopathy, peripheral neuropathy, gastroparesis and end-stage renal disease (ESRD). Her ESRD required HD with the construction of a left-arm basilic vein transposition arteriovenous fistula (AVF). A year later, she developed intermittent, painless ulcerations of the left hand. A Doppler ultrasound confirmed the diagnosis of DHIS. The patient was treated with AVF ligation surgery. At six months postoperatively, she had near complete re-epithelialisation of her ulcers. This case is unique in that the patient did not have preceding pain, likely due to her underlying diabetic neuropathy. While DHIS in haemodialysis patients with AVF is well documented in literature, digital ulceration in this context is an advanced form of this condition. Early recognition of digital ulceration as a complication of DHIS may enable early intervention and prevent permanent damage.

Predictors of family impact of juvenile localized scleroderma.

OBJECTIVE: To measure the impact juvenile localized scleroderma (jLS) has on family quality of life and to identify predictors of family impact in this population which may inform the development of tailored resources to enhance family functioning for patients with jLS. METHODS: A retrospective cohort study of pediatric patients with jLS and their families was conducted. Five questionnaires were administered at each visit: Pediatric Quality of Life Inventory Family Impact Module (PedsQL-FIM), PedsQL 4.0 Generic Core Scales (PedsQL-Generic), PedsQL Rheumatology Module (PedsQL-RM), Child Health Assessment Questionnaire (CHAQ), and Children's Dermatology Life Quality Index (CDLQI). Linear mixed models with random intercepts for each patient were used to find relationships between family impact scores and clinically relevant variables over time. Variables of interest included disease activity status, methotrexate use, jLS distribution, and scores for PedsQL-Generic and PedsQL-RM. RESULTS: The median baseline PedsQL-FIM total score was 80.9 (IQR = 76.6-97.4). Adjusting for age and sex, the most significant predictors of family impact were PedsQL-Generic scores and four of five PedsQL-RM dimensions (all P < .001); methotrexate use had borderline significance (P = .06). Family impact increased more significantly over time in older patients. In multivariable modeling, PedsQL-Generic total score and jLS "other" distribution were significant for predicting an increased PedsQL-FIM score (P = .003 and P = .03, respectively). CONCLUSIONS: JLS has a moderate family impact. Family impact is predicted by patients' general and disease-specific health-related quality of life (HRQL) and their jLS subtype. There is a trend toward increased family impact with methotrexate treatment. This study emphasizes the importance of family-centered care in jLS.

Understanding Dermatologic Concerns Among Persons Experiencing Homelessness: A Scoping Review and Discussion for Improved Delivery of Care.

There is a paucity of information surrounding dermatologic care for persons experiencing homelessness (PEH). This scoping review aims to map existing literature and provide a summary of the most common cutaneous manifestations among PEH, risk factors for dermatologic disease, describe any reported interventions, as well as identify research gaps for future studies. Search strategies developed for MEDLINE and hand searching yielded 486 articles. Out of the 486 articles screened, 93 articles met the inclusion criteria. The majority were cohort studies, cross-sectional studies, and case-control studies concentrated in North America and Europe. Excluding the pediatric population, the prevalence of dermatologic conditions ranged from 16.6% to 53.5%. Common skin conditions described in PEH were: acne, psoriasis, seborrheic dermatitis, atopic dermatitis, and lichen simplex chronicus. There were no studies comparing the extent or severity of these cutaneous diseases in PEH and the general population. PEH have a higher prevalence of skin infections and non-melanoma skin cancers. This scoping review has direct implications on public health interventions for PEH and highlights the need for evidence-based interventions to provide optimum and safe dermatologic healthcare for PEH. We propose several recommendations for improved care delivery, including addressing upstream factors and comorbidities impacting skin health, providing trauma informed care, reducing barriers to care, preventing and managing skin conditions, as well as including PEH in the planning and implementation of any proposed intervention.