RESUMEN
Systemic sclerosis (SSc) is typically characterized by positive antinuclear antibodies (ANA) and Raynaud's phenomenon (RP). We present the case of a male patient with progressive diffuse skin tightening, interstitial lung disease (ILD), pericardial tamponade, renal failure, and gastrointestinal dysmotility who was diagnosed with severe, rapidly progressive SSc despite negative ANA, absent RP, and a negative malignancy workup. The patient's clinical course was complicated by scleroderma renal crisis (SRC) requiring dialysis and eventual kidney transplantation. He also had severe gastrointestinal dysmotility requiring gastrostomy tube placement and total parenteral nutrition. Multiple agents were required for treatment, including mycophenolate mofetil (MMF) and rituximab. The patient eventually had improvement in his skin fibrosis and has been doing well in follow-up after kidney transplantation. Treatment of SSc can be challenging given the heterogeneity of the disease, and recognition of this subset of SSc patients is needed to help prevent early mortality among them.
RESUMEN
BACKGROUND/OBJECTIVE: To examine rates of counseling on driving for individuals with osteoarthritis (OA) and/or rheumatoid arthritis (RA) and evaluate the Health Assessment Questionnaire Disability Index (HAQ-DI) as a screening tool for further driving evaluation. METHODS: A cross-sectional survey was completed by individuals recruited via ResearchMatch (a national web-based recruitment tool) between March 5 and April 20, 2020. Individuals with a current US driver's license, ≥18 years old, with self-reported OA and/or RA diagnosis were surveyed about driving difficulty and vehicle modification and completed a HAQ-DI assessment. Respondents were dichotomized based on reporting vehicle modification(s) due to arthritis versus no modification(s) for univariate and multivariate analyses. RESULTS: Of 4,435 recruited patients, 304 (6.9%) met inclusion/exclusion criteria and completed the surveys. Of all respondents, 259 (85.2%) reported at least some difficulty with one or more driving activities, but only 47 (15.5%) reported discussion with a physician and/or healthcare professional. A total of 184 (60.5%) respondents had HAQ-DI ≥ 1 and were more likely to report vehicle modification(s) compared to respondents with HAQ-DI score < 1 (OR = 5.00, 95% CI = 2.69-9.32, p < 0.011) after controlling for age, gender, type of arthritis, and driving behaviors. CONCLUSION: Few respondents report discussion of driving difficulties with healthcare providers, although many report driving-related impairments, particularly those with HAQ-DI scores ≥ 1. Our data suggest a strong association between HAQ-DI scores and vehicle modification. The HAQ-DI may serve as a screening tool to predict a patient's need for driving evaluation and vehicle modification(s).