Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management. We also highlight the need for well-conducted clinical trials designed to identify new and effective treatments.
Immunoglobulin A (IgA) vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) share many pathological parallels and are viewed as related diseases by many groups. Current treatment guidelines remain vague, controversial, and without consensus, especially regarding the role of immunosuppressive medications. We present five cases of IgAVN encountered at our tertiary care center between 2016 and 2020, which were treated with different immunosuppression regimens. Infection was the leading cause of death in this series. These cases provide evidence that IgAVN should be distinguished from IgAN on a spectrum of IgA-associated glomerulonephritis. The outcomes presented herein suggest that the morbidity and systematic involvement IgAVN is greater than previously believed and that these substantial risks should be reflected in contemporary treatment guidelines.
Myalgia and mild elevation in muscle enzymes are common side effects of statin therapy. While these symptoms are generally self-limited, in rare cases, statin use is associated with an immune-mediated necrotizing myopathy caused by development of autoantibodies against HMG-CoA reductase. The primary presenting symptom of this condition is progressive symmetric proximal weakness that does not abate or worsens even after cessation of statin therapy and is associated with markedly elevated creatine kinase (CK) levels. To date, no randomized controlled trials have been conducted to identify the most effective treatment for statin-associated autoimmune myopathy. Treatment recommendations involve a combination of steroids and immunosuppressive drugs. This single-center case series highlights the clinicopathologic features diagnostic for statin-associated autoimmune myopathy as well as treatment challenges for the patient population. The series highlights a range of potential presentations, from mildly symptomatic despite highly elevated CK, to severe muscle weakness including dysphagia. Multiple patients required several immunosuppressant medications as well as intravenous immunoglobulin (IVIG) to achieve disease control. In this case series, marked improvement was noted in several diabetic patients with IVIG.
Objective: Gout is the most common inflammatory arthritis in the United States. Despite published guidelines, management remains suboptimal, leading to unnecessary morbidity and increased cost of care. We have designed the gout disease management program (GDMP) to improve outcomes, increase patient satisfaction, and decrease healthcare utilization. Methods: Gout patients were seen at their usual rheumatology clinical visit and offered participation in the GDMP. Data were collected between April 2017 and November 2019. Serum uric acid (SUA) levels were measured at the initial outpatient encounter, at the entrance to GDMP, and every 4 weeks until SUA was at the goal of ≤6 mg/dl. Through telephonic encounters, gout-related recent hospitalizations, and ER or urgent care visits since the last encounter were ascertained. Self-reported gout medication usage and adherence were also determined. Patient satisfaction with GDMP was surveyed using a 5-point Likert scale. Results: A total of 158 patients were enrolled, of which 112 had ≥ 1 telephone encounter and were included in our analyses. During the telephone phase, 79 patients (70%) achieved the SUA goal of ≤6.0 mg/dl. Only 3 patients (2.6%) required hospitalization or visits to an ER or urgent care center due to gout flare, and 98% rated their encounter as a 5 on the 5-point Likert scale. Conclusion: Our telephone-based management program for gout led to improved clinical outcomes as defined by the ACR guidelines, decreased healthcare visits, and had high patient satisfaction. Significance and Innovations: • First telephone-based, rheumatology providers-led study to manage gout • Additional evidence to confirm the feasibility and benefit of telemedicine in common diseases • First study to show excellent patient satisfaction
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